International Journal of Immunopathology and Pharmacology, Sep 1, 1996
The impact of immunologic changes in pregnancy of patients with connective tissue diseases (CTD) ... more The impact of immunologic changes in pregnancy of patients with connective tissue diseases (CTD) is of particular concern. Therefore, the interaction between these disorders and pregnancy should be carefully evaluated in each single patient. Among the relevant issues there are: effects of pregnancy on the course of maternal disease, effects of the disease and its treatment on fertility, pregnancy outcome, mode of delivery and breast feeding. The topic of CTD and pregnancy has become important as therapy has helped more patients to feel well enough to have a family, but it is extensive and controversial. Therefore, our presentation will be restricted at the experience of a Clinical Immunology Unit operating in a Department of Clinical Medicine. Immune mediated recurrent abortion and the antiphospholipids syndrome are treated in a separate section of this issue. Among the CTD, Rheumatoid Arthritis (RA) and Systemic Lupus Eriythematosus (SLE) have been studied extensively for the interrelationship with pregnancy. Experiences on pregnancy in patients with other CTD need to be confirmed in multicentric studies. Since the early uncontrolled studies, the effect of pregnancy on SLE has been a subject of controversy and contradiction. In the past, the absence of practical diagnostic techniques for patients with mild disease and absence of reasonable therapeutic modalities led to catastrophic events. Recent studies contradict the dogma of a higher risk of exacerbation during pregnancy, at least in the absence of active multisystem disease at conception. Whether flare rates increase during or after pregnancy is not yet clear because of variations in the characteristics of patients and in the definition offlare. Moreover, problems with discrimination between active SLE and pure pregnancy related abnormalities should be recognized. If only SLE-specific abnormalities are counted, the incidence of disease exacerbation is lower than when all possible abnormalities (i.e. knee effusions, hair loss, facial blush, etc.) are attributed to SLE.: In agreement with many studies, the course of SLE is unimpaired by subsequent pregnancy, low disease activity or a remission period of several months before pregnancy occurrs. Complications may be of significant clinical importance in patients with preexisting renal, CNS, or cardiac disease. In patients with renal involvement, an increase in daily proteinuria is observed during pregnancy, requiring postpartum intensification of immunosuppressive therapy
International Journal of Immunopathology and Pharmacology, 2012
Renal-limited vasculitis is a pauci-immune crescentic glomerulonephritis with no signs of systemi... more Renal-limited vasculitis is a pauci-immune crescentic glomerulonephritis with no signs of systemic involvement, representing one of the most common causes of rapidly progressive glomerulonephritis. The study aims to examine clinical and histological features in twenty-four patients with RLV diagnosed by the Nephrology Department of Sapienza University of Rome, Italy, evaluating the role of these parameters in predicting renal survival. Patients details, clinical and histological features and outcomes were recorded at the time of renal biopsy and over a mean follow-up period of 36±6 months. In our study, serum creatinine at presentation was significantly higher in patients who had a poor outcome than in those who survived with independent renal function (6.3±2.47 mg/dl vs 2.84±2.01 mg/dl, P= 0.002). The presence of C3c was found in the area of glomerular fibrinoid necrosis and in small arteries and arterioles with fibrinoid necrosis in 17 patients (P= 0.018). In conclusion, serum cre...
The objectives of this study were to investigate clitoral blood flow in SSc women compared with h... more The objectives of this study were to investigate clitoral blood flow in SSc women compared with healthy controls and to correlate it with microvascular damage and disease clinical variables. We also evaluated the correlation between clitoral blood flow and sexual dysfunction. Twenty-two SSc women and 20 healthy controls matched for sex and age were enrolled in this study. Baseline Doppler indices of the clitoral artery were measured. Peak systolic velocity, end diastolic velocity, resistive index (RI), pulsatile index (PI) and systolic/diastolic (S/D) ratio were measured. The female sexual function index (FSFI) was used to assess sexual function. The RI and S/D ratio were higher (P < 0.0001) in SSc women compared with healthy controls. The PI, RI and S/D ratio increased with progression of capillaroscopic damage. The RI and S/D ratio were higher (P < 0.01) in women with digital ulcers than in women without digital ulcers. No correlation exists between Doppler indices of the cl...
