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Pulmon Ology MCQ S

This document provides an overview of pulmonary medicine topics covered by Dr. Anand V Joshi, a consultant intensivist and physician. It lists the following areas: anatomy and physiology, general examination and approach, radiology, procedures like bronchoscopy, obstructive airway diseases, parenchymal disorders, infections, interstitial disorders, lung cancer, and pleural disorders. It then provides more details on the anatomy and physiology section, including clinical significance of tracheobronchial tree anatomy and alveolar capillary membrane structure. Clinical case examples are also presented testing knowledge of pulmonary anatomy, physiology, and radiology.

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Sai Shanker
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100% found this document useful (1 vote)
1K views280 pages

Pulmon Ology MCQ S

This document provides an overview of pulmonary medicine topics covered by Dr. Anand V Joshi, a consultant intensivist and physician. It lists the following areas: anatomy and physiology, general examination and approach, radiology, procedures like bronchoscopy, obstructive airway diseases, parenchymal disorders, infections, interstitial disorders, lung cancer, and pleural disorders. It then provides more details on the anatomy and physiology section, including clinical significance of tracheobronchial tree anatomy and alveolar capillary membrane structure. Clinical case examples are also presented testing knowledge of pulmonary anatomy, physiology, and radiology.

Uploaded by

Sai Shanker
Copyright
© © All Rights Reserved
We take content rights seriously. If you suspect this is your content, claim it here.
Available Formats
Download as PPTX, PDF, TXT or read online on Scribd
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Pulmonary Medicine

Dr Anand V Joshi

Consultant Intensivist & Physician


Oslers Academy, Hyderabad.India
drjoshianand@gmail.com

Pulmonary Medicine

Anatomy & Physiology


General examination & Approach
Radiology
Procedures - Bronchoschopy & other
Obstructive airway diseases
Parenchymal disorders
Infections (Pneumonia, TB)
Interstitial disorders
Lung cancer
Pleural disorders (Effusion, Empyema,
Pneumothorax)
Sleep disorders (sleep disordered breathing)

ANATOMY AND
PHYSIOLOGY

Introduction
For effective gas exchange
A. Lungs
B. Neuronal Input
C. Muscles of the chest wall
D. The circulatory system

Clinical reasons to know the anatomy of laryngeal


cartilages

Two laryngeal cartilages can be felt easily in the


neck. The Adams apple is the laryngeal cartilage.
Below that is a gap, then a smaller cartilage called
the cricoid cartilage

The cricothyroid membrane between the laryngeal


and cricoid cartilages can be pierced
(cricothyrotomy) in an emergency to allow a patient
with severe upper airways obstruction to breathe

Tracheobronchial Tree

Clinical Significance of trachea


bronchial tree

The rate at which the air moves in


and out depends on the resistance
offered.
The resistance is inversely
proportional to radius of airway.

The radius of the lumen of airways in


the respiratory tract depends on
Smooth muscle tone
Thickness of the epithelium and
submucosa
Luminal contents
Pressure inside and outside the
airways

ALVEOLAR CAPILLARY MEMBERANE


The Contact area is approx 50-100 m2.
Thickness is approx 0.6 um

Ficks law of diffusion

Pulmonary vasculature

1) In the normal lung which of the following is correct?


a) Cartilage is present in all respiratory bronchioles.
b) The majority of airway resistance is generated by small airways.
c) There is an intrapleural pressure of 30 cmH2O (3kPa) at the end of
normal expiration.
d) There is a resting pulmonary blood flow of 10L/min.
e) The V:Q ratio is greater in apical than basal segments of the lung when
upright and at rest.

Ans: The V:Q ratio is greater in apical than basal segments of the lung
when upright and at rest.

52) The pulmonary vascular system is different from the systemic


circulation in that the pulmonary system demonstrates which of the
following?
a) High pressures, high flow rates, highly compliant vessels
b) High pressures, high flow rates, low compliance vessels
c) Low pressures, high flow rates, high compliance vessels
d) Low pressures, low flow rates, high compliance vessels
e) Low pressures, low flow rates, low compliance vessels

Ans: Low pressures, low flow rates, high compliance vessels

37) Which of the following statements is true of the diffusion capacity


of carbon monoxide?
a) Depends on the thickness of the alveolar wall
b) Is a specific measure of lung perfusion
c) Is increased in cigarette smokers
d) Is increased in emphysema
e) Is not affected by changes in the surface area available for gas
exchange

Ans: Depends on the thickness of the alveolar wall

68) A 24-year-old man presents to the Emergency department and complains


of shortness of breath.
Before his chest x ray is taken he tells the casualty officer that he is known to
have an 'azygous lobe'.
In what region of the chest x ray would you expect to see an 'azygous lobe'?
a) Left lower zone
b) Left mid zone
c) Left upper zone
d) Right lower zone
e) Right upper zone

Ans: Right upper zone

Mediastinum

www.radiologymasterclass.co.uk

7) Which of the following is not true with regard to the radiological


appearance of a chest x ray?
a) Consolidation of the left lower lobe will elevate the left hemidiaphragm
b) Consolidation of the lingular lobe will obliterate the aortic knuckle and
pulmonary trunk in the PA view
c) Consolidation of the right middle lobe will extend to the right horizontal
transverse fissure and the right heart border in PA view.
d) Collapse of the right upper lobe displaces the horizontal fissure in the
PA view
e) The oblique fissures can only be seen on the lateral chest film

nswer: Consolidation of the left lower lobe will elevate the left hemidiaphragm

23) A 25-year-old woman is admitted with a four month history of cough


productive of mucoid sputum streaked with bright red blood, wheezing and
diarrhoea.
Her chest and abdominal examination are normal.
Which of the following investigations is the most discriminatory?
a) Bronchoscopy
b) Chest x ray
c) Computed tomography (CT) of chest
d) Echocardiogram
e) Ventilation-perfusion scan

Ans: Bronchoscopy

25) A 19-year-old woman became breathless while travelling on an


aeroplane.
Which one of the following features most strongly supports a
diagnosis of acute hyperventilation related to a panic disorder?
a) Carpal spasm
b) Finger paraesthesia
c) Hypotension
d) Light-headedness
e) Loss of consciousness

Ans: Carpal spasm

44) Which of the following is found in subjects acclimatised to life at high


altitudes?
a) Increased mean corpuscular haemoglobin concentration
b) Increased pulmonary artery pressure
c) Periodic respiration
d) Increased airway resistance
e) Reduced cardiac output

Ans: Increased pulmonary artery pressure

Several individuals are at 13,000 feet in the Andes in La Paz,


Bolivia. Which one is undergoing acclimatization to the
altitude?
A) Kat: just arrived by jet; hyperventilates; develops respiratory
alkalosis; faints
B) Tony: been there 2 months; full hematological adaptation; normal
activities
C) Nayra: born there; large lung capacity; high hematological
response; normal activities
D) David: beginning 5th day there; excreting bicarbonate; breathing
deeply; limited exercise

ANS : David: beginning 5th day there; excreting bicarbonate;


breathing deeply; limited exercise

LUNG FUNTION

Spirometry
DLCO
VO2(Exercise testing)
Methacholine challenge
(Bronchoprovocation tests)
Polysomnography
Overnight oximetry
ECG, 2-D Echo

Spirometry
Helpful in differentiating between obstructive and
restrictive lung disorders
Grading severity
Detecting early lung disease
Assess the effect of medication (reversibility)
Measure progress in disease treatment
As a part of pre aesthetic checkup

Parameters
FVC
FEV1
FEV1/FVC or FEV1/FVC
PEFR
FEF 25-75
VT, TLC, RV, FRC

Interpretation
FEV1/FVC < 80% - obstructive
FEV1/FVC < 80% & > 65% - Mild
obstruction
< 65% & > 50% - Moderate
< 50% & > 35% - Severe
< 35%

- Very severe

Interpretation
FEV1 is reduced due to decreased airflow, in turn due to
narrowed airways
FVC is also reduced, as gas is trapped behind the
narrowed bronchi
FEV1 < 1 lit is likely to have dyspnoea
FEV1 < 0.5 lit is likely to be breathless at rest & possibly
respiratory failure
FEV1 < 2 lit is unlikely to cause breathlessness due to
airway disease, except on vigorous excercise

Interpretation
FEV1/FVC > 100% Restrictive defect
Lung volumes to evaluate for Emphysema
PFT only gives a pattern
To be correlated by the physician

Spirometry

Spirometry

Spirometry

Spirometry
Characterizes the lesion:
A-Location of the lesion:
Intrathoracic
Extrathoracic
B-Behavior of the lesion during rapid inspiration and expiration:
Fixed
Variable
Variable Intrathoracic Lesion: Tracheomalacia & Intratracheal tumor.
Variable Extrathoracic Lesion: Vocal cord paralysis, Goiter, and Tumor
Intra or Extrathoracic Fixed Lesion: Tracheal stenosis & surgical stricture, and
compressing mass.

Spirometry

Spirometry

DLCO
DLCO measures the ability of the lungs to
transfer gas from inhaled air to the red blood
cells in pulmonary capillaries
The DLCO is designed to reflect properties of
the alveolar-capillary membrane
Strong affinity of Hb for CO, combined with
the enormous capacity of the red cell mass to
absorb CO, make the uptake of CO less
dependent on cardiac output.

