Background: The recently suggested distinct pathogenic pathways for myeloperoxidase (MPO) and proteinase 3 (PR3)\nanti-neutrophilic cytoplasmic antibodies (ANCA) associated vasculitis could result in different modes of presentation\nand outcome. Moreover, kidney outcome was related to a new histopathologic classification of pauci-immune\nglomerulonephritis. As reports were not always concordant, possible because differences in severity of organ\nlesions and ethnicity, we evaluated the outcome of a cohort of Central-East European patients with crescentic\nglomerulonephritis in relation with ANCA specificity and histopathological classification.\nMethods: Seventy-five patients were consecutively diagnosed by kidney biopsy (76 % MPO-ANCA specificity,\n52 % crescentic) and followed for a median period of 3.2 years. Study end-points were response to therapy, end\nstage renal disease (ESRD) and death.\nResults: PR3-ANCA patients were younger, in higher proportion male and had higher Birmingham Vasculitis\nActivity Scores (BVAS). The kidney disease was severe at presentation (median creatinine 5 mg/dL; 27 % required\ntemporary dialysis) and worst in PR3-ANCA positive patients (50 % patients needed temporary dialysis vs. 19 %). The\nlung was the second most affected organ (31 % severe lung hemorrhage). Lung and kidney damage were related; the\nodds of hemorrhagic alveolitis in patients needing dialysis at presentation were 4 (95 % CI 1ââ?¬â??13; p = 0.006) times higher\nthan in those who did not. The rate of response to therapy (without signs of active vasculitis and stable or declining\nserum creatinine) was 60 % and was associated with dialysis independency, older age and higher platelet number at\npresentation. The probabilities to survival 1 and 5 years for kidney and patient were 93 and 64 %, and respectively 88\nand 67 %. Kidney survival was predicted by response to therapy and dialysis independency at presentation. Patients\nwith BVAS < 15 and responding to induction therapy had better chances of survival. Neither response to therapy nor\noutcome was influenced by ANCA specificity or by the histopathological class.\nConclusions: When kidney damage is severe in ANCA vasculitis, the need of dialysis at presentation and the response\nto induction therapy overcome the prognostic utility of both ANCA specificity and histopathological class.
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