Erythrocyte glutathione transferase (e-GST) is a detoxifying enzyme hyper-expressed in nephropath... more Erythrocyte glutathione transferase (e-GST) is a detoxifying enzyme hyper-expressed in nephropathic patients and used recently as a biomarker for blood toxicity. Systemic sclerosis (SSc) is characterized by endothelial dysfunction and fibrosis of the skin and internal organs. Renal involvement is frequent in SSc patients. Here we show that e-GST is hyper-expressed in SSc patients (n = 102) and correlates (R(2) = 0.49, P < 0.0001) with the Medsger DSS and DAI Valentini indices that quantify the severity and activity of this disease. Interestingly, e-GST does not correlate with the impairment of kidney or other specific organs taken separately. e-GST hyper-expression seems to be linked to the presence of a factor (i.e., toxin) that triggers the autoimmune disease, and not to the damage of specific organs or to oxidative stress. e-GST may be proposed as an innovative non-antibody biomarker for SSc useful to check the progress of this disease and the efficiency of new therapeutic str...
Texas Heart Institute journal / from the Texas Heart Institute of St. Luke's Episcopal Hospital, Texas Children's Hospital, 2010
Cardiovascular involvement is the leading cause of morbidity and death in Churg-Strauss syndrome.... more Cardiovascular involvement is the leading cause of morbidity and death in Churg-Strauss syndrome. Herein, we describe the case of a 47-year-old man with Churg-Strauss syndrome, in whom the use of novel echocardiographic techniques revealed segmental cardiomyopathy. Tissue Doppler and speckle-tracking imaging showed that both longitudinal and radial strain were impaired at the septal level and that the impairment of circumferential strain affected left ventricular torsion. Our case shows that advanced echocardiography with myocardial strain imaging in multiple vectors can identify systolic-diastolic abnormalities in a patient with myocardial infiltration and a normal left ventricular ejection fraction.
Journal of biological regulators and homeostatic agents
Nickel (Ni) is the most common contact allergen among the general population in the industrialize... more Nickel (Ni) is the most common contact allergen among the general population in the industrialized world. Ni has been shown to exhibit immunomodulatory, if not immunotoxic, effects in several experiments conducted on humans and on rodents. This study tests the incidence of different infectious diseases in 100 patients with Ni hypersensitivity and compares it to data from 100 healthy volunteers. One hundred subjects with Ni hypersensitivity were enrolled. A group of 100 matched healthy volunteers with negative European standard patch test were enrolled as healthy controls. In patients with Ni hypersensitivity a higher incidence of recurrent herpes labialis (RHL), urinary tract infections (RUTI), genital candidiasis, and upper respiratory tract infections (RURTI) was detected. Fifteen patients with nickel allergic hypersensitivity (NAH) followed a Ni-poor diet. After a one-year diet a net reduction of incidence of RHL was found. Indeed, the number of episodes of RHL per year decreased...
The authors propose a microsurgical technique to treat upper limb chronic digital ischemia that i... more The authors propose a microsurgical technique to treat upper limb chronic digital ischemia that is resistant to medical therapy. The adventitiectomy technique on digital arteries is used here to remove the fibrous tissue and the vasoconstrictor sympathetic nervous fibers contained in it. This operation is a valid alternative to traditional proximal sympathectomy.
To determine whether inducible nitric oxide synthase (iNOS) stimulation of human atrial fragments... more To determine whether inducible nitric oxide synthase (iNOS) stimulation of human atrial fragments can be diminished by the naturally occurring signal molecules, such as morphine, anandamide, and estrogen. The use of iNOS as an indicator is justified since it has been associated with initiation of various types of cellular damage either directly or indirectly. Western blots were performed on control and drug-exposed atrial tissue before and after lipopolysaccharide (LPS) and interferon-gamma (IFN-gamma) exposure. Preincubation of the tissue with morphine, anandamide or estrogen prior to, but not after, the addition of LPS + IFN-gamma, blocked iNOS expression. The nitric oxide donor SNAP also blocked iNOS induction while preincubation of atrial fragments with an inhibitor of NOS, L-NAME, prior to morphine or anandamide exposure, restored LPS + IFN-gamma induction of iNOS. These data suggest a direct regulatory link at the transcriptional level between constitutive (c) NOS and iNOS in ...
