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

摘要

The recently suggested distinct pathogenic pathways for myeloperoxidase (MPO) and proteinase 3 (PR3) anti-neutrophilic cytoplasmic antibodies (ANCA) associated vasculitis could result in different modes of presentation and outcome. Moreover, kidney outcome was related to a new histopathologic classification of pauci-immune glomerulonephritis. 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 patients with crescentic glomerulonephritis in relation with ANCA specificity and histopathological classification. Seventy-five patients were consecutively diagnosed by kidney biopsy (76?% MPO-ANCA specificity, 52?% crescentic) and followed for a median period of 3.2?years. Study end-points were response to therapy, end stage renal disease (ESRD) and death. PR3-ANCA patients were younger, in higher proportion male and had higher Birmingham Vasculitis Activity Scores (BVAS). The kidney disease was severe at presentation (median creatinine 5?mg/dL; 27?% required temporary dialysis) and worst in PR3-ANCA positive patients (50?% patients needed temporary dialysis vs. 19?%). The lung was the second most affected organ (31?% severe lung hemorrhage). Lung and kidney damage were related; the odds of hemorrhagic alveolitis in patients 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 active vasculitis and stable or declining serum creatinine) 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 patient were 93 and 64?%, and respectively 88 and 67?%. Kidney survival was predicted by response to therapy and dialysis independency at presentation. Patients with BVAS?