title: Clinico-pathological features and outcomes of patients with propylthiouracil-associated ANCA vasculitis with renal involvement.
abstract: OBJECTIVE
  To retrospectively investigate clinico-pathological features and outcomes of patients with renal involvement in propylthiouracil (PTU)-associated antineutrophil cytoplasmic autoantibody (ANCA) vasculitis (PTU-AAV).
  
  
  METHODS
  Clinico-pathological features and outcomes of 12 patients (female 11, average age 32.4 ± 13.8 years) who developed AAV after treatment with PTU were collected and analyzed. ANCA was detected by both immunofluorescence (IF) and enzyme linked immunosorbent assay (ELISA). All patients had renal biopsy.
  
  
  RESULTS
  Twelve patients received PTU for 2-264 months (median 42 months) when PTUAAV was diagnosed. All patients had positive serum P-ANCA, 11 of them were MPO-ANCA, 1 was MPO and PR3-ANCA double positive. All patients presented with hematuria and proteinuria, 5 of them had gross hematuria, urine protein was 1.9 ± 1.6 g/24 h, 7 of 12 (58.3%) patients had renal dysfunction, among them 3 needed initial renal replacement therapy. Renal biopsy showed pauci-immune segmental necrotizing crescentic glomerulonephritis in ten patients, segmental necrotizing glomerulonephritis superimposed on membranous nephropathy in two patients. All patients withdrew PTU and received steroid and immunosuppressive therapy. After a median follow-up of 42 months (range 21-86), 3 patients developed to ESRD, 7 patients entered complete renal remission. Serum ANCA turned negative only in 2 patients, 10 patients had persistent positive serum ANCA. Three patients relapsed with the elevation of serum ANCA level.
  
  
  CONCLUSIONS
  Renal damage of PTU-AAV could be pauci-immune necrotizing crescentic glomerulonephritis, and necrotizing glomerulonephritis coexisted with membranous nephropathy. Most patients had persistent positive serum ANCA and had a risk of relapse and progression to ESRD even after PTU withdrawal and immunosuppressive therapy.
authors.affiliation: National Clinical Research Center of Kidney Diseases, Jinling Hospital, University School of Medicine, Nanjing, 210002, Jiangsu, China.
authors.forename: Yinghua
authors.initials: Y
authors.lastname: Chen
authors.forename: Hao
authors.initials: H
authors.lastname: Bao
authors.forename: Zhengzhao
authors.initials: Z
authors.lastname: Liu
authors.forename: Haitao
authors.initials: H
authors.lastname: Zhang
authors.forename: Caihong
authors.initials: C
authors.lastname: Zeng
authors.forename: Zhihong
authors.initials: Z
authors.lastname: Liu
authors.forename: Weixin
authors.initials: W
authors.lastname: Hu
mesh_terms: D000293:Adolescent; D000328:Adult; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000139:chemically induced / Q000150:complications* / Q000188:drug therapy*; D013956:Antithyroid Agents / Q000009:adverse effects; D002648:Child; D003520:Cyclophosphamide / Q000627:therapeutic use; D018450:Disease Progression; D006417:Hematuria / Q000209:etiology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007676:Kidney Failure, Chronic / Q000209:etiology* / Q000473:pathology / Q000628:therapy; D007678:Kidney Glomerulus / Q000473:pathology; D007684:Kidney Tubules / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D009173:Mycophenolic Acid / Q000031:analogs & derivatives / Q000627:therapeutic use; D011241:Prednisone / Q000627:therapeutic use; D011441:Propylthiouracil / Q000009:adverse effects; D011507:Proteinuria / Q000209:etiology; D012008:Recurrence; D012074:Remission Induction; D017582:Renal Replacement Therapy; D012189:Retrospective Studies; D013959:Thyroid Diseases / Q000188:drug therapy; D055815:Young Adult
chemical_list: D000893:Anti-Inflammatory Agents; D013956:Antithyroid Agents; D007166:Immunosuppressive Agents; D011441:Propylthiouracil; D003520:Cyclophosphamide; D009173:Mycophenolic Acid; D011241:Prednisone