Uses
DD of airways obstruction & lung volume
restriction
Screening for mild (early) interstitial lung
disease (ILD)
Detection of pulmonary vascular disease
Disability/impairment evaluations for ILD or
COPD
Follow-up for ILD/ Need for o2 therapy

62) A 55-year-old plumber presented with a dry nocturnal cough and


increasing exertional breathlessness.
On examination he had early finger clubbing, cyanosis and bilateral basal
crackles. A chest x ray showed bilateral lower zone shadowing.
Investigations revealed:
PaO2(breathing
8.2 kPa
(11.3-12.6)
air)
FEV1/FVC ratio
85%
Which of the following investigations is most likely to establish the
diagnosis?
a) Echocardiography
b) High resolution CT scan of chest
c) Measurement of diffusion capacity
d) Serum angiotensin-converting enzyme (ACE) level
e) Transbronchial lung biopsy

Ans: High resolution CT scan of chest

49) A 72-year-old lifelong smoker presents with progressive dyspnoea on


exertion. He has a chronic, non-productive cough.
On examination he is thin, breathing with pursed lips, respiratory rate
25/min, with mild wheezing on chest auscultation.
Investigations show
FEV1
0.8 L
FVC

1.6 L

pH

7.35

paCO2

6kPa (45mmHg)

paO2

7.2kPa (55mmHg)

What is the predominant mechanism of the airflow limitation in this


gentleman?
a) Bronchospasm
b) Foreign body obstruction
c) Increased airways resistance
d) Loss of elastic recoil
e) Mucus plugging in the small airways
Ans: Loss of elastic recoil

51) In restrictive lung disease due to respiratory muscle weakness,


which of the following statements is true?
a) Low FEV1/FVC, high RV/TLC
b) Low FEV1/FVC, normal TLC
c) Low VC, low FEV1, normal TLC, low RV/TLC
d) Low VC, low RV, low TLC
e) Low VC, low TLC, high RV/TLC

Ans: Low VC, low TLC, high RV/TLC

69) A 60-year-old female presents with recent onset dyspnoea and noisy
breathing.
Her chest x ray showed right deviation of the trachea due to a retrosternal
goitre.
Which of the following tests is most useful in the assessment of airflow
obstruction due to the goitre?
a) Flow volume curve
b) Forced expiratory flow volume in one second
c) Forced vital capacity
d) Peak expiratory flow rate
e) Residual volume

Ans: Flow volume curve

A 19-year-old man with recurrent admissions to hospital for exacerbations of his asthma attends
the Emergency department with a short history of increasing breathlessness and cough.
On examination he is obviously dyspnoeic and wheezy and becoming exhausted. His respiratory
rate is 16 breaths per minute, his HR is 125 bpm (sinus tachycardia) and his PEFR is 30%
predicted.
An arterial blood gas is taken and the results are as follows:
pH - 7.43 (7.36-7.44)
pO2 - 7.3 kPa (11.3-12.6)
pCO2 - 5.2 kPa (4.7-6.0)
Using the details above what severity is this patient's exacerbation?
A.
B.
C.
D.
E.

Acute severe exacerbation.


Life threatening exacerbation
Mild exacerbation
Moderate exacerbation
Near fatal exacerbation

ANS . Life threatening exacerbation

32) A 24-year-old asthmatic female is admitted with acute severe asthma.


Which of the following statements is correct?
a) A high inspired oxygen concentration should be used routinely
b) Agitation should be managed with a benzodiazepine
c) Inhaled salmeterol is indicated as first line therapy
d) Normal arterial pCO2 is reassuring
e) Pulsus paradoxus is a reliable sign of severity

Ans: A high inspired oxygen concentration should be used routinely

ASTHMA

Definition
Asthma is a chronic inflammatory
disease of the airways characterized
by
1)variable and recurring symptoms
2)reversible airflow obstruction
3)bronchospasm

Symptoms & Signs


Cough
Shortness of Breath
wheezing/chest tightness
Diurnal variability
Reversible/intermittent nature
Atopy
Nasal polyps
Skin atopy

Spirometry

FEV1 less than 80% predicted


Post-bronchodilator FEV1- more than 200
mL and the increase is more than 12 % of
the predicted value

Other tests

CXR
2-D ECHO
Skin atopy
IgE Rast to common allergens
Newer tests; exhaled nitric oxide,
breath condensate cytokines, and
analysis of induced sputum

Differential
Vocal Cord Dysfunction -involuntary closure
of the vc, during inspiration
Central airway obstruction- goitre, vascular
rings
COPD
Bronchiectasis
ABPA
Cardiac asthma
Pulmonary Embolism
Sarcoidosis
Allergic alveolitis
Eosinophillic syndromes

Exercise-induced asthma
Episodic bronchoconstriction following exercise
Prevalence varies from 7 to 20% in the general and
upto 80% in asthmatics
Minute ventilation rises with exercise
Triggered by the large volume of relatively cool & dry
air inhaled during vigorous activity.

Occupational asthma
Work place - immunologic or non-immunologic stimuli
Reactive airways dysfunction syndrome (RADS) or irritant-induced
asthma
5 to 25% of adult asthma
Low molecular wt. (Isocyanates) and high molecular wt. (flour)
antigens
Intensity of exposure, antigen property, host factors, such as atopy,
cigarette smoking, and genetic predisposition
Spirometry at work place and during the day-off

Rescue medications - Salbutamol, Ipratropium,


Formoterol
Preventer medications - Inhaled corticosteroids
(Beclomethasone/ Budesonide/ Fluticasone/
Mometasone/Ciclosenide)
SMART (Single/Symbicort maintenance and reliever
therapy): Formoterol+Budesonide

Stepwise Tx

Acute severe asthma


Early recognition and intervention are critical for
successful management of asthma exacerbations
Pulsus paradoxus (ie, a fall in systolic blood
pressure by at least 12 mmHg during inspiration)difficult
Use of accessory muscles of inspiration
Diaphoresis and inability to lie supine

Peak flow meter or spirometer is the best method for objective


assessment of the severity of an asthma attack
Peak flow - takes <1 min to perform, safe, inexpensive,
monitor a patient's response to tx
Peak flow below 200 L/min indicates severe obstruction for all
but unusually small adults
Marked hypoxaemia and hypercapnia (Pao2 <60 mm Hg,
oxygen saturation [SaO2] <90 %, Paco2 >50mm Hg) is
infrequent during asthma attacks and should raise alarm
CXR - Hyperinflation, pneumothorax, pneumomediastinum,
pneumonia, atelectasis are infrequent, occur in only about 2%
of CXRs

Tx of Acute Severe Asthma


Nebulised B2 agonists(Salbutamol)& anti-cholinergics
(Ipratropium)
Systemic Glucocorticoids: Oral Prednisolone 30-60mg; Inj
Methylprednisolone 40-125mg
Inj MgSo4 (2g over 20min)- decreases Ca++ influx into smooth
muscle
Helium-oxygen gas mixtures, IV leukotriene receptor
antagonists nebulized furosemide

38) Which one of the following is correct regarding long acting beta-2
agonists?
a) Are beneficial in acute viral croup.
b) Become less effective over time (tolerance).
c) Can be used to prevent activity-induced symptoms
d) Protect against allergen challenge for up to 48 hours.
e) Should not be used in association with erythromycin.

Ans: Can be used to prevent activity-induced symptoms

39) A 20-year-old male student is assessed for shortness of breath that


occurs whilst running.
He has no other symptoms and does not smoke. Examination, full blood
count, and chest x ray are normal.
Which of the following is most likely to be helpful in confirming the
suspected diagnosis?
a) Arterial blood gas studies before and after exercise
b) Determination of lung volumes and diffusing capacity
c) Measurement of venous blood lactate before and after exercise
d) Spirometry before and after administration of bronchodilators
e) Spirometry before and after exercise

Ans: Spirometry before and after exercise

60) A 15-year-old boy presented with wheezing when playing football and
nocturnal cough.
Which is the best test to confirm the underlying condition?
a) A trial of inhaled corticosteroids
b) A trial of inhaled salbutamol
c) A trial of oral corticosteroids
d) Serial peak expiratory flow rate measurements
e) Spirometry alone

Ans: A trial of inhaled salbutamol

19) A 55-year-old man who has a 25 year pack history of smoking


presents with a productive cough with mucoid sputum of two year
duration.
On examination he has scattered rhonchi and wheezing.
Which of the following is the likeliest diagnosis?
a) Bronchial asthma
b) Bronchiectasis
c) Chronic bronchitis
d) Fibrosing alveolitis
e) Pneumonitis

Ans: Chronic bronchitis

73) An elderly man with a history of asthma, congestive heart failure, and
peptic ulcer disease is admitted with bronchospasm and rapid atrial
fibrillation.
He is given nebulised salbutamol frequently, a loading dose of oral digoxin,
and oral prednisolone. His regular medications are continued. 24 hours after
admission his plasma potassium is noted to be 2.8 mmol/l.
Which of his medications is most likely to have caused this abnormality?
a) ACE inhibitor
b) Digoxin
c) Ranitidine
d) Salbutamol
e) Spironolactone

Ans: Salbutamol

COPD

Definition

GOLD Global initiative for chronic obstructive lung


disease

Airflow limitation that is not fully reversible. Its a


progressive abnormal inflammatory response of the
lungs bronchitis
to noxious
and gas.
Chronic
: Aparticles
chronic productive
cough for three months
in each of two successive years in a patient in whom other
causes of chronic cough have been excluded
Emphysema is defined by abnormal and permanent enlargement
of the airspaces that are distal to the terminal bronchioles.
There is destruction of the airspace walls, without fibrosis
Characteristic loss of elastic recoil, causing hyperinflation, and
air-trapping Barrel chest

CLINICAL PRESENTATION
History

Cough, Sputum and Exertional dyspnea

Physical Findings

Early stages Normal.

Severe disease prolonged expiratory phase and


wheezing.

Signs of hyperinflation - a barrel chest ; enlarged lung


volumes ; poor diaphragmatic excursion.

Sit in the characteristic "tripod" position to facilitate

Predominant emphysema
"pink puffers, thin and
noncyanotic at rest and have prominent use of accessory
muscles

Prominent chronic bronchitis bluebloaters , heavy and


cyanotic, due to corpulmonale and oedema.

paradoxical inward movement of the rib cage with inspiration


(Hoover's sign)

cor pulmonale, are relatively infrequent since


oxygen therapy.

Clubbing of the digits is not a sign of COPD, and its


presence should alert the clinician to initiate an investigation
for causes of clubbing.

supplemental

RISK FACTORS
Cigarette Smoking

FEV1 in a dose-response relationship to the intensity of


cigarette smoking

Airway Responsiveness and COPD

Dutch hypothesis asthma, chronic bronchitis, and


emphysema are variations of the same basic disease, which
is modulated by environmental and genetic factors
pathologically distinct entities

British hypothesis asthma and COPD are fundamentally


different diseases: Asthma is viewed as largely an allergic
phenomenon, while COPD results from smoking-related
inflammation and damage.

