Background: The clinicopathological characteristics and outcome with pauci-immune crescentic glomerulonephritis (CreGN) are presumed to vary over time. We examined the characteristics and outcome of Japanese patients with CreGN according to the treatment periods.
Patients and methods: From 1968 to 2011, we examined a total of 102 patients diagnosed with pauci-immune CreGN by renal biopsy. The patients were divided into three groups according to the treatment periods-Group I (1968-1988, n = 18), Group II (1989-2001, n = 37; when the nationwide survey of rapidly progressive glomerulonephritis [RPGN] was performed in Japan), and Group III (2002-2011, n = 47; after publication of the Japanese guideline for RPGN).
Results: There were no significant differences in blood pressure, renal function or anti-neutrophil cytoplasmic antibody titer between groups. On the other hand, the rate of crescent formation and degree of interstitial inflammatory cell infiltration were decreased in Group III. Serum creatinine (<3.0, 3.0-6.0, ≥6.0 mg/dL) and crescent formation (<30, 30-50, 50-80, ≥80 %) were significant renal prognostic factors in Group III [serum creatinine: hazard ratio (HR) 4.79, 95 % confidence interval (CI) 1.43-16.1, P = 0.011; crescent formation: HR 2.86, 95 % CI 1.06-7.73, P = 0.039]. Furthermore, renal survival rate of patients with crescent formation <50 % and patient survival rate of patients with serum creatinine <3 mg/dL were improved in Group III.
Conclusion: Patients with CreGN were diagnosed in the early phase of crescent formation and outcome has improved in recent years.