To study those conditions with a proven or hypothesised immunologic pathogenesis and denominated ... more To study those conditions with a proven or hypothesised immunologic pathogenesis and denominated under a working definition of undifferentiated connective tissue diseases (UCTD). A multicentre prospective study was organised involving 10 tertiary referral centers of internal medicine in Italy, with the aim of describing the natural history of UCTD and the prevalence of its different clinical and immunological manifestations. After a five-year follow-up period, data on 165 patients were available for analysis. UCTDs occur mainly in females in their fourth decade of life. Articular and mucocutaneous features and Raynaud's phenomenon represent the most common findings. Nevertheless, we also detected a relatively high incidence of permanent major organ damage. Regarding the immunologic parameters, we documented some conflicting results in the correlation between serologic abnormalities and clinical features. In 10 patients UCTD evolved to a major disease, generally systemic lupus er...
To evaluate intrarenal arterial stiffness by Doppler ultrasound and examine the correlation betwe... more To evaluate intrarenal arterial stiffness by Doppler ultrasound and examine the correlation between renal Doppler indices, glomerular filtration rate, and digital microvascular damage in systemic sclerosis patients. Thirty systemic sclerosis patients and 30 healthy controls were enrolled in this study. Doppler indices of intrarenal arterial stiffness, peak systolic flow velocity, end diastolic flow velocity, resistive index, pulsative index, and systolic/diastolic (S/D) ratio were measured on the interlobar artery of both kidneys. Glomerular filtration rate was measured using Tc(99m) diethylenetriamine pentaacetic acid (DTPA). Equation 7 from the Modification of Diet in Renal Disease was used to estimate glomerular filtration rate. Nailfold videocapillaroscopy findings were classified as early, active, and late patterns. The intrarenal arterial stiffness, evaluated by Doppler indices, was higher in systemic sclerosis patients than healthy controls. In systemic sclerosis patients pulsative index (r = -0.69), resistive index (r = -0.75), and S/D ratio (r = -0.74) showed a negative correlation with measured glomerular filtration rate (P &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0001). High correlation (P = 0008) was observed between measured and estimated glomerular filtration rate (r = 0.55). Pulsative index, resistive index, and S/D ratio significantly increased with progression of capillaroscopic damage. Conversely, measured glomerular filtration rate significantly decreased with capillaroscopic damage progression. Doppler indices of intrarenal arterial stiffness are noninvasive diagnostic tests to evaluate renal damage in SSc patients. Intrarenal arterial stiffness and glomerular filtration rate correlate with capillaroscopic microvascular damage.
The incidence of left ventricular (LV) diastolic dysfunction is increased in systemic sclerosis (... more The incidence of left ventricular (LV) diastolic dysfunction is increased in systemic sclerosis (SSc), while systolic dysfunction is present in a small percentage of patients. The aim of this study was to asses the LV &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;quot;regional&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;quot; diastolic abnormalities in SSc patients by the mean of Doppler tissue imaging (DTI). Echocardiographic echo-Doppler (DE) and DTI parameters were analyzed for 67 SSc patients: abnormal E/A ratio at DE was detected in 24, while abnormal e/a at DTI was observed in 41. A significant prevalence of DTI diastolic abnormalities in the segments reflecting longitudinal versus those reflecting radial LV motion was found. The segments of the basal regions of LV myocardium were significantly more involved than those of the middle portion. Linear correlation was observed between the extent of the diastolic abnormalities and the duration of disease. Longitudinal myocardial systolic velocities were significantly reduced in patients with abnormal e/a DTI.
