Anti-neutrophil antibody associated vasculitis in XXXX .

@entity1 @entity8 @entity101 @entity548 @entity63 @entity1910 @entity882 @entity786 @entity177 @entity288 @entity11278 @entity2106 @entity19417 @entity785
OBJECTIVES: To report 3 cases @entity19417 ( @entity19417 ) that developed in @entity1 suffering from @entity785 ( @entity785 ) and to review previously reported cases. METHODS: We describe 3 @entity1 diagnosed with @entity785 who developed severe @entity19417 that presented as @entity11278 . A retrospective review of the literature was performed using the PubMed database. RESULTS: The first @entity1 presented with rapidly progressive @entity177 and then with 2 episodes of @entity548 . She was partially refractory to treatment with @entity288 and @entity786 but responded promptly to treatment with rituximab. The second @entity1 suffered from 2 episodes of @entity548 ; the first responded to intravenous @entity288 , but the second was fatal despite aggressive immune suppression with @entity288 and @entity786 . The third @entity1 presented with a clinical picture compatible with @entity8 ( @entity8 ) but was later diagnosed with crescentic GN and subsequently died from probable @entity548 . Thirty-seven cases of @entity19417 in @entity785 @entity1 have been described in the English literature. Clinical manifestations include rapidly progressive GN, @entity548 , @entity63 , and @entity1910 . In contrast to @entity8 that usually develops early in the course of @entity785 , ANCA-associated GN in @entity785 @entity1 occurred later, after several years of illness. @entity101 , @entity2106 , and @entity882 that are the hallmark of @entity8 were observed only in a minority of @entity19417 cases. Almost all cases of @entity19417 in @entity785 were positive for MPO-ANCA. CONCLUSIONS: @entity19417 in the setting of @entity785 is a diagnostic challenge. Differential diagnosis from @entity8 is crucial as treatment approach for these conditions completely differs. High doses of @entity288 and immune suppression are advocated in severe @entity19417 . In resistant cases, treatment with rituximab may be considered.