CXR
Characteristic features in lung parenchyma
Rapidly tapering vascular shadows
Increased radiolucency of the lung
Flat diaphragm
Long, narrow heart
Increased retrosternal airspace on a lateral
radiograph
Bullae are due to locally severe disease
Prominent hilar vascular shadows
Cardiac enlargement due to pulmonary hypertension
and cor pulmonale (secondary to hypoxia

CT
CT has greater sensitivity and specificity for
emphysema, but not chronic bronchitis
Centriacinar emphysema - preferentially in the
upper lobes
Panacinar emphysema - involves the lung
bases and involves the entire secondary
pulmonary lobule

Centri-acinar

Pan-lobular

Bullae

Plumonary Function Test

Interpretation in COPD
FEV1/FVC ratio less than 0.70

FEV1 less than 80 percent of predicted


Decreased IC and VC
Increased TLC, FRC and RV are indicative of
hyperinflation.
DLCO decreases in proportion to the severity of
emphysema

Gold Criteria for COPD Severity


GOLD
Stage

Severity

Symptoms

Spirometry

At Risk

Chronic cough, sputum production Normal

Mild

With or without chronic cough or


sputum production

FEV1/FVC <0.7 and


FEV1 80% predicted

IIA

Moderate

With or without chronic cough or


sputum production

FEV1/FVC <0.7 and


50% FEV1 <80%
predicted

III

Severe

With or without chronic cough or


sputum production

FEV1/FVC <0.7 and


30% FEV1 <50%
predicted

IV

Very
Severe

With or without chronic cough or


sputum production

FEV1/FVC <0.7 and


FEV1 <30% predicted
or

Management
The only intervention which has proven
benefit in arresting the disease is
smoking cessation
The fall in lung function
stabilises/plateaus when patient quits
smoking

Mx of Exacerbation
Controlled O2 inhalation (risk of CO2 retention secondary to lack
of hypoxic drive)
Antibiotics (sputum c/s), Steroids
Nebulised bronchodilators
Non-invasive ventilation (BiPAP)
Invasive mechanical ventilation

65) A 65-year-old man with known chronic obstructive pulmonary disease


(COPD), treated with inhalers, was admitted with a six week history of
gradually increasing shortness of breath.
He was apyrexial, mildly confused with a respiratory rate of 26 breaths per
minute and there were no changes on the chest x ray.
Investigations revealed:
PaO2
7.8kPa
(9-12.6)
PaCO2

8.5kPa

(4.7-6.0)

PH

7.3

(7.36-7.44)

What is the most appropriate immediate management?


a) High flow oxygen therapy
b) Intravenous aminophylline
c) Intravenous hydrocortisone
d) Intubation and mechanical ventilation.
e) Nebulised salbutamol and ipratropium bromide.

Ans: Nebulised salbutamol and ipratropium bromide.

21) In which of the following have randomised controlled trials shown that
long term oxygen therapy (LTOT) reduces mortality?
a) Asthma
b) Cor pulmonale due to chronic airflow obstruction
c) Cryptogenic fibrosing alveolitis
d) Cystic fibrosis
e) Pulmonary sarcoidosis

Ans: Cor pulmonale due to chronic airflow obstruction

43) Which one of the following is correct regarding severe bullous


emphysema?
a) Helium dilution is more accurate than body plethysmography in
measuring residual volume.
b) Hypoxaemia at rest will improve with exercise.
c) Pulmonary compliance is reduced.
d) Reduced elastic recoil opposes airway collapse in expiration.
e) The carbon monoxide transfer factor is reduced.

Ans: The carbon monoxide transfer factor is reduced.

41) A 65-year-old woman, has smoked 50 cigarettes a day for 40 years.


She has had increasing dyspnoea for the several years, but no cough. A
chest x ray shows increased lung size along with flattening of the
diaphragms, consistent with emphysema.
Over the next several years she develops worsening peripheral oedema.
Her vital signs show T 36.7 C, P 80, RR 15, and BP 120/80 mm Hg.
Which of the following cardiac findings is most likely to be present?
a) Constrictive pericarditis
b) Left ventricular (LV) aneurysm
c) Mitral valve stenosis
d) Non-bacterial thrombotic endocarditis
e) Right ventricular hypertrophy

Ans: Right ventricular hypertrophy

35) Which of the following statements is true regarding smoking in


pregnancy?
a) Dysmorphic facies is a recognised complication
b) Maternal smoking may adversely affect testicular function in male
children
c) Smoking reduces maturation of the fetal lung
d) The newborn baby may require adjustments in drug dosages because of
it
e) The reduction in birth weight is related to the number of cigarettes
smoked per day

The reduction in birth weight is related to the number of cigarettes smoked per

Bronchiectasis

Definition

Abnormal and permanent dilatation of one or


more bronchi

Etio-pathogenesis

Congenital
Acquired

Congenital
Ciliary dyskinetic syndrome
Young syndrome
Cystic fibrosis
William campbell syndrome (Bronchomalacia)
Mounier-kuhn syndrome
Swyer James syndrome
Yellow nail syndrome

Genetics of cystic fibrosis

Autosomal recessive with mutations in CFTRgene


located on chromosome 7.

Five major classes :

Classes IIII "severe, pancreatic insufficiency


+ high sweat NaCl

Class IV, V "mild, pancreatic sufficiency +


intermediate/normal sweat NaCl

The prevalence of CF varies with the ethnic origin of a


population.
Caucasians>african americans> asians

Acquired

Viral infections (Measles)


Tuberculosis
Mycobacteri Other Than TB
Bacterial & fungal infections
ABPA
Foreign body aspiration
ILD (traction)

Types

Cylindrical
Follicular
Cystic
Wet & Dry (Bronchiectasis sicca)

Clinical features
Persistent cough with mucopurulent sputum
Haemoptysis, sometimes massive (bronchial arterial
bleed)
SOB
Wheeze
Pleuritic pain
Weight loss/fever/weakness/tiredness
Clubbing
Cyanosis & plethora
Mid-inspiratory crackles (localized)
Severe case with cor pulmonale & RVF

Spirometry

Obstruction
Restrictive defect
Combined

Special tests
Sweat chloride test for CF
Genetic analysis for CF
Saccharin test for ciliary dyskinesia
Electron microscopy of sperm tail & cilia
Peripheral eosinophillia
Serum IgE, Aspergillus precipitins

Radiology
CXR;
1) Sensitivity < 50%
2) Evidence of dilated bronchi
3) Appearance consistent with volume
loss
4) Ring Shadow (End-on view),
5) Signet ring appearance
6) Parallel line (Tram track appearance)

CT

Gold standard
HRCT
1 to 1.5 mm cuts
Better localisation
Replaced bronchography

Treatment

Infection control (Antibiotics) after


sputum c/s
Postural drainage, Chest physio

Antibiotics
Check for Pseudomonas colonisation
Gram ve cover (Ceftazidime, Ciprofloxacin)
Gram +ve cover (Amox+clavulanic acid)
Prolonged course; 2 weeks, preferably IV
Nebulised Tobramycin for prevention and long term
Cyclical antibiotics (3 antibiotics, alternating every
month)

Mucus clearance (N-acetylcysteine, aerosolized


recombinant D-Nase - dornase alpha, a
deoxyribonuclease enzyme)
Steroids
Anti-fungals
Nebulised bronchodilators
Immunisation (Pneumococcus, Haemophillus,
Influenza)

Surgery

Localised disease
Destroyed lobe
Haemoptysis (recurrent and massive)
Colonisation with resistant organisms like MAI
complex

40) The parents of a child with cystic fibrosis (CF) consult you wishing to
know what is the risk of their next child being a carrier of the condition.
Which one of the following percentages is the correct risk?
a) 0%
b) 25%
c) 50%
d) 75%
e) 100%

Ans: 50%

9) A 16-year-old boy with cystic fibrosis presents with


abdominal pain.
Which of the following is most likely to be the cause?
a) Irritable bowel syndrome
b) Meconium ileus equivalent syndrome
c) Pyelonephritis
d) Renal calculi
e) Ulcerative colitis

Answer: Meconium ileus equivalent syndrome

61) A 68-year-old man presents with a one month history of dyspnoea and a 3
kg weight loss.
On examination there were signs of a large left pleural effusion, confirmed on
chest x ray.
Pleural fluid analysis revealed:
Protein

38 g/L

Cytology

a few lymphocytes and red blood


cells

Which one of the following investigations should be considered next?


a) Bronchoscopy
b) CT scan of thorax
c) Repeat pleural aspiration with biopsy
d) Thoracoscopic pleural biopsy
e) Tuberculin test

Ans: CT scan of thorax

Exudate (local disease)


(High protein).

Malignancy Lung, breast,


pleural.

Infection Pneumonia,
empyema, pleuritis, viral disease

Autoimmune Rheumatoid,
SLE

Vascular PTE

Cardiac Pericarditis,
CABG

Respiratory Haemothorax,
Chylothorax

Abdominal Subphrenic
abscess
Transudate (systemic illness)
(Low protein <30g).

Cardiac CCF, PTE

Liver Ascites,
Cirrhosis

Renal
Glomerulonephritis,
Nephrotic syndrome

Ovarian Meigs
syndrome

63) A 60-year-old man was admitted with community-acquired


pneumonia and deteriorated over the next few hours.
Which one of the following indicates a poor prognosis?
a) A total white cell count of 17 x 109/L (4-11)
b) Blood pressure of 110/70 mm Hg
c) Respiratory rate of 35 breaths/min
d) Rigors
e) Temperature of 39C

Ans: Respiratory rate of 35 breaths/min

CURB-65 score
C: confusion present (abbreviated mental test score <8/10)
U: (plasma) urea level >7 mmol/L
R: respiratory rate >30/min
B: systolic BP <90 mmHg; diastolic BP <60 mmHg
65: age >65
1 point for each of the above:
Score 01: Treat as outpatient
Score 2: Admit to hospital
Score 3+: Often require ICU care
Mortality rates increase with increasing score.
Other markers of severe community- acquired pneumonia
Chest X-ray: more than one lobe involved

PaO2 <8 kPa

Low albumin (<35 g/L)

White cell count (<4 109/L or >20 109/L)

Blood culture positive

Other co-morbidities

Absence of fever in the elderly

Community-acquired pneumonia : caused by bacterial or viral pathogens, most commonly


Streptococcus pneumoniae
Atypical pathogens : (Legionella pneumophila, Mycoplasma pneumoniae and Chlamydia spp.)
are atypical because :
cause more systemic symptoms
require serological testing rather than culture to confirm the diagnosis
do not respond to penicillins and require macrolide or tetracycline treatment
Legionella pneumophila is main cause of severe pneumonia.
Infections causing pneumonia trigger an inflammatory reaction, causing airspaces within affected
alveoli to become replaced with inflammatory exudate
Patients with pneumonia have symptoms of cough, haemoptysis, pleuritic chest pain, fever and
shortness of breath
Clinical signs of lobar pneumonia include dullness to percussion, crepitations and bronchial
breathing over the affected area of lung
The diagnosis of pneumonia requires demonstration of consolidation, usually on chest X-ray

Organisms implicated in hospital-acquired pneumonia


Gram-negative bacteria (Pseudomonas spp.,
Escherichia spp., Klebsiella spp.)

Anaerobic bacteria (Enterobacter spp.)

Staphylococcus aureus (including MRSA)


Acinetobacter spp.