Erectile dysfunction (ED) prevalence in men with systemic sclerosis (SSc) is high and can be cons... more Erectile dysfunction (ED) prevalence in men with systemic sclerosis (SSc) is high and can be considered a manifestation of endothelium damage. This study aims to correlate ED with endothelial dysfunction and digital vascular damage in SSc patients. The main outcome measures were erectile function, Doppler indices of cavernous arteries, flow-mediated dilation (FMD), capillaroscopic damage, skin perfusion, and digital pulsatility. In 23 SSc patients, erectile function was investigated using the International Index of Erectile Function-5 (IIEF-5). Doppler indices of cavernous arteries, peak systolic velocity (PSV), end-diastolic velocity (EDV), and resistive index (RI) were measured at the penoscrotal junction before and after pharmacostimulation with 20 mg prostaglandin E1. FMD and nailfold videocapillaroscopy (NVC) were used to evaluate endothelial dysfunction and capillaroscopic damage. Skin perfusion and digital sphygmic waves were measured by laser Doppler perfusion imaging (LDPI) and digital photoplethysmography (PPG), respectively. IIEF-5 score was reduced in 83% of patients. PSV was reduced in 96%, while EDV was increased in 48% of patients. A positive correlation was observed between IIEF-5 score and FMD (r = 0.71, P &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.0001) and between the former and PSV (r = 0.76, P &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.0001). A negative correlation was observed between EDV and FMD (r = -0.509, P &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.01). FMD and Doppler indices were reduced with capillaroscopic damage progression. A positive correlation exists between LDPI perfusion and IIEF-5 score (r = 0.743, P &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.0001) and between the former and PSV (r = 0.714, P &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.0001), while a negative correlation is observed between LDPI perfusion and EDV (r = -0.677, P &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.0001). A positive correlation exists between PPG pulsatility and IIEF-5 score (r = 0.752, P &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.0001) and between the former and PSV (r = 0.747, P &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.0001), while negative correlation is observed between PPG pulsatility and EDV (r = -0.689, P &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.0001). In SSc patients, ED correlates with endothelial dysfunction and digital microvascular damage.
International Journal of Immunopathology and Pharmacology, Sep 1, 1996
The impact of immunologic changes in pregnancy of patients with connective tissue diseases (CTD) ... more The impact of immunologic changes in pregnancy of patients with connective tissue diseases (CTD) is of particular concern. Therefore, the interaction between these disorders and pregnancy should be carefully evaluated in each single patient. Among the relevant issues there are: effects of pregnancy on the course of maternal disease, effects of the disease and its treatment on fertility, pregnancy outcome, mode of delivery and breast feeding. The topic of CTD and pregnancy has become important as therapy has helped more patients to feel well enough to have a family, but it is extensive and controversial. Therefore, our presentation will be restricted at the experience of a Clinical Immunology Unit operating in a Department of Clinical Medicine. Immune mediated recurrent abortion and the antiphospholipids syndrome are treated in a separate section of this issue. Among the CTD, Rheumatoid Arthritis (RA) and Systemic Lupus Eriythematosus (SLE) have been studied extensively for the interrelationship with pregnancy. Experiences on pregnancy in patients with other CTD need to be confirmed in multicentric studies. Since the early uncontrolled studies, the effect of pregnancy on SLE has been a subject of controversy and contradiction. In the past, the absence of practical diagnostic techniques for patients with mild disease and absence of reasonable therapeutic modalities led to catastrophic events. Recent studies contradict the dogma of a higher risk of exacerbation during pregnancy, at least in the absence of active multisystem disease at conception. Whether flare rates increase during or after pregnancy is not yet clear because of variations in the characteristics of patients and in the definition offlare. Moreover, problems with discrimination between active SLE and pure pregnancy related abnormalities should be recognized. If only SLE-specific abnormalities are counted, the incidence of disease exacerbation is lower than when all possible abnormalities (i.e. knee effusions, hair loss, facial blush, etc.) are attributed to SLE.: In agreement with many studies, the course of SLE is unimpaired by subsequent pregnancy, low disease activity or a remission period of several months before pregnancy occurrs. Complications may be of significant clinical importance in patients with preexisting renal, CNS, or cardiac disease. In patients with renal involvement, an increase in daily proteinuria is observed during pregnancy, requiring postpartum intensification of immunosuppressive therapy
