BACKGROUND: The recently suggested distinct pathogenic pathways for @entity3335 ( @entity3335 ) and @entity7134 ( @entity7134 ) anti-neutrophilic cytoplasmic antibodies (ANCA) associated @entity1333 could result in different modes of presentation and outcome. Moreover, kidney outcome was related to a new histopathologic classification of pauci-immune @entity631 . As reports were not always concordant, possible because differences in severity of organ lesions and ethnicity, we evaluated the outcome of a cohort of Central-East European @entity1 with crescentic @entity631 in relation with ANCA specificity and histopathological classification. METHODS: Seventy-five @entity1 were consecutively diagnosed by kidney biopsy (76  % @entity3335 -ANCA specificity, 52  % crescentic) and followed for a median period of 3.2  years. Study end-points were response to therapy, @entity454 ( @entity454 ) and @entity204 . RESULTS: @entity7134 -ANCA @entity1 were younger, in higher proportion male and had higher Birmingham @entity1333 Activity Scores (BVAS). The @entity8 was severe at presentation (median @entity1011 5  mg/dL; 27  % required temporary dialysis) and worst in @entity7134 -ANCA positive @entity1 (50  % @entity1 needed temporary dialysis vs. 19  %). The lung was the second most affected organ (31  % severe @entity548 ). Lung and @entity8 were related; the odds of @entity548 in @entity1 needing dialysis at presentation were 4 (95  % CI 1-13; p   =   0.006) times higher than in those who did not. The rate of response to therapy (without signs of @entity1333 and stable or declining serum @entity1011 ) was 60  % and was associated with dialysis independency, older age and higher platelet number at presentation. The probabilities to survival 1 and 5  years for kidney and @entity1 were 93 and 64  %, and respectively 88 and 67  %. Kidney survival was predicted by response to therapy and dialysis independency at presentation. @entity1 with BVAS   <   15 and responding to induction therapy had better chances of survival. Neither response to therapy nor outcome was influenced by ANCA specificity or by the histopathological class. CONCLUSIONS: When @entity8 is severe in ANCA @entity1333 , the need of dialysis at presentation and the response to induction therapy overcome the prognostic utility of both ANCA specificity and histopathological class.

ANCA positive crescentic XXXX outcome in a Central East European cohort: a retrospective study.

@entity1 :: ('9606', 'Species') :: ['Patients', 'patients', 'patient'] @entity8 :: ('MESH:D007674', 'Disease') :: ['kidney damage', 'kidney disease'] @entity3335 :: ('4353', 'Gene') :: ['MPO', 'myeloperoxidase'] @entity548 :: ('MESH:D006470', 'Disease') :: ['hemorrhagic alveolitis', 'lung hemorrhage'] @entity1333 :: ('MESH:D014657', 'Disease') :: ['Vasculitis', 'active vasculitis', 'vasculitis'] @entity1011 :: ('MESH:D003404', 'Chemical') :: ['creatinine'] @entity7134 :: ('5657', 'Gene') :: ['proteinase 3', 'PR3'] @entity631 :: ('MESH:D005921', 'Disease') :: ['glomerulonephritis'] @entity204 :: ('MESH:D003643', 'Disease') :: ['death'] @entity454 :: ('MESH:D007676', 'Disease') :: ['ESRD', 'end stage renal disease']
@entity631 :: (MESH:D005921,Disease) :: ['glomerulonephritis']