Pharmacology of penicillins
Mechanism of action
Penicillins comprise a - lactam ring which binds this transpeptidase required for cross linking
peptidoglycans, thus inhibiting cell wall production.
Uses
Four groups:
1. Standard penicillin (e.g. benzyl penicillin, phenoxymethyl penicillin) : streptococcus ( pneumonia
and sore throat)
2. Antistaphylococcal penicillin (e.g. flucloxacillin): staphylococcal pneumonia.
3. Aminopenicillins (e.g. ampicillin, amoxicillin) : Broad spectrum (Gram-ve + Gram+ve). Used to treat
CA-LRTI/URTI (e.g. infective exacerbation of COPD, community-acquired pneumonia).
4. Antipseudomonal penicillins (e.g. piperacillin, azlocillin) : Broader spectrum with pseudomonas
included. (e.g. in patients with cystic fibrosis or with hospital-acquired pneumonia)
Pharmacokinetics
Water soluble; hence given intravenously.
. Rapidly excreted by the kidney, hence usually given at least 34 times daily.
Main side-effects
. Hypersensitivity ; Gastrointestinal upset
. Clostridium difficile diarrhoea (aminopenicillins)
. Rash in patients with EpsteinBarr virus infection (aminopenicillins)

Pharmacology of macrolide antibiotics (erythromycin, clarithromycin, azithromycin)


Mechanism of action
Inhibits protein synthesis by ribosomal binding and stop bacterial growth (bacteriostatic), allowing
the hosts immune system to clear the infection.
Uses
Community-acquired pneumonia caused by atypical organisms or in people allergic to penicillin.
Azithromycin or clarithromycin have better activity against H. influenzae and cause fewer
gastrointestinal side-effects.
Pharmacokinetics
Oral route is preferred. Can be given intravenously but erythromycin causes vein irritation
Metabolized by and inhibit the cytochrome p450 system in the liver.
Potential for drug interactions - particularly with warfarin (increased risk of bleeding) and
theophylline (risk of toxicity including arrhythmias and convulsions)
Main side-effects
Gastrointestinal upset
Hypersensitivity
Hepatitis
Reversible hearing loss

5) Which of the following statements is not true of primary pulmonary


tuberculosis?
a) A positive tuberculin skin test develops within two weeks of infection
b) It is characteristically asymptomatic
c) Miliary spread is commoner in a younger age group
d) Pleural effusion occurs before tuberculin skin testing is positive
e) The initial immunological response causes hilar lymphadenopathy

nswer: A positive tuberculin skin test develops within two weeks of infection

Primary tuberculosis
When M. tuberculosis reaches the pulmonary alveoli it:
Invades alveolar macrophages and replicates
Is taken up by (but does not replicate in) dendritic cells and is transported to local lymph nodes
A few bacteria may escape into the blood stream
Infection causes a local inflammatory reaction at infected sites and forms granuloma that contains the
infection. THIS IS PRIMARY INFECTION
Possible outcomes at this stage include:
Eradication of infection and healing, sometimes with calcification
Latent infection - immune system develops memory cells (2-10 weeks from Primary infection)
Failure of the immune process, resulting in widespread dissemination throughout the body, causing
miliary TB
In the lung, the site of primary infection is the periphery of the lung (subpleural) in the mid or upper
zones.
Ghons focus - The small area of granulomatous inflammation seen on chest X-ray
Ghons complex - conjunction with hilar lymphadenopathy.
A patient with primary TB is usually not unwell and may not know they have been infected until they
have a chest X-ray or develop postprimary TB. They are not infectious.

Reactivation tuberculosis
The majority of TB cases are due to reactivation
of latent infection.
The initial contact usually occurred many years or
decades earlier.
In patients with HIV infection newly acquired TB
infection is also common.
Factors implicated in the reactivation of latent TB

HIV co-infection
Immunosuppressant therapy
Diabetes mellitus
End-stage chronic kidney disease
Malnutrition
Ageing

Diagnosis of Active TB

Diagnosis of Latent TB
Tuberculin skin test (TST)
A positive result is indicated by a delayed hypersensitivity reaction evident 4872 hours after the
intradermal injection of purified protein derivative (PPD) resulting in:
1. A raised indurated lesion >6 mm diameter in non- vaccinated adults
2. A raised indurated lesion >15 mm in BCG-vaccinated adults.
False negative (anergic) TSTs :Immunosuppression , HIV infection (CD4+ <200/mm3), sarcoidosis,
drugs (chemotherapy, anti-TNF therapy, steroids), extremes of age.
False positives : Cross-reactivity with non-tuberculous mycobacteria (NTM) and BCG vaccination.
Interferon-gamma release assays (IGRAs)
. IGRAs detect T-cell secretion of interferon-gamma (IFN-) following exposure to M tuberculosisspecific antigens (ESAT-6, CFP-10,).
. The test does not differentiate between active and latent infection.
. However, it is highly specific compared with the TST and has a similar or better sensitivity.

42) A 51-year-old businessman complains of dyspnoea on exertion. He


recently returned from a business trip to the USA.
He has distant heart sounds on auscultation of the chest. A chest radiograph
reveals that there is a thin rim of calcification surrounding the cardiac outline.
Which of the following conditions is most likely responsible for these findings?
a) Group B coxsackie virus
b) Metastatic carcinoma
c) Sarcoidosis
d) Tuberculosis
e) Uraemia

Ans: Tuberculosis

45) Which of the following statement is true of infections with Mycobacterium


tuberculosis?
a) A positive tuberculin test indicates active disease
b) In pregnant women treatment should not be given until after delivery
c) Lymph node positive disease requires longer treatment than pulmonary
disease
d) Non-sputum producing patients are non-infectious
e) Pyrazinamide has high activity against active extracellular organisms

Ans: Non-sputum producing patients are non-infectious

People who should be treated for latent TB infection (after excluding active
TB)
People aged 35 years with positive TST or IGRA
Healthcare workers with positive TST or IGRA
Patients commencing anti-TNF therapy with positive IGRA HIV-positive
people with positive IGRA
People with evidence of previous TB on chest X-ray and
inadequate treatment.

64) A 28-year-old man who had been diagnosed two weeks previously with
tuberculosis of the mediastinal lymph nodes and who had been started on
chemotherapy with rifampicin, isoniazid and pyrazinamide was admitted
because of the increasing dyspnoea and stridor.
Chest x ray showed compression of both main bronchi by carinal lymph node
enlargement.
What is the next step in management?
a) Mediastinoscopy and biopsy
b) Refer for tracheal stent insertion/tracheostomy
c) Refer for urgent CT scan of the mediastinum
d) Start corticosteroids
e) The addition of ethambutol

Ans: Start corticosteroids

The immune reconstitution inflammatory syndrome (IRIS)


AKA - immune reconstitution disease, immune reconstitution syndrome, or immune restoration
disease
The condition results from rapid restoration of pathogen-specific immune responses to opportunistic
infections, causing either the deterioration of a treated infection or the new presentation of a
previously subclinical infection.
Its a widely recognised phenomenon that can complicate antiretroviral therapy (ART), in HIV
associated tuberculous infections.
Management includes anti inflammatory drugs, short course of steroids.

66) A 28-year-old man had been treated for pulmonary tuberculosis with
rifampicin, isoniazid, pyrazinamide and ethambutol for four weeks.
Pre-treatment liver function tests (LFTs) were normal but his most recent
investigations revealed:
Serum total
98 mol/l (0-18)
bilirubin
Serum alanine
620u/l
(5-45)
aminotransferase
Serum aspartate
450 u/l
(5-45)
aminotransferase
Serum alkaline
720 u/l
(40-110)
phosphatase
Which one of the following is the most appropriate next step?
a) Stop all treatment
b) Stop ethambutol
c) Stop isoniazid
d) Stop pyrazinamide
e) Stop rifampicin
Ans: Stop all treatment

12) A 36-year-old man who is a known asthamatic complains of persistent


cough and shortness of breath, which is unresponsive to his normal inhaled
therapy.
A CXR shows fibrosis of both upper lobes.
What is the most likely diagnosis?
a) Allergic bronchopulmonary aspergillosis
b) Ankylosing spondylitis
c) Cystic fibrosis
d) Primary pulmonary hypertension
e) Systemic sclerosis

nswer: Allergic bronchopulmonary aspergillosis

Diseases caused by Aspergillus fumigatus


The small (5 mm) spores are readily inhaled.
They are present in the atmosphere throughout the year,
especially late autumn.
They can be grown from the sputum in up to 15% of
patients with asymptomatic chronic lung disease .
They are a cause of extrinsic asthma in atopic individuals.

Investigations :

Characteristics Eosinophilia and very high IgE levels (both


that specific to Aspergillus and nonspecific).
Skin-prick testing to protein allergens from A. fumigatus is
positive.
Sputum may show eosinophils and mycelia.
Precipitating antibodies in the serum are also positive.

Management :

Steroids for Asthamatic attacks


Antifungal - Itraconazole, voriconazole for non Aggressive
invasion, Ampho B for Invasive.
Surgery for Fungal ball/ Aspergilloma.

6) A 43-year-old man with asthma develops worsening breathlessness and


his full blood count has revealed an eosinophilia. A diagnosis of allergic
bronchopulmonary aspergillosis (ABPA) is suspected.
Which of the following statements is true with regard to this diagnosis?
a) Circulating IgG precipitins to Aspergillus fumigatus are positive
b) Pleural effusion is a complication
c) Recurrent haemoptysis is a characteristic feature
d) The CO transfer factor is unaffected
e) The immediate skin test to an extract of Aspergillus fumigatus is negative

Answer: Circulating IgG precipitins toAspergillus fumigatusare positive

13) A 22-year-old woman recently returned from a holiday in Malta was


admitted with a three day history of fever, generalised lymphadenopathy
and a macular rash over the trunk and legs.
Which of the following is the most likely diagnosis?
a) Actinomycosis
b) Familial Mediterranean fever
c) Infectious mononucleosis
d) Sarcoidosis
e) Tuberculosis

Ans: Infectious mononucleosis

LYMPHADENOPAT
HY

58) A 26-year-old man with a history of alcohol and drug abuse was
admitted with a 14 day history of fever, cough and fatigue.
He was emaciated. His temperature was 39.4C. Cervical and axillary
lymphadenopathy were present. Chest x ray revealed bilateral areas of
pulmonary shadowing.
Which of the following is the most likely diagnosis?
a) Alcoholic cardiomyopathy
b) Pneumococcal pneumonia
c) Pneumocystis pneumonia
d) Pulmonary tuberculosis
e) Tricuspid endocarditis

Ans: Pneumocystis pneumonia

Pneumocystis jiroveci
Pneumocystis pneumonia (PCP) is one of the most common opportunistic infections.
It affects Immunosuppressed - long-term corticosteroids, monoclonal antibody therapy, methotrexate
for autoimmune disease, post-solid organ transplantation or stem cell transplantation, HIV; those at
particular risk have CD4 counts <200/mm3.
Pneumocystis jiroveci is found in the air, and pneumonia arises from re-infection rather than
reactivation of persisting organisms acquired in childhood.
Clinically, the pneumonia with a high fever, breathlessness and dry cough, rapid desaturation on
exercise or exertion.
The typical CXR is of a diffuse bilateral alveolar and interstitial shadowing - butterfly pattern, Other
includes localized infiltration, nodules, cavitation , pneumothorax, or Photo negative X-ray .
Diagnosis should be confirmed by indirect immunofluorescence on induced sputum or
bronchoalveolar lavage fluid.
First-line treatment of PCP is with high-dose co-trimoxazole . Respiratory failure responds very
well to steroids.