International Journal of Immunopathology and Pharmacology, 2012
Renal-limited vasculitis is a pauci-immune crescentic glomerulonephritis with no signs of systemi... more Renal-limited vasculitis is a pauci-immune crescentic glomerulonephritis with no signs of systemic involvement, representing one of the most common causes of rapidly progressive glomerulonephritis. The study aims to examine clinical and histological features in twenty-four patients with RLV diagnosed by the Nephrology Department of Sapienza University of Rome, Italy, evaluating the role of these parameters in predicting renal survival. Patients details, clinical and histological features and outcomes were recorded at the time of renal biopsy and over a mean follow-up period of 36±6 months. In our study, serum creatinine at presentation was significantly higher in patients who had a poor outcome than in those who survived with independent renal function (6.3±2.47 mg/dl vs 2.84±2.01 mg/dl, P= 0.002). The presence of C3c was found in the area of glomerular fibrinoid necrosis and in small arteries and arterioles with fibrinoid necrosis in 17 patients (P= 0.018). In conclusion, serum cre...
The objectives of this study were to investigate clitoral blood flow in SSc women compared with h... more The objectives of this study were to investigate clitoral blood flow in SSc women compared with healthy controls and to correlate it with microvascular damage and disease clinical variables. We also evaluated the correlation between clitoral blood flow and sexual dysfunction. Twenty-two SSc women and 20 healthy controls matched for sex and age were enrolled in this study. Baseline Doppler indices of the clitoral artery were measured. Peak systolic velocity, end diastolic velocity, resistive index (RI), pulsatile index (PI) and systolic/diastolic (S/D) ratio were measured. The female sexual function index (FSFI) was used to assess sexual function. The RI and S/D ratio were higher (P < 0.0001) in SSc women compared with healthy controls. The PI, RI and S/D ratio increased with progression of capillaroscopic damage. The RI and S/D ratio were higher (P < 0.01) in women with digital ulcers than in women without digital ulcers. No correlation exists between Doppler indices of the cl...
Erythrocyte glutathione transferase (e-GST) is a detoxifying enzyme hyper-expressed in nephropath... more Erythrocyte glutathione transferase (e-GST) is a detoxifying enzyme hyper-expressed in nephropathic patients and used recently as a biomarker for blood toxicity. Systemic sclerosis (SSc) is characterized by endothelial dysfunction and fibrosis of the skin and internal organs. Renal involvement is frequent in SSc patients. Here we show that e-GST is hyper-expressed in SSc patients (n = 102) and correlates (R(2) = 0.49, P < 0.0001) with the Medsger DSS and DAI Valentini indices that quantify the severity and activity of this disease. Interestingly, e-GST does not correlate with the impairment of kidney or other specific organs taken separately. e-GST hyper-expression seems to be linked to the presence of a factor (i.e., toxin) that triggers the autoimmune disease, and not to the damage of specific organs or to oxidative stress. e-GST may be proposed as an innovative non-antibody biomarker for SSc useful to check the progress of this disease and the efficiency of new therapeutic str...
Texas Heart Institute journal / from the Texas Heart Institute of St. Luke's Episcopal Hospital, Texas Children's Hospital, 2010
Cardiovascular involvement is the leading cause of morbidity and death in Churg-Strauss syndrome.... more Cardiovascular involvement is the leading cause of morbidity and death in Churg-Strauss syndrome. Herein, we describe the case of a 47-year-old man with Churg-Strauss syndrome, in whom the use of novel echocardiographic techniques revealed segmental cardiomyopathy. Tissue Doppler and speckle-tracking imaging showed that both longitudinal and radial strain were impaired at the septal level and that the impairment of circumferential strain affected left ventricular torsion. Our case shows that advanced echocardiography with myocardial strain imaging in multiple vectors can identify systolic-diastolic abnormalities in a patient with myocardial infiltration and a normal left ventricular ejection fraction.
