Outcome of ANCA-associated XXXX : a 5-year retrospective study.

@entity1 @entity8 @entity295 @entity1333 @entity12534 @entity6129 @entity1431 @entity2883 @entity1011 @entity204 @entity7134 @entity454
BACKGROUND: Renal involvement is frequently present in antineutrophil cytoplasmic autoantibody (ANCA)-associated systemic @entity1333 and is an important cause of @entity454 ( @entity454 ). METHODS: This retrospective, multicenter, sequential cohort study reports presenting features and outcome of 246 new @entity1 diagnosed in London, UK, between 1995 and 2000. RESULTS: Diagnostic subgroups were @entity6129 , 120 @entity1 (49%); @entity2883 ( @entity2883 ), 82 @entity1 (33%); renal-limited @entity1333 , 33 @entity1 (13.5%); and @entity12534 , 11 @entity1 (4.5%). Median age was 66 years, 57% were @entity1 , and median @entity1011 level at presentation was 3.87 mg/dL (342 micromol/L). ANCA was present in 92%. Cumulative @entity1 survival at 1 and 5 years was 82% and 76%, respectively. Mortality was associated with age older than 60 years (P < 0.001), development of @entity454 (P < 0.001), initial @entity1011 level greater than 2.26 mg/dL (200 micromol/L; P = 0.01), and @entity295 (P < 0.048). @entity454 occurred in 68 @entity1 (28%), of whom 47% died. Fifty-six @entity1 who presented with a @entity1011 level greater than 5.65 mg/dL (500 micromol/L) survived, and 31 @entity1 (55%) achieved dialysis independence. Relapse occurred in 34% after a median of 13 months and was more common in @entity1 with @entity2883 (P = 0.048) and @entity7134 -ANCA (P = 0.034). @entity1431 occurred in 41% and was associated with @entity295 (P < 0.001). CONCLUSION: Mortality and morbidity of ANCA-associated systemic @entity1333 are improving compared with previous series, but remain high. @entity8 often affects older @entity1 , who have a particularly poor outcome. Early diagnosis improves outcome. @entity1431 , caused by immunosuppressive therapy, should be avoided because of the close association with @entity295 and @entity204 .