46) Which of the following statements is true of psittacosis


(ornithosis)?
a) Infection responds rapidly to penicillin therapy
b) It does spread from person to person
c) It is more of a risk to children than to adults who are exposed to
birds
d) It is only a risk from contact with psittacines (parrots), not other
birds
e) It usually causes many polymorphs to be present in the sputum

Ans: It does spread from person to person

27) Which of the following is true concerning whooping cough (pertussis)?


a) Is a greater threat to children during the second six months of life, after
maternal antibody has declined, than during the first six months
b) Is associated with convulsions less frequently than is the case with other
febrile conditions
c) Is characteristically associated with a polymorph leucocytosis
d) The vaccine may lead to hemiplegia
e) Rapidly resolves with antibiotic treatment

Ans: The vaccine may lead to hemiplegia

14) A 45-year-old seaman presents with cough and fever.


A CXR demonstrates a cavitating lung lesion.
Which of the following is the most likely cause?
a) Amoebiasis
b) Brucellosis
c) Histoplasmosis
d) Sarcoidosis
e) Syphilis

Ans: Histoplasmosis

CAVITY IN LUNG
Cancer
Autoimmune (Wegeners, RA)
Vascular (septic emboli)
Infectious (Tb, Abscess)
Trauma
Young (bronchogenic cyst, laryngotracheal
papillomatosis)

Multiple thin-walled cavities


Pneumatocele + bullae
Infections (Tb, cocci)
Tumors (Squamous cell)
Cysts (bronchogenic, trauma)
Hydrocarbon ingestion

59) What is the most likely cause of upper lobe fibrosis on chest x ray?
a) Ankylosing spondylitis
b) Cryptogenic fibrosing alveolitis
c) Rheumatoid arthritis
d) Scleroderma
e) Systemic lupus erythematosus

Ans: Ankylosing spondylitis

APICAL LESIONS ON CXR

B roncho-pulmonary aspergillosis
R adiation fibrosis
E xtrinsic allergic alveolitis
A nkylosing spondylitis
S ilicosis
T uberculosis
X Histiocytosis-X

4) A 45-year-old man develops facial swelling and breathlessness.His chest x


ray reveals paratracheal lymphadenopathy.
Which of the following statements is most accurate regarding the superior
vena caval obstruction (SVCO)?
a) It may be associated with voice hoarseness
b) It is associated with Kussmaul's sign
c) The commonest symptom is stridor
d) The most common cause is squamous cell carcinoma
e) Treatment of choice is radiotherapy

Answer: It may be associated with voice hoarseness

Whatcausesahoarsevoice?
Soundismadeduringspeechbychangesinair
pressureinthelarynx,whichcausethevocalcords
tosnaptogether.Ahoarsevoicecanbecausedby
thefollowing.
Laryngealorvocalcordlesions

Laryngitis(e.g.viralinfection,
smoking,acidreflux)

Voiceoveruse

Inhaledsteroids

Vocalcordnodules

Laryngealcarcinoma

Damagetonervesthatsupplythelarynx
Lungcancer
Thyroidsurgery
Thyroidcancer
Dissectionofthethoracicaorta

1) Which of the following is not a recognised feature of Pancoast's tumour?


a) Erosion of the first rib
b) Ipsilateral Horner's syndrome
c) Pain in the arm radiating to the fourth and fifth fingers
d) Wasting of the dorsal interossei
e) Weakness of abduction at the shoulder

Ans: Weakness of abduction at the shoulder

Effects of direct local spread of lung cancer to adjacent structures

from the head and upper limbs to the heart. Causes facial congestion, upper limb oedema and headaches. (S

(left or right) . Causes hoarseness and ineffective breathy cough

on of the pupil of the eye on the affected side (miosis).Sunken eye (enophthalmos) and reduced sweating of t

wer brachial plexus (Pancoasts tumour)


s of the hand and pain in the arm and hand

esophagus, causing difficulty in swallowing

1. A 65-year-old man, with a history of smoking presents with chronic cough,


haemoptysis and weight loss. His chest x ray shows a cavitating lesion.
What is the likely diagnosis?
a) Adenocarcinoma
b) Alveolar cell carcinoma
c) Undifferentiated large cell carcinoma
d) Small cell carcinoma
e) Squamous cell carcinoma

Answer: Squamous cell carcinoma

Histological classification of lung cancer


Non-small cell lung cancer
Squamous cell (3545%) : derived from
bronchial epithelial cells
Adenocarcinoma (15%) : derived from
glandular tissue in the airway submucosa
Large cell carcinoma (10%)
Carcinoid (1%)
Bronchoalveolar cell carcinoma (rare)

Mostly localised
Adenocarcinoma and Large cell carcinoma
presents at peripheral nodule or masses
squamous-cell carcinoma is the most common
to cavitate, followed by adenocarcinoma and
large cell carcinoma.
Multiple cavitary lesions are rare,seen in
multifocal bronchoalveolar cell carcinoma.
Responds poorly to chemotherapy .
Surgery can be curative for patients with
localized disease

Small cell lung cancer


Derived from endocrine cells in the lung

Mostly disseminated
Typically central mass
Small cell carcinoma is never known to
cavitate.
Grows fast, therefore initially is responsive to
chemotherapy.
Metastasizes early, therefore surgery has no
role in the management of this condition

74) A 58-year-old man presents with weight loss and haemoptysis. He has
smoked most of his life.
On examination he is clubbed and has clinical evidence of right pleural
effusion. His serum calcium is 3.2 mM (2.2-2.6 mmol/l). A bone scan is normal.
From which of the following histological type of lung cancer is he most likely to
suffer?
a) Adenocarcinoma
b) Large cell carcinoma
c) Mesothelioma
d) Small cell carcinoma
e) Squamous cell carcinoma

Ans: Squamous cell carcinoma

Active cigarette smoking ,Overall, the risk of lung cancer is increased 13 times. A
person who has a 40 pack year smoking history over 20 years has a 6070 times
greater risk of lung cancer than a non-smoker.

Risk factors for lung cancer other than active cigarette smoking
Environmental smoke exposure (passive smoking)
Marijuana and cocaine smoking
Occupational carcinogens, including: asbestos arsenic chromium petroleum products and tar

Environmental carcinogens radon (gaseous decay product of uranium and radium


that emits alpha particles, may accumulate in homes built above granite)

Why Smoking ?
Bronchi are normally lined by cuboidal epithelium. If the epithelium is subjected to
repeated damage or irritation (e.g. by cigarette smoking) it may change its form to
squamous epithelium to withstand this (metaplasia). Early malignant changes
(dysplasia) may arise during this process which may progress to cancer (neoplasia)

55) A 48-year-old woman presents to her GP with Cushingoid facies and


hyperpigmentation of the skin on her face and chest. She has smoked 20
cigarettes per day for 30 years.
Examination reveals no gross abnormalities. Her chest x ray reveals a 2 cm
irregularly shaped mass in the right upper lobe, in proximity to the
mediastinum. A CT guided needle biopsy of the lung lesion is performed.
Which would be the most likely cytologic finding?
a) Adenocarcinoma
b) Benign bronchial adenoma
c) Bronchoalveolar cell carcinoma (BAC)
d) Small cell (oat cell) carcinoma
e) Squamous cell carcinoma

Ans: Small cell (oat cell) carcinoma

Small cell lung cancer arises from endocrine cells in the lung which are
members of the APUD system (amine precursor uptake and
decarboxylation).
Small cell tumours comprise 2030% of lung cancers and are rapidly
growing and highly malignant.
These tumours have usually metastasized, either overtly or occultly, at the
time of presentation.
As the tumour cells have an endocrine origin they often secrete hormones
which have systemic effects.

3) A 56-year-old woman is recently diagnosed with small cell carcinoma of the


lung.
Which of the following non-metastatic manifestations is she most likely to
develop?
a) Eaton-Lambert syndrome
b) Ectopic PTH-related peptide secretion
c) Erythema gyratum repens
d) Hypertrophic pulmonary osteoarthropathy (HPOA)
e) Myasthenia gravis

Answer: Eaton-Lambert syndrome

Paraneoplastic phenomena associated with lung cancer


Cachexia
Weight loss and wasting
Musculoskeletal and skin
Finger clubbing
Hypertrophic pulmonary osteoarthropathy with pain, tenderness and swelling over
affected bones
Acanthosis nigricans (localized hyperpigmentation)
Endocrine
Inappropriate ADH secretion
Ectopic adrenocorticotrophic hormone secretion
Hypercalcaemia caused by secretion of parathyroid hormone-like substance
Gynaecomastia resulting from secretion of human chorionic gonadotrophin
Neurological
EatonLambert syndrome (antibodies to voltage-gated calcium channels causing
proximal muscle weakness)
Peripheral neuropathies
Subacute cerebellar degeneration
Cortical degeneration
Polymyositis

47) A 60-year old man with a history of non-small cell lung cancer was treated
with a right lower lobectomy 12 months ago.
He had an chest and abdominal CT scan one month ago which revealed
hepatic mass lesions and hilar lymphadenopathy. He now presents with
malaise and fatigue.
Protein +++ His results show:Urinalysis
24 hour urine
2.7 g/24hr
protein
Serum urea
30 mmol/L
(2.5-7.5)
Serum creatinine

450 mol/L

(60-110)

A renal biopsy shows focal deposition of IgG and C3 with a granular pattern.
What is the most likely diagnosis?
a) Goodpasture's syndrome
b) Membranous glomerulonephritis
c) Minimal change glomerulonephritis
d) Nodular glomerulosclerosis
e) Rapidly progressive glomerulonephritis

Ans: Membranous glomerulonephritis

Paraneoplastic glomerulonephritis associated with solid tumors

Lien, Y.-H. H. & Lai, L.-W. (2010) Pathogenesis, diagnosis and management of paraneoplastic glomerulonephritis
Nat. Rev. Nephrol. doi:10.1038/nrneph.2010.171

15) A 55-year-old man presents with ataxia and bilateral gynaecomastia.


