Introduction: PRES, as a complication of juvenile lupus, is rarely reported in the literature. In... more Introduction: PRES, as a complication of juvenile lupus, is rarely reported in the literature. In this study, six juvenile lupus patients admitted with diagnosis of PRES were assessed on the basis of clinical characteristics, imaging findings, disease activity status, treatment response and prognosis. Methodology: Six juvenile (≤ 16 years) lupus patients with a diagnosis of PRES were included. Demographic, clinical, and laboratory features and outcomes of all six patients were noted. Literature review was performed on PubMed search forum. Search terms in English included Juvenile SLE, Lupus and PRES. Result: The youngest patient was seven years old while the oldest was sixteen years. All patients had history of lupus nephritis , presented with seizure and hypertension. In imaging, four out of six patients had hyperintensities in atypical distribution suggesting atypical PRES. All the patients had significant clinical recovery with resolution of hyperintensities in five out of six patients on repeat imaging. Conclusion: Juvenile lupus with PRES is considered an unusual neurologic manifestation triggered by multiple factors. It can be stipulated that PRES in juvenile lupus cases often remain undiagnosed. Early suspicion and treatment institution with reversal of triggers can result in a favorable outcome in these patients.
ObjectivesDysregulation of interferon-alpha (IFN-α) is considered central to the immunological ab... more ObjectivesDysregulation of interferon-alpha (IFN-α) is considered central to the immunological abnormalities observed in SLE. Short-term mortality during high disease activity in lupus is up to 30%. Adenovirus vector-introduced IFN-α into a lupus-prone mouse causes the development of glomerulonephritis and death within weeks. We studied serum IFN-α as a biomarker of in-hospital mortality in patients of SLE with high disease activity.MethodsSerum IFN-α (ELISA) was measured in patients hospitalised for acute severe lupus in a tertiary care rheumatology unit in India and the levels were compared between survivors and non-survivors. Serum IFN-α was compared with traditional clinical and serological markers associated with disease activity to assess which better prognosticates survival.ResultsIn a cohort of 90 patients with a mean Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) of 19.3 (±5.5), the mean serum IFN-α was 88±144 pg/dL. Levels were undetectable in patients with i...
INTRODUCTION PVD, one of the major macrovascular complications of T2DM, usually begins at an earl... more INTRODUCTION PVD, one of the major macrovascular complications of T2DM, usually begins at an earlier age and remains subclinical for a long duration. So early detection of PVD will help to assess its true prevalence and hence prevention of overt manifestation. OBJECTIVES To assess the true prevalence of PVD in T2 DM from Eastern India and its correlation with clinical and biochemical parameters METHODS It was a cross sectional study with consecutive diabetes patients attending OPD from 2015 January to 2016 July. Each was subjected to meticulous history, measurement of ABPI by handheld Doppler and various laboratory parameters. Patients having known vascular disease due to other causes were excluded. RESULTS Out of 103 patients, PVD was present in around 25% cases with positive association with age, male gender, and high body weight. Hypertension, hyperglycemic state (PPBS, HbA1C), total cholesterol, triglyceride and LDL independently increases the risk of PVD. Though low levels of H...
Introduction IgG4RD overlapping with clinically overt autoimmune diseases (Rheumatoid arthritis (... more Introduction IgG4RD overlapping with clinically overt autoimmune diseases (Rheumatoid arthritis ( RA) or anti-phospholipid syndrome (APS) is rarely reported in the literature. Here we report a tale of two cases with known autoimmune diseases – RA and primary APS presented with IgG4-related lesions involving unusual sites – oesophagus and right atrium of the heart respectively Case – The first case was a known RA presented with dysphagia, imaging suggestive of encasing mass in oesophagus causing stricture , biopsy was suggestive of IgG4RD. The second patient was a known case of primary APS presented with breathlessness, imaging revealed right atrial mass with histopathology was suggestive of IgG4RD . Both patients had significant improvement after starting steroid. Discussion – A few cases of known RA presenting with IgG4 mass is reported in literature though RA patients are frequently found to have increased number of IgG4 in their sera. Primary APS with IgG4 lesion is extremely unc...
