Renal involvement in XXXX .

@entity1712 @entity1 @entity8 @entity3551 @entity1494 @entity1333 @entity12534 @entity565 @entity740 @entity177 @entity1463 @entity1986 @entity395 @entity631
BACKGROUND: @entity12534 ( @entity12534 ) is a @entity395 characterized by @entity565 , @entity1712 , and systemic @entity1333 . Renal involvement is not regarded as a prominent feature, and its prevalence and severity vary widely in published reports that usually refer to small series of selected @entity1 . METHODS: We examined the prevalence, clinicopathologic features, and prognosis of @entity8 in 116 @entity1 with @entity12534 . RESULTS: There were 48 @entity1 and 68 @entity1 with a mean age of 51.9 years (range, 18 to 86 years). Signs of @entity8 were present in 31 @entity1 (26.7%). Rapidly progressive @entity177 was documented in 16 @entity1 (13.8%); @entity1494 , 14 @entity1 (12.1%); and chronic @entity8 , 1 @entity1 . There were 3 additional cases of @entity3551 . Sixteen @entity1 underwent renal biopsy, which showed necrotizing crescentic @entity631 in 11 @entity1 . Other diagnoses were eosinophilic @entity1986 , @entity631 , and focal @entity1463 . Antineutrophil cytoplasmic antibody (ANCA) was positive in 21 of 28 @entity1 (75.0%) with @entity8 versus 19 of 74 @entity1 without (25.7%; P < 0.001). In particular, all @entity1 with necrotizing crescentic @entity631 were ANCA positive. After a median follow-up of 4.5 years, 10 @entity1 died (5 @entity1 with @entity8 ) and 7 @entity1 developed mild @entity740 . Five-year mortality rates were 11.7% (95% confidence interval, 3.9 to 33.3) in @entity1 with @entity8 and 2.7% (95% confidence interval, 0.7 to 10.7) in those without (P = 0.10). CONCLUSION: @entity8 are present in about one quarter of @entity1 with @entity12534 . The prevailing picture is ANCA-associated necrotizing crescentic @entity631 ; however, other forms of @entity8 also may occur. Outcome and long-term follow-up usually are good.
