[Crescentic glomerulonephritis and cerebral vasculitis in the course of Henoch-Schonlein purpura].
Henoch-Schonlein purpura (HSP) is a systemic vasculitic disorder involving both arterioles and capillaries. Although mainly a disease of early childhood, it can occur at any age. HSP is typically recognized as a syndrome with four major components: rash, joint manifestations, abdominal symptoms and renal disease. It is usually a mild condition with a tendency to relapses and generally has a good prognosis. Occasionally, however, it takes on an aggressive course. Gastrointestinal involvement is potentially the most serious complication of HSP. It may mimic an abdominal emergency and in its severest form result in small bowel infarction and/or perforation. Renal manifestations range from asymptomatic haematuria and/or proteinuria through a nephrotic syndrome to progressive glomerulonephritis leading to end stage renal failure. Apart from the major components outlined above, HSP may affect almost every other bodily organ. Vasculitis involving the myocard, lungs (pulmonary haemorrhage), ureter (stenosing ureteritis) and nervous system have been reported. We describe a case of HSP in a 50 year old woman which was complicated by the development of necrotizing crescentic glomerulonephritis and a left hemiparesis due to cerebral vasculitis. Interestingly, this patient had first appeared at the age of 9 years with a nephrotic syndrome and had been diagnosed by renal biopsy at the age of 31 as IgA nephropathy (IgAN). On her current admission, steroid and immunosuppressive therapy resulted in an improvement of renal function and an almost complete disappearance of her neurologic deficit.
Korzets
Ze'ev
Z
Department of Nephrology, Meir Hospital, Kfar Saba, Israel.
Magen
Eva
E
Tetro
Jane
J
Bernheim
Joelle
J
Yaffe
Daniel
D
Bernheim
Jacques
J
D005260:Female; D005921:Glomerulonephritis / Q000175:diagnosis / Q000209:etiology* / Q000473:pathology; D006801:Humans; D011695:IgA Vasculitis / Q000150:complications* / Q000175:diagnosis / Q000473:pathology; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D010291:Paresis / Q000209:etiology; D020293:Vasculitis, Central Nervous System / Q000175:diagnosis / Q000209:etiology* / Q000473:pathology