Journal of biological regulators and homeostatic agents
Nickel (Ni) is the most common contact allergen among the general population in the industrialize... more Nickel (Ni) is the most common contact allergen among the general population in the industrialized world. Ni has been shown to exhibit immunomodulatory, if not immunotoxic, effects in several experiments conducted on humans and on rodents. This study tests the incidence of different infectious diseases in 100 patients with Ni hypersensitivity and compares it to data from 100 healthy volunteers. One hundred subjects with Ni hypersensitivity were enrolled. A group of 100 matched healthy volunteers with negative European standard patch test were enrolled as healthy controls. In patients with Ni hypersensitivity a higher incidence of recurrent herpes labialis (RHL), urinary tract infections (RUTI), genital candidiasis, and upper respiratory tract infections (RURTI) was detected. Fifteen patients with nickel allergic hypersensitivity (NAH) followed a Ni-poor diet. After a one-year diet a net reduction of incidence of RHL was found. Indeed, the number of episodes of RHL per year decreased...
The authors propose a microsurgical technique to treat upper limb chronic digital ischemia that i... more The authors propose a microsurgical technique to treat upper limb chronic digital ischemia that is resistant to medical therapy. The adventitiectomy technique on digital arteries is used here to remove the fibrous tissue and the vasoconstrictor sympathetic nervous fibers contained in it. This operation is a valid alternative to traditional proximal sympathectomy.
To determine whether inducible nitric oxide synthase (iNOS) stimulation of human atrial fragments... more To determine whether inducible nitric oxide synthase (iNOS) stimulation of human atrial fragments can be diminished by the naturally occurring signal molecules, such as morphine, anandamide, and estrogen. The use of iNOS as an indicator is justified since it has been associated with initiation of various types of cellular damage either directly or indirectly. Western blots were performed on control and drug-exposed atrial tissue before and after lipopolysaccharide (LPS) and interferon-gamma (IFN-gamma) exposure. Preincubation of the tissue with morphine, anandamide or estrogen prior to, but not after, the addition of LPS + IFN-gamma, blocked iNOS expression. The nitric oxide donor SNAP also blocked iNOS induction while preincubation of atrial fragments with an inhibitor of NOS, L-NAME, prior to morphine or anandamide exposure, restored LPS + IFN-gamma induction of iNOS. These data suggest a direct regulatory link at the transcriptional level between constitutive (c) NOS and iNOS in ...
To study those conditions with a proven or hypothesised immunologic pathogenesis and denominated ... more To study those conditions with a proven or hypothesised immunologic pathogenesis and denominated under a working definition of undifferentiated connective tissue diseases (UCTD). A multicentre prospective study was organised involving 10 tertiary referral centers of internal medicine in Italy, with the aim of describing the natural history of UCTD and the prevalence of its different clinical and immunological manifestations. After a five-year follow-up period, data on 165 patients were available for analysis. UCTDs occur mainly in females in their fourth decade of life. Articular and mucocutaneous features and Raynaud's phenomenon represent the most common findings. Nevertheless, we also detected a relatively high incidence of permanent major organ damage. Regarding the immunologic parameters, we documented some conflicting results in the correlation between serologic abnormalities and clinical features. In 10 patients UCTD evolved to a major disease, generally systemic lupus er...