Which of the following is the most likely diagnosis?
a) Bronchial carcinoma
b) Hypereosinophilic syndrome
c) Klinefelter's syndrome
d) Long term treatment with cyclophosphamide for Wegener's granulomatosis
e) Long term treatment with oral steroids for chronic asthma

Ans: Bronchial carcinoma

56) A 65-year-old woman presented with increasing fatigue, dyspnoea and a


dry cough.
Her chest x ray shows an area of dense pneumonia-like consolidation in the
right lower lobe.
A course of antibiotics did not improve her symptoms or chest x ray.
Bronchioalveolar lavage (BAL) retrieved 'atypical' cells.
What is the most likely diagnosis?
a) Bronchioloalveolar cell carcinoma
b) Mycoplasma pneumonia
c) Pulmonary alveolar proteinosis
d) Pulmonary embolism with infarction
e) Sarcoidosis

Ans: Bronchioloalveolar cell carcinoma

67) A 73-year-old male smoker presents with haemoptysis of three weeks


duration.
Examination reveals left supraclavicular lymphadenopthy. A chest radiograph
reveals a left sided hilar mass.
Which of the following would be an appropriate next step in the investigation
of this patient?
a) Bronchoscopy
b) CT thorax and upper abdomen
c) Lymph node biopsy
d) PET scanning
e) Sputum cytology

Ans: CT thorax and upper abdomen

Distant Metastasis from lung cancer


Bone
pain, hypercalcaemia, pathological fractures, compression of adjacent neurological tissue
Liver
hepatomegaly, obstructive jaundice
Brain
headache, seizures, confusion, raised intracranial pressure, focal neurological deficit (e.g.
hemiplegia, hemianopia, depending on site of metastasis)
Adrenals
hypoadrenalism

2) A 75-year-old man with squamous cell carcinoma of the lung is thought to


have resectable disease.
Which of the following would be a contraindication to surgery?
a) Clubbing
b) Forced expiratory volume (FEV)1 of 0.75 L
c) His age of 75 years
d) Pleural effusion
e) Syndrome of Inappropriate ADH

Answer: Forced expiratory volume (FEV)1 of 0.75 L

Management of Lung Cancer


Cell type (NSCLC versus SCLC),
Stage of disease
Functional status of the patient
Non-Small Cell Lung Cancer
Stages IA, IB, IIA, IIB, and some IIIA:
Surgical resection for stages IA, IB, IIA, and IIB
Surgical resection with complete-mediastinal lymph node dissection and
neoadjuvant CRx for stage IIIA disease with minimal N2 involvement (discovered
at thoracotomy or mediastinoscopy)
Postoperative RT for patients for N2 disease
Curative potential RT for nonoperable patients

ypes of stage T3 tumors:

c resection with involved chest wall + RT


3) tumors: preoperative RT + CRx followed by en bloc resection with postoperative RT
<2 cm from carina) without mediastinal nodes: sleeve resection if possible preserving distal normal lun

y, clinically evident N2 disease (discovered preoperatively) and IIIB disease that can be inc
( if performance status and general medical condition are reasonable)
ollowed by RT,

IB disease with carinal invasion (T4) but without N2 involvement:


onectomy with tracheal sleeve resection with direct reanastomosis to contralateral mainstem bro

V and more advanced IIIB disease:


ocal sites
ambulatory patients;
ube drainage of large malignant pleural effusions
on of primary tumor and metastasis for isolated brain or adrenal metastases

Small Cell Lung Cancer

Limited stage (good performance status): combination CRx + concurrent chest


RT
Extensive stage (good performance status): combination CRx
Complete tumor responders (all stages): consider prophylactic cranial RT Poorperformance-status patients (all stages):
Modified-dose combination CRx Palliative RT

Broncholoalveolar or Adenocarcinoma with EGF Receptor Mutations


Gefitinib or erlotinib, inhibitors of EGF receptor kinase activity

53) In which of the following cases of lung cancer would surgical resection of
the tumour be a reasonable therapeutic option?
a) A 56-year-old woman with an adenocarcinoma of the right lung. CT scan
shows enlarged lymph nodes in the right and left hilum. PFTs show an FEV1
of 2.25 L. (55% predicted).
b) A 59-year-old man who is found at bronchoscopy to have a tumour in the
right mainstem bronchus extending to within 1 cm of the carina. Pulmonary
function tests (PFTs) show an FEV1 of 2.1 litres (65% of predicted normal).
c) A 62-year-old lady with a small peripheral mass who has elevated liver
enzymes and a computed tomography (CT) scan showing probable metastatic
deposits in the liver. Lung function tests show an FEV1 of 3.5 litres (80% of
predicted normal).
d) A 70-year-old man with a right lower lobe tumour 2 cm in diameter with no
evidence of regional adenopathy or distant spread of disease. Lung funcion
studies show an FEV1 of 0.8 litres (28% predicted).
e) A 71-year-old man with a 3 cm tumour obstructing the right lower lobe
bronchus. Lung function tests show an FEV1 of 2.1 L. (60% predicted).
Ans: A 71-year-old man with a 3 cm tumour obstructing the right lower lobe
bronchus. Lung function tests show an FEV1 of 2.1 L. (60% predicted).

54) Which of the following statements regarding prognosis in lung cancer is


true?
a) Combined modality therapy (chemotherapy, radiation therapy and surgery)
has improved overall lung cancer survival to 40% at five years.
b) Overall lung cancer survival is less than 15% at five years.
c) Patients undergoing radiation therapy have a five year survival of 40%.
d) Patients who qualify for surgery have a 50% five year survival.
e) With chemotherapy, overall survival in small cell (oat cell) carcinomas has
risen to 60% at five years.

ns: Overall lung cancer survival is less than 15% at five years.

Prognosis
Unfortunately, compared with some other types of cancer, the outlook for lung
cancer is not very good.
The statistics we have here are for all types of lung cancer in England and
Wales.
Overall, 30% will survive for 1 year or more after they are diagnosed.
Around 10% will survive for 5 years or more.
And about 5% will survive for 10 years or more after they are diagnosed.

8) A 40-year-old worker presents with wheezing and breathlessness which


seem to improve over weekends and holiday periods when he is not working.
To which of the following is he most likely to be exposed at work?
a) Aspergillus clavatus
b) Avian bloom
c) Exposure to spores of Actinomyces
d) Platinum salts
e) Work in the silver industry

Answer: Platinum salts

34) In asbestos related disorders which of the following statements is correct?


a) Basal fibrotic shadowing on CXR suggests coincidental idiopathic fibrosing
alveolitis
b) Increased incidence of primary lung cancer
c) Pleural effusion develops more than 20 years after causative asbestos
exposure
d) Pleural plaques are recognised precursors of mesothelioma
e) The risk of malignant mesothelioma is greatly increased in smokers
compared with non-smokers

Ans: Increased incidence of primary lung cancer

Occupations associated with asbestos


exposure

Pulmonary conditions caused by asbestos


exposure

Production and transport of asbestos fibres


Mining
Milling
Transport and unloading (e.g. dockworkers)

Pleural plaques :
These are benign calcified patches of pleural
thickening.Usually asymptomatic.

Manufacture of insulation products


Roofing Flooring Textiles Cements
Work with insulation products
Building/demolition
Shipping/aircraft/motor vehicles
manufacturers and mechanics
Firefighters asbestos dust may be released
from fires in insulated buildings
Other (e.g. people working with sheet metal,
electrics,
boilers, railways)

Asbestosis :
Mild to moderate symptomatic Inflammation and
fibrosis of the lung parenchyma ; Occurs 2030
years after exposure.
Lung cancer :
Asbestos exposure increases the risk of small
cell and non-small cell lung cancer by several
times in both smokers and non-smokers
Pleural mesothelioma :
Highly malignant Pleural tumour with Mean
survival from diagnosis is 814 months.
Mean time interval is 41 years

The diagnosis of mesothelioma is made by CT guided biopsy or by VATS as the pleural


involvement is path.
Very Poor prognosis.

16) A 36-year-old woman presents with dyspnoea, cough and fever.Crackles


are heard on auscultation of the lungs. Circulating precipitans to
Micropolyspora faeni are positive.
Which of the following is the most likely diagnosis?
a) Allergic bronchopulmonary aspergillosis
b) Brucellosis
c) Farmer's lung
d) Malt worker's lung
e) Pigeon fancier's lung

Ans: Farmer's lung

sensitivity pneumonitis
spread diffuse inflammatory reaction in both the small airways of the lung and the alveoli.
en triggers, like microbial spores contaminating vegetable matter (e.g. straw, hay, mushroom compost).
rette smokers have a lower risk of developing the disease due to decreased antibody reaction to the ant

lly fever, malaise, cough and shortness of breath come on several hours after exposure to the causative
e end- inspiratory crackles and wheezes throughout the chest.
res of idiopathic pulmonary fibrosis

28) Which of the following is a typical feature of farmer's lung?


a) Basal crackles
b) Eosinophilia
c) Haemoptysis
d) Increased pCO2
e) Positive serum paraproteins

Ans: Basal crackles

Investigations
Chest X-ray : Fluffy nodular shadows, streaky shadows, in the upper zones. Later, honeycomb lung occurs.
High-resolution CT : Reticular and nodular changes with ground glass opacity.
Lung function tests : Restrictive ventilatory defect with a decrease in carbon monoxide gas transfer.
Peripheral smear : PMN leucocyte count is raised in acute cases. Eosinophilia is not a feature.
Blood Antibodies : Poor specificity. Pigeon fanciers have precipitating IgG antibodies against pigeon protein
and droppings . Precipitating antibodies are evidence of exposure, not disease.
Bronchoalveolar lavage : Increased T lymphocytes and granulocytes.

22) A 55-year-old woman on treatment for longstanding rheumatoid


arthritis has recently become dyspnoeic on mild exertion and developed a
dry cough.
The oxygen saturation was found to be 87% on air. The chest x ray
showed a diffuse bilateral interstitial infiltrate. An extensive infection
screen was negative and her symptoms were thought to be drug-induced.
Which drug is most likely to have caused this adverse effect?
a) Azathioprine
b) Cyclosporin
c) Hydroxychloroquine
d) Methotrexate
e) Sulfasalazine

Ans: Methotrexate

70) A 70-year-old man presented with increasing dyspnoea.