Blau syndrome ( BS ) , considered a rare pediatric autoinflammatory disease, is characterized by ... more Blau syndrome ( BS ) , considered a rare pediatric autoinflammatory disease, is characterized by a triad of granulomatous arthritis, dermatitis and uveitis . Here we present a tale of three families evaluated in our outpatient department over the last two years (2020-2022). More than one member of the family was affected with either skin, ophthalmological and joint involvement with either biopsy-proven granuloma or a genetic mutation at NOD2 gene suggesting the diagnosis of BS. The third family had a novel pathogenic mutation in the NOD2 gene with clinical features of BS
Introduction. SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome is a rare ... more Introduction. SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome is a rare autoinflammatory condition describing the constellation of inflammatory skin, bone, and joint manifestations which result in diagnostic difficulty and therapeutic challenge. Case. Here, we present a case of a young male diagnosed with SAPHO syndrome with osteoarticular and cutaneous involvement from an early age in his life. He suffered diagnostic challenges for a long time and was hence inadequately treated. He had minimal response to conventional DMARDs but showed excellent response to TNF inhibitor (adalimumab). Later, he defaulted treatment and presented with acute anterior uveitis which was also dramatically improved with adalimumab and tofacitinib although financial constraint was always an issue for the patient. Conclusion. The uniqueness of this case was that the patient had a multiorgan involvement including osteoarticular system, skin, and eye. Both TNFi (adalimumab) and JAKini...
Takayasu's arteritis is a chronic, progressive, granulomatous vasculitis involving large arte... more Takayasu's arteritis is a chronic, progressive, granulomatous vasculitis involving large arteries especially aorta and its branches. Hyperthyroidism is a condition which involves excess synthesis and secretion of thyroid hormones by the thyroid gland. A 36 year old female patient presented at the General Medicine Outdoor of a tertiary-care hospital situated in the Eastern part of India with clinical features and laboratory reports suggestive of hyperthyroidism. There was also history of ischemic stroke 8 years back leading to right sided weakness in this patient. On careful physical examination left sided radial, brachial, femoral and dorsalis pedis arterial pulses were found to be impalpable. Digital subtraction angiography of aorta and its branches revealed narrowing of left common carotid and left subclavian artery suggestive of Takayasu's arteritis. This existence of Takayasu's arteritis and hyperthyroidism may not be just fortuitous. An underlying autoimmune mechani...
Hepatitis E virus is one of the leading causes of acute liver failure in clinical situation espec... more Hepatitis E virus is one of the leading causes of acute liver failure in clinical situation especially in immune-compromised patients , it has a high incidence in Asian countries and many people died due to lack of proper treatment . In this study , steroid (prednisolone ) was used as therapeutic challenge in such patients and significant improvement in clinical and biochemical parameters were found ( SGPT , prothrombin time and total bilirubin ) . This observational study highlights the possibility of steroid treatment as a therapeutic rescue in patients with hepatitis E. Further studies are required to evaluate whether steroid treatment has a supportive role for patients with HEV induced acute liver injury and improves their spontaneous outcome Key word: hepatitis E , steroid (prednisolone ) , SGPT , Prothrombin time ------------------------------------------------------------------------------------------------------------------------------------Date of Submission: 22-01-2018 Dat...
Introduction: PRES, as a complication of juvenile lupus, is rarely reported in the literature. In... more Introduction: PRES, as a complication of juvenile lupus, is rarely reported in the literature. In this study, six juvenile lupus patients admitted with diagnosis of PRES were assessed on the basis of clinical characteristics, imaging findings, disease activity status, treatment response and prognosis. Methodology: Six juvenile (≤ 16 years) lupus patients with a diagnosis of PRES were included. Demographic, clinical, and laboratory features and outcomes of all six patients were noted. Literature review was performed on PubMed search forum. Search terms in English included Juvenile SLE, Lupus and PRES. Result: The youngest patient was seven years old while the oldest was sixteen years. All patients had history of lupus nephritis , presented with seizure and hypertension. In imaging, four out of six patients had hyperintensities in atypical distribution suggesting atypical PRES. All the patients had significant clinical recovery with resolution of hyperintensities in five out of six patients on repeat imaging. Conclusion: Juvenile lupus with PRES is considered an unusual neurologic manifestation triggered by multiple factors. It can be stipulated that PRES in juvenile lupus cases often remain undiagnosed. Early suspicion and treatment institution with reversal of triggers can result in a favorable outcome in these patients.
