title: Long-term outcomes in antineutrophil cytoplasmic autoantibody-positive eosinophilic granulomatosis with polyangiitis patients with renal involvement: a retrospective study of 14 Chinese patients.
abstract: The clinic-pathological features and outcomes of Chinese patients with antineutrophil cytoplasmic autoantibody (ANCA)-positive eosinophilic granulomatosis with polyangiitis (EGPA) and renal involvement have not been studied.
  
  
  
  Fourteen EGPA patients with renal involvement were included. All patients underwent renal biopsy. Clinic-pathological features and outcomes were retrospectively analyzed.
  
  
  
  The most common initial symptom of EGPA was asthma (57.1 %), followed by hemoptysis (21.4 %), gross hematuria (14.3 %), and arthritis (7.1 %). All patients had positive serum ANCA (anti-MPO in 12, anti-PR3 in 2). Elevated eosinophils (median 15 %, range 10-45 %) were found in all patients. The median serum IgE level was 463 g/L (range 200-1000 g/L). All patients presented with renal dysfunction, with a median SCr of 5.4 mg/dL (range 1.47-11 mg/dL), seven patients (50 %) required initial renal replacement therapy. Thirteen patients showed hematuria and proteinuria (median 1.1 g/24 h, range 0.5-7.8 g/24 h). Renal biopsy showed pauci-immune segmental necrotizing glomerulonephritis with crescents in 13 patients and acute interstitial nephritis in one patient. Twelve patients (85.7 %) showed renal interstitial eosinophil infiltration, among whom three had eosinophilic granuloma. Among seven patients (71.4 %) who required initial dialysis, 5 discontinued dialysis, one died, one received maintenance dialysis after glucocorticoids plus immunosuppressive for induction treatment. Twelve patients were followed up for a median of 43.5 months (range 6-83 months), during follow-up, two patients progressed to end-stage renal disease, nine had chronic kidney disease with eGFR < 60 mL/min, and two patients had normal eGFR.
  
  
  
  Renal involvement in ANCA-positive EGPA could be severe and showed varied renal histology. Although intensive immunosuppressive therapy effectively improved the renal function, the long-term renal survival was poor. Early diagnosis and treatment are essential to improve long-term renal survival.
authors.affiliation: National Clinical Research Center of Kidney Diseases, Jinling Hospital, Nanjing University School of Medicine, 305 East Zhongshan Road, Nanjing, 210002, Jiangsu, China.
authors.forename: Yinghua
authors.initials: Y
authors.lastname: Chen
authors.affiliation: Jiangsu Jiangyin People's Hospital, Jiangyin, China.
authors.forename: Yuemei
authors.initials: Y
authors.lastname: Ding
authors.affiliation: National Clinical Research Center of Kidney Diseases, Jinling Hospital, Nanjing University School of Medicine, 305 East Zhongshan Road, Nanjing, 210002, Jiangsu, China.
authors.forename: Zhengzhao
authors.initials: Z
authors.lastname: Liu
authors.affiliation: National Clinical Research Center of Kidney Diseases, Jinling Hospital, Nanjing University School of Medicine, 305 East Zhongshan Road, Nanjing, 210002, Jiangsu, China.
authors.forename: Haitao
authors.initials: H
authors.lastname: Zhang
authors.affiliation: National Clinical Research Center of Kidney Diseases, Jinling Hospital, Nanjing University School of Medicine, 305 East Zhongshan Road, Nanjing, 210002, Jiangsu, China.
authors.forename: Zhihong
authors.initials: Z
authors.lastname: Liu
authors.affiliation: National Clinical Research Center of Kidney Diseases, Jinling Hospital, Nanjing University School of Medicine, 305 East Zhongshan Road, Nanjing, 210002, Jiangsu, China. huwx@vip.163.com.
authors.forename: Weixin
authors.initials: W
authors.lastname: Hu
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood*; D001772:Blood Cell Count; D002681:China; D015267:Churg-Strauss Syndrome / Q000150:complications / Q000276:immunology*; D003404:Creatinine / Q000097:blood; D003520:Cyclophosphamide / Q000627:therapeutic use; D018450:Disease Progression; D004359:Drug Therapy, Combination; D004804:Eosinophils; D005260:Female; D005919:Glomerular Filtration Rate; D006417:Hematuria / Q000209:etiology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007676:Kidney Failure, Chronic / Q000276:immunology; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use; D008875:Middle Aged; D009173:Mycophenolic Acid / Q000627:therapeutic use; D011241:Prednisone / Q000627:therapeutic use; D011507:Proteinuria / Q000209:etiology; D006435:Renal Dialysis; D051436:Renal Insufficiency, Chronic / Q000150:complications / Q000276:immunology* / Q000473:pathology / Q000628:therapy*; D012189:Retrospective Studies; D013997:Time Factors; D055815:Young Adult
chemical_list: D000893:Anti-Inflammatory Agents; D019268:Antibodies, Antineutrophil Cytoplasmic; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D003404:Creatinine; D009173:Mycophenolic Acid; D011241:Prednisone; D008775:Methylprednisolone
keywords: Antineutrophil cytoplasmic autoantibody; Eosinophilic granulomatosis with polyangiitis; Outcome