Korean Journal of Nephrology 1994;13(2):327-332.
폐출혈 및 뇌출혈을 동반한 특발성 급속 진행성 사구체신염 1예
장미경 , 이영호 , 김난희 , 차대룡 , 권영주 , 조원용 , 김영규 , 원남희
Abstract
Rapidly progressive glomerulonephritis (RPGN) is one of the most calamitous renal disease which consists clinically of sudden and relentless deterioration in renal function within weeks to months and is associated with the pathologic finding of extensive extracapilary prolif- eration, ie, crescentic glomerulonephritis (CGN). RPGN is associated with multisystem disorder such as Wegeners granulomatosis and Henoch-Schonlein purpura, or with infectious disorder as bacterial endocar- ditis. In addition, it may occur in patients without a definite diagnosis of a systemic illness. However classifying the latter patients as vasculitis is hampered by the lack of tissue confirmation from the representative vasculitis lesion. In the past several years a dramatic improvement in the prognosis of RPGN as part of systemic vasculitis has been reported with different immunosuppressive regimens. Since the time interval between initial symptom and start of immunosuppressive therapy directly affect prognosis, early diagnosis is of crucial importance. Recently suggested that autoantibodies against myeloid lysosomal enzymes ie, P-ANCA were closely associated with vasculitis related and idiopathic crescentic glomerulonephritis. On this paper we are reporting a case of rapidly progressive glomerulonephtitis with pulomonary and cerebral hemorrhage in a 68 year old man and this finding support that the pauciimmune idiopathic crescentic glomerulonephritis is related to the spectrum of vascul itides.
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