ObjectivesDysregulation of interferon-alpha (IFN-α) is considered central to the immunological ab... more ObjectivesDysregulation of interferon-alpha (IFN-α) is considered central to the immunological abnormalities observed in SLE. Short-term mortality during high disease activity in lupus is up to 30%. Adenovirus vector-introduced IFN-α into a lupus-prone mouse causes the development of glomerulonephritis and death within weeks. We studied serum IFN-α as a biomarker of in-hospital mortality in patients of SLE with high disease activity.MethodsSerum IFN-α (ELISA) was measured in patients hospitalised for acute severe lupus in a tertiary care rheumatology unit in India and the levels were compared between survivors and non-survivors. Serum IFN-α was compared with traditional clinical and serological markers associated with disease activity to assess which better prognosticates survival.ResultsIn a cohort of 90 patients with a mean Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) of 19.3 (±5.5), the mean serum IFN-α was 88±144 pg/dL. Levels were undetectable in patients with i...
INTRODUCTION PVD, one of the major macrovascular complications of T2DM, usually begins at an earl... more INTRODUCTION PVD, one of the major macrovascular complications of T2DM, usually begins at an earlier age and remains subclinical for a long duration. So early detection of PVD will help to assess its true prevalence and hence prevention of overt manifestation. OBJECTIVES To assess the true prevalence of PVD in T2 DM from Eastern India and its correlation with clinical and biochemical parameters METHODS It was a cross sectional study with consecutive diabetes patients attending OPD from 2015 January to 2016 July. Each was subjected to meticulous history, measurement of ABPI by handheld Doppler and various laboratory parameters. Patients having known vascular disease due to other causes were excluded. RESULTS Out of 103 patients, PVD was present in around 25% cases with positive association with age, male gender, and high body weight. Hypertension, hyperglycemic state (PPBS, HbA1C), total cholesterol, triglyceride and LDL independently increases the risk of PVD. Though low levels of H...
Introduction IgG4RD overlapping with clinically overt autoimmune diseases (Rheumatoid arthritis (... more Introduction IgG4RD overlapping with clinically overt autoimmune diseases (Rheumatoid arthritis ( RA) or anti-phospholipid syndrome (APS) is rarely reported in the literature. Here we report a tale of two cases with known autoimmune diseases – RA and primary APS presented with IgG4-related lesions involving unusual sites – oesophagus and right atrium of the heart respectively Case – The first case was a known RA presented with dysphagia, imaging suggestive of encasing mass in oesophagus causing stricture , biopsy was suggestive of IgG4RD. The second patient was a known case of primary APS presented with breathlessness, imaging revealed right atrial mass with histopathology was suggestive of IgG4RD . Both patients had significant improvement after starting steroid. Discussion – A few cases of known RA presenting with IgG4 mass is reported in literature though RA patients are frequently found to have increased number of IgG4 in their sera. Primary APS with IgG4 lesion is extremely unc...
Blau syndrome ( BS ) , considered a rare pediatric autoinflammatory disease, is characterized by ... more Blau syndrome ( BS ) , considered a rare pediatric autoinflammatory disease, is characterized by a triad of granulomatous arthritis, dermatitis and uveitis . Here we present a tale of three families evaluated in our outpatient department over the last two years (2020-2022). More than one member of the family was affected with either skin, ophthalmological and joint involvement with either biopsy-proven granuloma or a genetic mutation at NOD2 gene suggesting the diagnosis of BS. The third family had a novel pathogenic mutation in the NOD2 gene with clinical features of BS
Introduction. SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome is a rare ... more Introduction. SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome is a rare autoinflammatory condition describing the constellation of inflammatory skin, bone, and joint manifestations which result in diagnostic difficulty and therapeutic challenge. Case. Here, we present a case of a young male diagnosed with SAPHO syndrome with osteoarticular and cutaneous involvement from an early age in his life. He suffered diagnostic challenges for a long time and was hence inadequately treated. He had minimal response to conventional DMARDs but showed excellent response to TNF inhibitor (adalimumab). Later, he defaulted treatment and presented with acute anterior uveitis which was also dramatically improved with adalimumab and tofacitinib although financial constraint was always an issue for the patient. Conclusion. The uniqueness of this case was that the patient had a multiorgan involvement including osteoarticular system, skin, and eye. Both TNFi (adalimumab) and JAKini...
