title: Long-term observations of clinicopathological characteristics and outcome of Japanese patients with pauci-immune crescentic glomerulonephritis.
abstract: 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.
  
  
  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.
  
  
  CONCLUSIONS
  Patients with CreGN were diagnosed in the early phase of crescent formation and outcome has improved in recent years.
authors.affiliation: Division of Blood Purification, Kanazawa University Hospital, Kanazawa, Japan.
authors.forename: Kiyoki
authors.initials: K
authors.lastname: Kitagawa
authors.forename: Kengo
authors.initials: K
authors.lastname: Furuichi
authors.forename: Yasuyuki
authors.initials: Y
authors.lastname: Shinozaki
authors.forename: Tadashi
authors.initials: T
authors.lastname: Toyama
authors.forename: Shinji
authors.initials: S
authors.lastname: Kitajima
authors.forename: Akinori
authors.initials: A
authors.lastname: Hara
authors.forename: Yasunori
authors.initials: Y
authors.lastname: Iwata
authors.forename: Norihiko
authors.initials: N
authors.lastname: Sakai
authors.forename: Shuichi
authors.initials: S
authors.lastname: Kaneko
authors.forename: Takashi
authors.initials: T
authors.lastname: Wada
mesh_terms: D000328:Adult; D000368:Aged; D005260:Female; D005921:Glomerulonephritis / Q000401:mortality / Q000473:pathology / Q000503:physiopathology*; D006801:Humans; D007564:Japan / Q000453:epidemiology; D007668:Kidney / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D015996:Survival Rate; D016896:Treatment Outcome