BACKGROUND: This study was conducted to standardize treatment and determine patient and renal outcome in Japanese anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis/rapidly progressive glomerulonephritis (AAV/RPGN) patients, because the prognosis of AAV/RPGN patients in Japan had been poor compared with that of other countries. METHODS: The participants in this retrospective cohort study were 824 ANCA-positive RPGN patients, 705 of whom were only myeloperoxidase (MPO)-ANCA positive. RESULTS: Among the early-years cohort (group A; cases diagnosed between 1988 and 1998), patients frequently died due to opportunistic infection. Therefore, we recommended a reduced dose of prednisolone (oral prednisolone dose <0.8 mg/kg/day) with or without cyclophosphamide for initial treatment of Japanese RPGN patients. After this recommendation, 1-year survival of the patients improved: 75% in group A, 79% in group B (between 1999 and 2002), and 81% in group C (after 2003). During the entire observation period, average serum creatinine level at the start of treatment decreased, and improvement of 1-year renal survival was also found (72% in group A, 83% in group B, and 83% in group C), while the recurrence rate was significantly increased in group C (0.05/patient-year in group A, 0.07/patient-year in group B, and 0.13/patient-year in group C). CONCLUSIONS: Oral prednisolone dose <0.8 mg/kg/day with or without cyclophosphamide as an initial treatment could improve patient survival in older Japanese AAV/RPGN patients. However, maintenance treatment avoiding relapse should be established to improve renal outcomes.
BACKGROUND: This study was conducted to standardize treatment and determine patient and renal outcome in Japanese anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis/rapidly progressive glomerulonephritis (AAV/RPGN) patients, because the prognosis of AAV/RPGNpatients in Japan had been poor compared with that of other countries. METHODS: The participants in this retrospective cohort study were 824 ANCA-positive RPGNpatients, 705 of whom were only myeloperoxidase (MPO)-ANCA positive. RESULTS: Among the early-years cohort (group A; cases diagnosed between 1988 and 1998), patients frequently died due to opportunistic infection. Therefore, we recommended a reduced dose of prednisolone (oral prednisolone dose <0.8 mg/kg/day) with or without cyclophosphamide for initial treatment of Japanese RPGNpatients. After this recommendation, 1-year survival of the patients improved: 75% in group A, 79% in group B (between 1999 and 2002), and 81% in group C (after 2003). During the entire observation period, average serum creatinine level at the start of treatment decreased, and improvement of 1-year renal survival was also found (72% in group A, 83% in group B, and 83% in group C), while the recurrence rate was significantly increased in group C (0.05/patient-year in group A, 0.07/patient-year in group B, and 0.13/patient-year in group C). CONCLUSIONS: Oral prednisolone dose <0.8 mg/kg/day with or without cyclophosphamide as an initial treatment could improve patient survival in older Japanese AAV/RPGNpatients. However, maintenance treatment avoiding relapse should be established to improve renal outcomes.
Authors: Herbert A Hauer; Ingeborg M Bajema; Hans C van Houwelingen; Franco Ferrario; Laure-Hélène Noël; Rüdiger Waldherr; David R W Jayne; Niels Rasmussen; Jan A Bruijn; E Christiaan Hagen Journal: Kidney Int Date: 2002-01 Impact factor: 10.612
Authors: C F Franssen; C A Stegeman; C G Kallenberg; R O Gans; P E De Jong; S J Hoorntje; J W Tervaert Journal: Kidney Int Date: 2000-06 Impact factor: 10.612
Authors: David Jayne; Niels Rasmussen; Konrad Andrassy; Paul Bacon; Jan Willem Cohen Tervaert; Jolanta Dadoniené; Agneta Ekstrand; Gill Gaskin; Gina Gregorini; Kirsten de Groot; Wolfgang Gross; E Christiaan Hagen; Eduardo Mirapeix; Erna Pettersson; Carl Siegert; Alberto Sinico; Vladimir Tesar; Kerstin Westman; Charles Pusey Journal: N Engl J Med Date: 2003-07-03 Impact factor: 91.245