Background: The clinic-pathological features and outcomes of Chinese patients with antineutrophil cytoplasmic\nautoantibody (ANCA)-positive eosinophilic granulomatosis with polyangiitis (EGPA) and renal involvement have\nnot been studied.\nMethods: Fourteen EGPA patients with renal involvement were included. All patients underwent renal biopsy.\nClinic-pathological features and outcomes were retrospectively analyzed.\nResults: The most common initial symptom of EGPA was asthma (57.1 %), followed by hemoptysis (21.4 %),\ngross hematuria (14.3 %), and arthritis (7.1 %). All patients had positive serum ANCA (anti-MPO in 12, anti-PR3 in 2).\nElevated eosinophils (median 15 %, range 10ââ?¬â??45 %) were found in all patients. The median serum IgE level was\n463 g/L (range 200ââ?¬â??1000 g/L). All patients presented with renal dysfunction, with a median SCr of 5.4 mg/dL\n(range 1.47ââ?¬â??11 mg/dL), seven patients (50 %) required initial renal replacement therapy. Thirteen patients showed\nhematuria and proteinuria (median 1.1 g/24 h, range 0.5ââ?¬â??7.8 g/24 h). Renal biopsy showed pauci-immune segmental\nnecrotizing glomerulonephritis with crescents in 13 patients and acute interstitial nephritis in one patient. Twelve\npatients (85.7 %) showed renal interstitial eosinophil infiltration, among whom three had eosinophilic granuloma.\nAmong seven patients (71.4 %) who required initial dialysis, 5 discontinued dialysis, one died, one received\nmaintenance dialysis after glucocorticoids plus immunosuppressive for induction treatment. Twelve patients were\nfollowed up for a median of 43.5 months (range 6ââ?¬â??83 months), during follow-up, two patients progressed to\nend-stage renal disease, nine had chronic kidney disease with eGFR < 60 mL/min, and two patients had normal eGFR.\nConclusions: Renal involvement in ANCA-positive EGPA could be severe and showed varied renal histology.\nAlthough intensive immunosuppressive therapy effectively improved the renal function, the long-term renal\nsurvival was poor. Early diagnosis and treatment are essential to improve long-term renal survival.
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