Takayasu's arteritis is a chronic, progressive, granulomatous vasculitis involving large arte... more Takayasu's arteritis is a chronic, progressive, granulomatous vasculitis involving large arteries especially aorta and its branches. Hyperthyroidism is a condition which involves excess synthesis and secretion of thyroid hormones by the thyroid gland. A 36 year old female patient presented at the General Medicine Outdoor of a tertiary-care hospital situated in the Eastern part of India with clinical features and laboratory reports suggestive of hyperthyroidism. There was also history of ischemic stroke 8 years back leading to right sided weakness in this patient. On careful physical examination left sided radial, brachial, femoral and dorsalis pedis arterial pulses were found to be impalpable. Digital subtraction angiography of aorta and its branches revealed narrowing of left common carotid and left subclavian artery suggestive of Takayasu's arteritis. This existence of Takayasu's arteritis and hyperthyroidism may not be just fortuitous. An underlying autoimmune mechani...
Hepatitis E virus is one of the leading causes of acute liver failure in clinical situation espec... more Hepatitis E virus is one of the leading causes of acute liver failure in clinical situation especially in immune-compromised patients , it has a high incidence in Asian countries and many people died due to lack of proper treatment . In this study , steroid (prednisolone ) was used as therapeutic challenge in such patients and significant improvement in clinical and biochemical parameters were found ( SGPT , prothrombin time and total bilirubin ) . This observational study highlights the possibility of steroid treatment as a therapeutic rescue in patients with hepatitis E. Further studies are required to evaluate whether steroid treatment has a supportive role for patients with HEV induced acute liver injury and improves their spontaneous outcome Key word: hepatitis E , steroid (prednisolone ) , SGPT , Prothrombin time ------------------------------------------------------------------------------------------------------------------------------------Date of Submission: 22-01-2018 Dat...
Objectives. Myositis-specific autoantibodies (MSAs) are increasingly used to delineate distinct s... more Objectives. Myositis-specific autoantibodies (MSAs) are increasingly used to delineate distinct subgroups of JDM. The aim of our study was to explore without a priori hypotheses whether MSAs are associated with distinct clinicalpathological changes and severity in a monocentric JDM cohort. Methods. Clinical, biological and histological findings from 23 JDM patients were assessed. Twenty-six histopathological parameters were subjected to multivariate analysis. Results. Autoantibodies included anti-NXP2 (9/23), anti-TIF1g (4/23), anti-MDA5 (2/23), no MSAs (8/23). Multivariate analysis yielded two histopathological clusters. Cluster 1 (n = 11) showed a more severe and ischaemic pattern than cluster 2 (n = 12) assessed by: total score severity 5 20 (100.0% vs 25.0%); visual analogic score 56 (100.0% vs 25.0%); the vascular domain score >1 (100.0% vs 41.7%); microinfarcts (100% vs 58.3%); ischaemic myofibrillary loss (focal punched-out vacuoles) (90.9 vs 25%); and obvious capillary loss (81.8% vs 16.7). Compared with cluster 2, patients in cluster 1 had strikingly more often anti-NXP2 antibodies (7/11 vs 2/12), more pronounced muscle weakness, more gastrointestinal involvement and required more aggressive treatment. Furthermore, patients with anti-NXP2 antibodies, mostly assigned in the first cluster, also displayed more severe muscular disease, requiring more aggressive treatment and having a lower remission rate during the follow-up period. Conclusion. Marked muscle ischaemic involvement and the presence of anti-NXP2 autoantibodies are associated with more severe forms of JDM.
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