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Pneumo-Renal Syndrome in Anti- Neutrophil Cytoplasm Antibody (ANCA)-Associated Small-Vessel Vasculitis
Objectives: We aimed to evaluate the prevalence of lung hemorrhage, its determinants and its prognostic significance in adults patients with severe kidney involvement due to ANCA-associated vasculitis diagnosed and treated in a nephrology department.
Material and Methods: Seventy-five patients consecutively diagnosed by kidney biopsy with crescentic pauci-immune glomerulonephritis entered this cohort study and were grouped according to the presence of diffuse alveolar hemorrhage (DAH – diagnosed as diffuse alveolar pattern on chest radiographs and anemia without evidence of another external bleeding). ANCAs were assessed by capture PR3-ANCA and MPO–ANCA ELISA or by indirect immunofluorescence.
Outcomes: Patients were followed for a median period of 38 (11.7; 65.8) months. The median age was 61.6 years. Median creatinine was 5.7 mg/dL and 17% of the patients needed temporary dialysis. Most of the patients (76%) had MPO-ANCA; 31% had pneumo-renal syndrome. DAH patients had more severe and active kidney disease, as reflected by higher serum creatinine (7.1 mg/dL vs 4.45 mg/dL; p=0.006) and higher hematuria (610/mm3 vs 230/mm3, p=0.003). The risk of DAH was not influenced by gender, age or ANCA specificity, but by smoking (smokers had a 4 (95%CI 1.18-14.2; p=0.002) times higher the risk of lung hemorrhage) and by season (patients diagnosed in winter and autumn had a 6 (95% CI 1.6-20.9; p=0.005) times higher the risk of lung hemorrhage). The proportion of responders and of patients with relapses, and time to maintenance dialysis or to death were similar irrespective to the occurrence of DAH at presentation.
Conclusions: In patients with ANCA associated pauci-immune glomerulonephritis, cold season, smoking and active kidney disease, but not ANCA specificity or inflammation were associated with lung hemorrhage. Although diffuse alveolar hemorrhage was the main cause of death, it was not related
to short- or long-term outcome.