A RARE CASE OF MICROSCOPIC POLYANGIITIS

Harshita Ganji*, Raghavendra Prasad and Prabhakar Kamarthy

ABSTRACT

INTRODUCTION: Microscopic polyangiitis (MPA) is vasculitis of small vessels. The incidence of MPA is approximately 1:100.000 per year. A dermato-pulmonary-renal syndrome is the feature of the disease. Lung hemorrhage is observed in 12 to 29% of the patients with MPA and is an important contributory factor to morbidity and mortality. CASE REPORT: A 32 year old male patient presented with history of decreased urine output since 4 days, generalised weakness since 1 week, blackish discoloration of tips of the fingers and toes. On examination, Gangrene is present over distal aspect of fingers and toes. Patient was started on hemodialysis in view of deranged blood urea and serum creatinine. Patient developed dyspnoea on 4th day. Chest ray was done and it showed bilateral pulmonary infiltrations. Perinuclear Anti-Neutrophil Cytoplasmic Antibodies (p-ANCA) specific to myeloperoxidase shows positive. Patient was started on methylprednisolone 125mg/kg/day. Patient had sudden onset of chest pain followed by cardiac arrest and death. CONCLUSION: A Young male presenting with above clinical scenario, vasculitis should be suspected. Usually it occurs in 50-70 years males, but occuring in a 32 yr old male is a rare presentation.

Keywords: Microscopic polyangiitis, Perinuclear Anti-Neutrophil Cytoplasmic Antibodies, methylprednisolone, vasculitis