To evaluate intrarenal arterial stiffness by Doppler ultrasound and examine the correlation betwe... more To evaluate intrarenal arterial stiffness by Doppler ultrasound and examine the correlation between renal Doppler indices, glomerular filtration rate, and digital microvascular damage in systemic sclerosis patients. Thirty systemic sclerosis patients and 30 healthy controls were enrolled in this study. Doppler indices of intrarenal arterial stiffness, peak systolic flow velocity, end diastolic flow velocity, resistive index, pulsative index, and systolic/diastolic (S/D) ratio were measured on the interlobar artery of both kidneys. Glomerular filtration rate was measured using Tc(99m) diethylenetriamine pentaacetic acid (DTPA). Equation 7 from the Modification of Diet in Renal Disease was used to estimate glomerular filtration rate. Nailfold videocapillaroscopy findings were classified as early, active, and late patterns. The intrarenal arterial stiffness, evaluated by Doppler indices, was higher in systemic sclerosis patients than healthy controls. In systemic sclerosis patients pulsative index (r = -0.69), resistive index (r = -0.75), and S/D ratio (r = -0.74) showed a negative correlation with measured glomerular filtration rate (P &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0001). High correlation (P = 0008) was observed between measured and estimated glomerular filtration rate (r = 0.55). Pulsative index, resistive index, and S/D ratio significantly increased with progression of capillaroscopic damage. Conversely, measured glomerular filtration rate significantly decreased with capillaroscopic damage progression. Doppler indices of intrarenal arterial stiffness are noninvasive diagnostic tests to evaluate renal damage in SSc patients. Intrarenal arterial stiffness and glomerular filtration rate correlate with capillaroscopic microvascular damage.
The incidence of left ventricular (LV) diastolic dysfunction is increased in systemic sclerosis (... more The incidence of left ventricular (LV) diastolic dysfunction is increased in systemic sclerosis (SSc), while systolic dysfunction is present in a small percentage of patients. The aim of this study was to asses the LV &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;quot;regional&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;quot; diastolic abnormalities in SSc patients by the mean of Doppler tissue imaging (DTI). Echocardiographic echo-Doppler (DE) and DTI parameters were analyzed for 67 SSc patients: abnormal E/A ratio at DE was detected in 24, while abnormal e/a at DTI was observed in 41. A significant prevalence of DTI diastolic abnormalities in the segments reflecting longitudinal versus those reflecting radial LV motion was found. The segments of the basal regions of LV myocardium were significantly more involved than those of the middle portion. Linear correlation was observed between the extent of the diastolic abnormalities and the duration of disease. Longitudinal myocardial systolic velocities were significantly reduced in patients with abnormal e/a DTI.
Erectile dysfunction (ED) prevalence in men with systemic sclerosis (SSc) is high and can be cons... more Erectile dysfunction (ED) prevalence in men with systemic sclerosis (SSc) is high and can be considered a manifestation of endothelium damage. This study aims to correlate ED with endothelial dysfunction and digital vascular damage in SSc patients. The main outcome measures were erectile function, Doppler indices of cavernous arteries, flow-mediated dilation (FMD), capillaroscopic damage, skin perfusion, and digital pulsatility. In 23 SSc patients, erectile function was investigated using the International Index of Erectile Function-5 (IIEF-5). Doppler indices of cavernous arteries, peak systolic velocity (PSV), end-diastolic velocity (EDV), and resistive index (RI) were measured at the penoscrotal junction before and after pharmacostimulation with 20 mg prostaglandin E1. FMD and nailfold videocapillaroscopy (NVC) were used to evaluate endothelial dysfunction and capillaroscopic damage. Skin perfusion and digital sphygmic waves were measured by laser Doppler perfusion imaging (LDPI) and digital photoplethysmography (PPG), respectively. IIEF-5 score was reduced in 83% of patients. PSV was reduced in 96%, while EDV was increased in 48% of patients. A positive correlation was observed between IIEF-5 score and FMD (r = 0.71, P &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.0001) and between the former and PSV (r = 0.76, P &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.0001). A negative correlation was observed between EDV and FMD (r = -0.509, P &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.01). FMD and Doppler indices were reduced with capillaroscopic damage progression. A positive correlation exists between LDPI perfusion and IIEF-5 score (r = 0.743, P &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.0001) and between the former and PSV (r = 0.714, P &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.0001), while a negative correlation is observed between LDPI perfusion and EDV (r = -0.677, P &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.0001). A positive correlation exists between PPG pulsatility and IIEF-5 score (r = 0.752, P &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.0001) and between the former and PSV (r = 0.747, P &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.0001), while negative correlation is observed between PPG pulsatility and EDV (r = -0.689, P &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.0001). In SSc patients, ED correlates with endothelial dysfunction and digital microvascular damage.
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