Clinical characteristics of rapidly progressive glomerulonephritis / 대한내과학회지

ABSTRACT

BACKGROUND: Rapidly progressive glomerulonephritis (RPGN) is microscopically characterized by formation of crescents in more than 50% of glomeruli observed. The patients usually move on rapidly to renal failure and the prognosis is not favorable. But there was only a few study because of the rarity in incidence. METHODS: We reviewed and analyzed the records of 15 patients diagnosed as crescentic glomerulonephritis (CrGN) by renal biopsy from March 1990 to December 2003. RESULTS: Fifteen out of 1055 biopsy cases were CrGN including 6 (40%) of pauci-immune glomerulonephritis (PIGN) and 9 (60%) of immune complex glomerulonephritis (ICGN). Underlying diseases of PIGN were unknown 2, Wegener's granulomatosis 2, focal segmental glomerulosclerosis 1, and rectal cancer 1. For ICGN were IgA nephropathy 3, lupus nephritis class IV 3, Henoch-Schonlein purpura 2, and HBV-associtated membranoproliferative glomerulonephritis type I. The incidence of major manifestation in PIGN vs. ICGN was respectively hypertension 50% vs. 22.2%, nephrotic syndrome 50% vs. 88.9%, percents of crescents 73.9% vs. 57.3%. The levels of BUN (mg/dL) and serum creatinine (mg/dL) were higher in PIGN as 76.8 +/- 14.3 and 6.6 +/- 1.2 vs. 26.9 +/- 8.9 and 1.6 +/- 0.3 in ICGN. With methylprednisolone pulse, 5 out of 7 patients showed some improvement in their renal function. A case of Wegener's granulomatosis taken oral prednisolone and another case of lupus nephritis given cyclophosphamide pulse also had relatively favorable course. At the end of follow-up, the more crescents they had the higher creatinine level (r=0.711, p<0.01). CONCLUSION: RPGN manifested nephrotic syndrome commonly and many of them progressed to the chronic kidney disease or even developed end stage renal disease. But appropriate immunosuppre- ssive treatment could help to preserve renal function. When considering the proportion of crescentic glomeruli, it was related to the worse prognosis. It is necessary to make an effort to diagnose early and treat vigorously.