Rapidly Progressive Glomerulonephritis -A Review of 26 Cases- / 대한신장학회잡지

ABSTRACT

OBJECTIVES: Rapidly progres s ive glomerulonephritis (RPGN) is a clinico- pathologic entity characterized by extens ive crescent formation(usually involving 50% or more of glomeruli) as the principal his tologic finding and a rapid deterioration of kidney function, which can lead to end s tage renal disease within a few weeks. T he etiology and incidence of RPGN has been well defined in Europe and North America, however, there has been no report of a large series in Korea. T he aim of the present s tudy was to analyze the etiology and clinico- pathologic features of 26 patients with RPGN, seen during 1983-1997. METHODS: T wenty-six patients with RPGN(crescents in > 50% of glomeruli) were obs erved during a period of las t 14 years. Male to female ratio was 11.4, and the mean age was 30(6-75) years. Mean time from the initial symptoms to the ESRD was 3.1 months . RESULTS: The incidence of RPGN in our series was 2.1% of primary glomerulonephritis. Immunecomplex mediated disease was presented in 14 cases (54%), including 6 sys temic lupus erythematos us, 3 post- streptococcal glomerulonephritis, 3 Henoch- Schonlein purpura, and 2 IgA nephropathy. Pauci- immune disease was presented in 12 cases (46%), including 3 Wegener' s granulomatos is, one necrotizing crescentic glomerulonephritis, and 8 idiopathic crescentic glomerulonephritis. However, there was none of anti-GBM- mediated disease in our s tudy. ANCA were found in 6 patients. All 3 patients with WG were C- ANCA pos itive, whereas one patient with PSGN, necrotizing cres centic GN, and idiopathic crescentic GN were P- ANCA pos itive, respectively. Initial clinical and laboratory features included edema(80%), hypertens ion(72%), oliguria(68%), a decreased renal function(serum creatinine > 5mg/dL, 35%), and gros s hematuria(36%). Renal biopsy showed large crescents more than 80% of the glomeruli in 14 cases (54%) which were predominantly fibrocellular. Fifteen patients (58%) were treated with prednis olone alone, and 12 of them received puls e doses of corticosteroids. Five patients were treated with prednisolone and cyclophos phamide IV pulse. Two cases received plasma exchange. During the mean follow-up of 31+/-37 months, 18 patients (69%) developed inexorable progression of renal failure, three(12%) showed recovery of renal function, and two(8%) showed partial improvement, which is followed by varying degrees of renal insufficiency. During follow-up, three patients died two from res piratory failure with severe pulmonary hemorrhage and one from opportunistic pulmonary infection during immunosuppressive therapy. Poor prognos is is as sociated with hypertension, increased serum creatinine level at the time of diagnosis, large crescents more than 85% of glomeruli, and glomerular scleros is . CONCLUSION: We conclude that an earlier diagnos is including kidney biopsy and the more aggressive treatment are essential in the management of RPGN.