In his history he had suffered a myocardial infarction two years previously
which had been complicated by ventricular arrhythmias.
At admission his oxygen saturations were 85% on air and a chest x ray
revealed bilateral patchy infiltration of both lung fields with a cardiothoracic
ratio of 20/30 cm.
Which of the following drugs that he has been prescribed is most likely to
explain these findings?
a) Amiodarone
b) Aspirin
c) Atorvastatin
d) Furosemide
e) Ramipril

Ans: Amiodarone

11) A patient with rheumatoid arthritis complains of progressive


breathlessness.
Which of the following is the most likely cause?
a) Asthma
b) Fibrosing alveolitis
c) Pulmonary embolus
d) Pulmonary eosinophilia
e) Pulmonary nodules

Answer: Fibrosing alveolitis

Diffuse parenchymal lung


disease
Non infectious

Hoarseness

( Pneumoconiosis - coal
worker, asbestosis,
silicosis + Rheumatoid
lung disease )

Asymptomat
ic

Pleural adhesions, thickening


and effusions are the most
common lesions.
The effusion is often unilateral and
tends to be chronic.
It has a low glucose content but this
is not specific.
Progressive
breathlessness

26) Which of the following statements concerning industrial lung disorders is


correct?
a) Occupational asthma occurs more frequently in atopic persons
b) Pneumoconiosis can be diagnosed in the absence of chest x ray
abnormalities
c) Silo filler's disease is caused by allergy to grain
d) Widespread crepitations are typically heard in extrinsic allergic alveolitis
(EEA)
e) Symptoms occur within minutes of exposure to mouldy hay in farmer's lung

Ans: Occupational asthma occurs more frequently in atopic persons

is means the accumulation of dust in the lungs and the reaction of the tissue to its presence. It is main

plant material is ensiled, it begins to ferment. Oxygen used in fermentation combines with nitrates in the

Extrinsic Allergic Alveolitis aka Hypersensitivity pneumonitis. Predominantly involve apices, but
other areas are also involved, causing wide spread end inspiratory crackles and wheeze.

Typically fever, malaise, cough and shortness of breath come on several hours after exposure to
the causative antigen. Thus, a farmer forking hay in the morning may notice symptoms during the
late afternoon and evening with resolution by the following morning.
It is more common for the individuals to trigger asthma from inhalation of antigens from a variety
of mites that infest stored grain and other vegetable material.

30) A 45-year-old female presents with a six month history of exertional


dyspnoea and is diagnosed with pulmonary fibrosis (PF).
Over the last one year she has received a variety of medications.
Which of the following drugs could be responsible?
a) Dexamethasone
b) Ibuprofen
c) Nalidixic acid
d) Penicillamine
e) Sulphasalazine

Ans: Sulphasalazine

31) Which of the following statements regarding cryptogenic fibrosing


alveolitis (CFA) is correct?
a) 80% of patients initially respond well to immunosuppression
b) Active inflammation may be suggested by a CT scan
c) Lung volumes show a raised residual volume/total lung capacity ratio
d) Peak flow rate is a good guide to severity
e) Peak incidence seen in the fourth decade

ns: Active inflammation may be suggested by a CT scan

IPF / Usual interstitial pneumonia (UIP) / cryptogenic fibrosing alveolitis (CFA).


Mean onset is in the late 60s / males.
Unknown aetiology
Risk factors cigarette smoking, chronic aspiration, antidepressants, wood and metal dusts and
infections

There are patchy fibrosis of the interstitium


(often with intervening normal lung),
Subpleural and paraseptal changes,
Minimal or absent inflammation,
Acute fibroblastic proliferation and
Collagen deposition (fibroblastic foci) and
honeycombing.

Clinically progressive breathlessness, a non-productive cough and cyanosis, respiratory failure,


pulmonary hypertension and cor pulmonale.
Fine bilateral end-inspiratory crackles, gross finger clubbing.
An acute form known as the HammanRich syndrome occasionally occurs

Investigations
Chest X-ray - glass appearance, irregular reticulonodular shadowing (in the lower zones) and a
honeycomb.
High-resolution CT scan (HRCT) bilateral changes involving the lower lobes. Subpleural reticular
pattern, ground glass, honeycombing,thick-walled cysts 0.52 cm in diameter in terminal and
respiratory bronchioles, and traction bronchiectasis.
Respiratory function tests : Restrictive pattern with reduced FVC, the FEV1 to FVC ratio is normal
to high (with both values being reduced). DLCO reduced. PEFR may be normal.
Blood gases : hypoxaemia, with normal or low PaCO2 owing to hyperventilation.

Blood tests. ANA & RA are present in one-third of patients. ESR is mildly elevated.

Bronchoalveolar lavage : High counts with Neutrophilia and macrophages


Histological : excludes other conditions like sarcoidosis or lymphangitis carcinomatosa.
Diagnosis : Signs + characteristic HRCT changes.
Poor Prognosis
The median survival time : 5 years, mortality is very high in the more acute forms.

57) Progressive massive fibrosis (PMF) is most likely to be found in which of


the following?
a) Complicated silicosis
b) Extrinsic allergic alveolitis
c) Lobar pneumonia
d) Sarcoidosis
e) Simple coal worker's pneumoconiosis

Ans: Complicated silicosis

masses several centimetres in diameter, almost invariably in the upper lobes and sometimes having necrotic

es with fibrogenic promoting factor seems responsible.[like in Caplans syndrome].


ften positive.

ring from asbestosis or silicosis.

pical destruction and disruption of the lung, resulting in emphysema and airway damage.

w a mixed restrictive and obstructive ventilatory defect with loss of lung volume, irreversible airflow limitation a

ort dyspnoea, usually with a cough. The sputum may be black.

ss (or even develop) after exposure to coal dust has ceased and may lead to respiratory failure.

50) A 43-year-old Caribbean female comprehensive school teacher complains


of slowly increasing breathlessness. She has no smoking history.
Investigations reveal she has bilateral enlarged hilar lymph nodes, elevated
serum calcium, interstitial lung disease, and enlarged liver and spleen.
What is the most likely diagnosis?
a) Coccidioidomycosis
b) Hyperparathyroidism
c) Hypervitaminosis D
d) Sarcoidosis
e) Tuberculosis

Ans: Sarcoidosis

Sarcoidosis is a common disease of unknown aetiology that is often detected by routine chest X-ray.
The peak incidence is in the 3rd and 4th decades, with a female preponderance.

Immunopathology

Typical sarcoid granulomas consist of epithelioid cells, macrophages and lymphocytes, mainly T cells.
Tuberculin test is negative. Lymphopenia - Low T cells but slightly increased B cells
BAL shows increased cells with lymphocytes predominance
Transbronchial biopsies show infiltration of the alveolar walls and interstitial spaces
Its not a pre malignant state or immunosuppresed state.

Clinical features
Sarcoidosis can affect many different organs of the body.
Respiratory symptoms or abnormalities (without symptoms) found on chest X-ray (50%).
Fatigue or weight loss (5%), peripheral lymphadenopathy (5%) and fever (4%).
Stage I: bilateral hilar lymphadenopathy (BHL) alone
Stage II: BHL with pulmonary infiltrates
Stage III: pulmonary infiltrates without BHL
Stage IV: fibrosis.

Bilateral hilar lymphadenopathy


Characteristic feature,usually symptomless and only detected on chest X-ray
Pulmonary infiltration
Parenchymal sarcoidosis may be asymptomatic. CXR may be normal , but CT will always show involvement.
The combination of pulmonary infiltration and normal lung function tests is highly suggestive of sarcoidosis.
Extrapulmonary manifestations
Skin lesions : Most common (10%), as erythema nodosum ; With bilateral Hilar LNAP is always Sarcoid.
Eye lesions. Anterior uveitis Conjunctivitis , retinal lesions. Uveoparotid fever - bilateral uveitis and parotid
enlargement, occasionally, facial nerve palsy.
Keratoconjunctivitis sicca and lacrimal gland enlargement also occurs.
Metabolic manifestations : Hypercalcaemia (10%) and Hypercalciuria can cause Calculi and
nephrocalcinosis. The cause is an increased circulating 1,25-dihydroxyvitamin D 3, with 1 -hydroxylation
occurring in sarcoid macrophages in the lung in addition to that taking place in the kidney.
Bone and joint involvement. Arthralgia and bone cyst.

Investigations
Imaging. Chest X-ray. High-resolution CT .
Full blood count. mild normochromic, normocytic anaemia with raised ESR.
Serum biochemistry. High serum calcium and hypergammaglobulinaemia.
Transbronchial biopsy is positive in 90% of cases . Non-caseating granulomas in extrapulmonary.
Serum angiotensin-converting enzyme (ACE) level is raised in 75% of patients. Other d/d
lymphoma, pulmonary tuberculosis, asbestosis and silicosis.Assesses disease activity and response to
treatment.
Lung function tests show a restrictive lung defect .Decreased TLC, FEV 1 and FVC, and gas transfer.
Lung function is usually normal with extrapulmonary disease or only having hilar LNAP.
Treatment
1. No Treatment : Hilar LNAP, Infiltrates on CXR and Normal lung function. Just follow up 3-6 months.
2. Corticosteroids :
1. No Improvement for 6 months of follow up in above cases
2. Abnormal Lung function
3. Deteriorating lung function
4. Eye, Hypercalcemia, Eythema nodosum, Heart and CNS involvement
Prognosis
Mortality is 10%
Lung function and DLCO are best guide for the progress

An 18-year-old woman presents with red, tender lumps on her shins and arthralgia.
Chest x ray shows bilateral hilar lymphadenopathy and clear lung fields.
A clinical diagnosis of sarcoidosis is made.
Which one of the following is the most appropriate management plan?
a) 24 hour urinary calcium measurement
b) Follow up appointment with chest x ray in three months
c) Mediastinoscopy and lymph node biopsy
d) Skin biopsy
e )Thoracic CT scan

Ans : Follow up appointment with chest x ray in three months

A 60-year-old woman was referred to the rapid access lung cancer clinic after having a chest x
ray which she was told was abnormal. She reported feeling unwell for the past six weeks with
lethargy, intermittent fever and myalgia. She noticed that she became more breathless on
exertion and had difficulty climbing steep hills.
She had one episode of haemoptysis which she attributed to nose bleeds, which she was
experiencing frequently. She had a 40 pack/year smoking history and kept no pets at home. She
was a retired nurse and had worked in the NHS for 40 years.
On examination she looked unwell and had a temperature of 38.4C. Her blood pressure was
elevated at 160/100 mmHg, pulse 100 beats per minute and oxygen saturations of 94% on room
air. On palpation of the carotid pulse, she had marked tenderness of her right carotid artery.
There was no palpable lymphadenopathy in her cervical, axillary or inguinal regions. Her heart
sounds were normal with no audible murmurs. Respiratory examination revealed vesicular
breath sounds which were of normal intensity. There was no audible wheeze or crepitations.
Abdominal examination was normal.
A repeat chest radiograph revealed alveolar shadowing in the right upper lobe. The previous
chest radiograph, which she brought with her to clinic, showed alveolar shadowing in the right
mid-zone.

