A case of limited systemic sclerosis with p-ANCA, complicated by multiple cerebral hemorrhages.
ANCA associated vasculitides are a group of disorders characterized by the presence of necrotizing inflammation of blood vessels that involve venules, capillaries, arterioles, arteries, and veins. They are characterized by the presence of antineutrophil cytoplasmic antibodies (ANCA). ANCA associated vasculitis commonly affects the kidney producing proteinuria, hematuria and variable degrees of renal failure. Other organs involved include the lung, skin and peripheral nervous system. We present a patient with limited systemic sclerosis, who later developed pauci-immune glomerulonephritis with p-ANCA (antimyeloperoxidase) specificity and, whose disease was later complicated by cerebral vasculitis with multiple intracerebral hemorrhages.
Akkara Veetil
Bharath Manu
BM
Pennsylvania Hospital, Philadelphia, PA, 19107, USA. bharathmanu@yahoo.co.in
Schimmer
Barry M
BM
D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood / Q000276:immunology*; D002543:Cerebral Hemorrhage / Q000209:etiology*; D017809:Fatal Outcome; D005260:Female; D005921:Glomerulonephritis / Q000150:complications / Q000473:pathology; D006801:Humans; D008875:Middle Aged; D045745:Scleroderma, Limited / Q000150:complications* / Q000276:immunology; D020293:Vasculitis, Central Nervous System / Q000150:complications*
D019268:Antibodies, Antineutrophil Cytoplasmic