Renal thrombotic microangiopathy and cerebral venous thrombosis in a young man.

We report a patient of primary catastrophic antiphospholipid syndrome who presented with rapidly progressive renal failure and seizures. He was detected to have thrombotic microangiopathy on kidney biopsy and deep cerebral venous thrombosis. The patient was successfully managed with anticoagulants, steroids, plasmapheresis and cyclophosphamide.

Prevalence of renal involvement in Indian patients with systemic sclerosis.

BACKGROUND: Renal involvement in systemic sclerosis (SSc) either in the form of scleroderma renal crisis (SRC) or nonrenal crisis abnormalities has been reported to be in the range of 60-80%. Renal involvement is thought to be rare in Indian patients with SSc. However, there is paucity of data. AIMS: To study the frequency and pattern of renal involvement in Indian patients with SSc. SETTINGS AND DESIGN: A single center prospective, cross sectional study. MATERIALS AND METHODS: We prospectively evaluated the patients with SSc attending the Rheumatology Clinic. All patients were evaluated for renal involvement. All patients underwent measurement of blood pressure, urine examination, glomerular filtration rate (GFR) estimation using Cockcroft-Gault formula and kidney biopsy when indicated. STATISTICAL ANALYSIS: Statistical analysis was performed using SAS 8.0 statistical package. RESULTS: Eighty-seven patients were included in the study from July 2001 to December 2004. Mean age of patients was 36.88 +/- 12.51 years. About 30% of patients had diffuse cutaneous SSc. None of these patients had SRC either at enrollment in the study or during follow-up. Eleven (12.6%) patients had hypertension. Six (6.9%) patients had abnormal urinary findings in the form of either active urinary sediment or significant proteinuria. Only one patient had azotemia (plasma creatinine > 1.8 mg/dl). Calculated GFR CONCLUSION: SRC is very rare in Indian patients with SSc. However, non-renal crisis abnormalities appear to be as common in Indian patients as compared to the western literature.

Sjogren's syndrome presenting with interstitial lung disease.

We report a patient of primary Sjogren's syndrome presenting with interstitial lung disease. The clinical picture was dominated by respiratory symptoms leading to a delay in diagnosis.

Emergencies in rheumatology.

Rheumatological conditions can sometimes present as emergencies. These can occur due to the disease process or may be iatrogenic. Some of the important articular emergencies are septic arthritis, acute polyarthritis and atlanto-axial dislocation. Classical polyarteritis nodosa may present with massive gastro-intestinal bleeding, intestinal perforation or acute pancreatitis. Adult respiratory distress syndrome, bilateral pneumonitis and diffuse alveolar haemorrhage due to systemic lupus erythematosus or systemic necrotising vasculitis and ventilatory failure due to polymyositis are some of the respiratory emergencies. Scleroderma is well known to cause renal crisis which can be fatal if not diagnosed and managed promptly. Microscopic polyangiitis and Wegener's granulomatosis may cause rapidly progressive renal failure. Cerebrovascular accident, cortical vein thrombosis, seizures and acute psychosis are important neurological complications of rheumatic disease. Cardiac emergencies include tamponade, acute myocarditis and acute myocardial infarction. Vision can be threatened in Behcet's disease, temporal arteritis and seronegative spondylarthritis. Catastrophic antiphospholipid syndrome is a devastating emergency. The management of above emergencies includes critical care, immunosuppression when indicated and withdrawal of the offending drug. Anticoagulants have to be used in the management of antiphospholipid syndrome. A good understanding of these conditions is of paramount importance for proper management.