INVESTIGATIONS
Haemoglobin :
115 g/L (115-165)

Serum sodium
140 mmol/L
(137-144)

Serum urea
13.7 mmol/L
(2.5-7.5)

Anti-nuclear antibody
Positive
Perinuclear-ANCA
(pANCA)
Negative

White cell count :


10.0 109/L (4-11)

Serum potassium
4.2 mmol/L
(3.5-4.9)

Serum creatinine
170 mol/L
(60-110)

Cytoplasmic-ANCA
(cANCA)
Positive
Anti-GBM
Negative

Neutrophils 6.4 109/L (1.5-7) ; Lymphocytes 2.0 109/L (1.5-4.0); Monocytes 0.8 109/L
(0-0.8:Eosinophils 0.7 109/L (0.04-0.4) ;Basophils 0.1 109/L (0-0.1)
Platelets 500 109/L (150-400)
ESR (Westergren) 100 mm/1st hour (0-15)

A Heaf test showed a Grade 2 reaction.

A. Bronchoalveolar cell carcinoma


B. Bronchiolitis obliterans organising pneumonia
C. Goodpasture's syndrome
D. Lymphangitis carcinomatosa
E. Wegener's granulomatosis

ANS. Wegeners Granulomatosis

ms, clinical signs and laboratory findings are classical of Wegener's granulomatosis.
causing vasculitis (causing carotid artery tenderness), Wegener's is also a cause of migrating alveolar
SR, renal dysfunction and positive C-ANCA are also in keeping with the diagnosis.
also be raised in Wegener's granulomatosis.

Wegeners granulomatosis (granulomatosis with polyangiitis)


Unknown aetiology predominantly affects small arteries. It is characterized by lesions involving
the upper respiratory tract, lungs and kidneys.
Severe rhinorrhoea, mucosal ulceration, cough, haemoptysis and pleuritic pain.
Single or multiple nodular masses or pneumonic infiltrates with cavitation are seen on chest Xray. These appear to migrate, with large lesions clearing in one area and new lesions
appearing elsewhere.
The typical histological changes are usually best seen on renal biopsy, which shows
necrotizing microvascular glomerulonephritis.
This disease responds well to treatment with cyclophosphamide 150200 mg daily. Rituximab
is also being used.
A variant of Wegeners granulomatosis called midline granuloma affects the nose and
paranasal sinuses and is particularly mutilating; it has a poor prognosis.

and cough, which has been increasing over the last two weeks.
uating joint pains and had a couple of episodes of sinusitis over the last one year. He had no family histo
n 10 L oxygen.

Hb

128 g/L

(130 - 180)

Na+

136 mmol/L

(137 - 144)

WBC

11.5 109/L

(4 - 11)

K+

5.7 mmol/L

(3.5 - 4.9)

Platelets

452 109/L

(150 - 400)

Urea

42.7 mmol/L

(2.5 - 7.5)

CRP

56 mg/L

(< 10)

Creatinine

650 mol/L

(60 - 110)

Urinalysis

Protein ++

Blood +++

Nitrates negative

Leucocytes negative

Chest x ray
Anti-GBM antibodies

bilateral infiltrates, more


dense at the bases
negative

c-ANCA

positive

He deteriorates and is intubated on ITU and also received renal replacement therapy.
Given the most likely diagnosis, what course of treatment should be commenced?
A.
B.
C.
D.
E.

Cyclophosphamide and prednisolone


IV co-amoxiclav and clarithromycin
Plasmapheresis and prednisolone
Plasmapheresis, cyclophosphamide and prednisolone
Tamiflu

ANS . Plasmapheresis, cyclophosphamide and prednisolone


This patient has renal and pulmonary involvement.
Likely diagnosis , Wegener's granulomatosis.
Treatment is with IV cyclophosphamide and steroids.
However in severe life threatening Wegener's, or in patients with primarily renal involvement
needing dialysis, plasmapheresis is used to rapidly remove the immune complexes.
His respiratory symptoms are caused by pulmonary haemorrhage and although Goodpasture's
can present similarly the inclusion of his upper respiratory tract symptoms along with renal failure
leans more to a diagnosis of Wegener's.

Goodpastures syndrome
Its Autoimmune antibodies targeting lungs and kidneys.
Symptoms : URTI , Cough and intermittent haemoptysis, Anaemia, although massive bleeding may
occur.
The chest X-ray shows transient blotchy shadows that are due to intrapulmonary haemorrhage.
The features usually precede the development of an acute glomerulonephritis by several weeks or
months.
The course of the disease is variable: some patients spontaneously improve while others proceed to
renal failure.

It is due to a type II cytotoxic reaction driven by antibodies directed against the basement
membrane of both kidney and lung. It has been proposed that there is a shared antigen.
ANCA may be positive.
An association with influenza A2 virus has been reported.
Treatment is with corticosteroids.
Dramatic improvement has been seen with plasmapheresis to remove the autoantibodies.

33) A 57-year-old male is admitted with acute dyspnoea and chest pain. A
pulmonary embolism (PE) is confirmed.
Which of the following is a recognised feature of a significant pulmonary
embolism?
a) An arterial pH less than 7.2
b) An increase in serum troponin levels
c) Blood gases show increased pCO2 on air
d) Normal D-dimer levels
e) Reduced plasma lactate levels

Ans: An increase in serum troponin levels

Pulmonary embolism

DEFINITION : PE refers to obstruction of the pulmonary artery or one of its


branches by material (eg, thrombus, tumor, air, or fat) that originated elsewhere
in the body
Classification :
Acute or chronic
Massive or submassive

Saddle PE lodges at the bifurcation of the main pulmonary artery (submassive)

Massive PE :
SBP <90 mmHg or a drop by 40 mmHg for a period >15 minutes
Associated elevated JVP

NATURAL HISTORY

Untreated PE is associated with a mortality rate of approximately 30%


Recurrent embolism is the most common cause of death
Morbidity among untreated survivors is unknown, but appears to be high

Prognosis
Right ventricular (RV) dysfunction :
Two-fold increase in PE-related mortality
Brain natriuretic peptides :
Six-times increased mortality with BNP >100 pg/mL and 16-times with an NTproBNP >600 ng/L
DVT :
Coexisting DVT are at increased risk for mortality
Troponin :
Elevated levels are associated with an increased risk of death
RV thrombus & Hyponatraemia :
Increased mortality

Pathophysiology
Most PE arise deep venous system of the
lower extremities
Also originate in the right heart or the
pelvic, renal, or upper extremity veins
Iliofemoral veins are the source of most
clinically recognized PE (50-80%)
Calf vein thrombi - only 20 to 30 % cause PE,
others resolve spontaneously.
Smaller thrombi more likely to produce
pleuritic chest pain
Only 10% PE cause infarction

Risk factors

Immobility
Surgery within the last 3 months,
Stroke
Paraplegia
Hx of venous thromboembolism (Hypercoagulable
states)
Malignancy
Central venous instrumentation within the last
three months
Chronic heart disease
Obesity (BMI 29 kg/m2),
Heavy cigarette smoking (>25 cigarettes per day)
Hypertension

Presentation
Dyspnoea at rest or with exertion
(sudden)
Pleuritic chest pain
Cough
Orthopnea
Calf or thigh pain
Wheezing
Tachypnea, Tachycardia, Accentuated
P2 , jugular venous distension

Diagnosis
History
ABG - hypoxaemia and respiratory alkalosis
ECG - Sinus tachy, S1Q3T3 pattern, RV strain,
new incomplete RBBB, atrial arrythmias
Troponin (30-50%)
BNP - raised
2-D ECHO - Acute cor pulmonale, PAH
D-Dimer - Good sensitivity and NPV, but poor
specificity and PPV
High in infections, malignancy, recent
surgery

Imaging
CXR : Normal, plate-like atelectasis, oligaemia,
effusion
V/Q scan : Perfusion defects
Pulmonary angiogram : Gold standard
CTPA : 98% accuracy

CTPA

Treatment
Anti-coagulants (LMWH, Warfarin); 6
months or longer for recurrent PE
Thrombolysis, rarely
Prevention of DVT
IVC filter
Throboendarterectomy
Treat underlying cause

17) With which of the following is obstructive sleep apnoea


characteristically associated?
a) Hypersomnolence
b) Impotence
c) Insomnia
d) Macrognathia
e) Polydipsia

Ans: Hypersomnolence

Obstructive Sleep Apnoea


Partial Obstruction of Upper airway
leading to sleep disorder.
Cardinal features Obstructive apnoeas, hypopnoeas, or
respiratory effort related arousals.
Daytime sleepiness, fatigue, or poor
concentration
Disturbed sleep, snoring, restlessness,
or resuscitative snorts

Who is at risk?
Obesity
Craniofacial abnormalities
Upper airway soft tissue abnormalities
Potential risk factors include heredity, smoking,
nasal congestion, and diabetes

Severity
Based on Apnea Hyponea Index (AHI)
Mild : AHI between 5 and 15 respiratory events/hr of
sleep
Moderate : 15 and 30 respiratory events/hr of sleep
Severe : AHI greater >30 respiratory events/hr of
sleep
and/or a saturation below 90% for more than 20%
of the total sleep time.

Complications
Hypertension
CHF
Depression
Arrythmias, IHD, Death
Worsening asthma
Nasal congestion, Conjunctivitis

Treatment
Behavioural modifications (wt. reduction, alcohol, avoid
sedatives, drugs)
Positive airway pressure (CPAP, BiPAP)
Oral appliances (Mandibular advancement splints)
Surgery (UPPP, tonsillectomy, adenoidectomy, polyp and
sinus surgery, palatal advancement)

18) By which of the following is sleep apnoea syndrome best


diagnosed?
a) Blood gases during apnoeic episodes
b) EEG
c) Polygraphic sleep studies
d) Presence of HLA-DR2 and DQw1
e) Therapeutic trial of amphetamines

Ans: Polygraphic sleep studies

24) A 24-year-old male presents after developing a bluish discolouration of


the body, lips and nails. He denies any relevant past medical history.
Examination reveals a central cyanosis and a grey complexion.
Investigation revealed:

Haemoglobin

17.0 g/dl

(13.0-18.0)

PaO2

13.0 kPa

(11.3-12.6)

SaO2(using an

85%

(>95)

oximeter)
What is the most likely diagnosis?
a) Argyria
b) Cyanotic congenital heart disease
c) Haemochromatosis
d) Methaemoglobinaemia
e) Methylene blue poisoning

Ans: Methaemoglobinaemia

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