title: Necrotizing and crescentic lupus nephritis with antineutrophil cytoplasmic antibody seropositivity.
abstract: OBJECTIVE
  Lupus nephritis is a classic immune complex glomerulonephritis. In contrast, antineutrophil cytoplasmic antibodies are associated with necrotizing and crescentic glomerulonephritis, in the absence of significant immune deposits. Antineutrophil cytoplasmic antibodies are detected by indirect immunofluorescence in 20% of patients with systemic lupus erythematosus. We report 10 cases of necrotizing and crescentic lupus nephritis with antineutrophil cytoplasmic antibody seropositivity.
  
  
  METHODS
  Ten patients with systemic lupus erythematosus, antineutrophil cytoplasmic antibody positivity, and renal biopsy findings of lupus nephritis and antineutrophil cytoplasmic antibody-associated glomerulonephritis were identified. The clinical features, pathologic findings, and outcomes are described.
  
  
  RESULTS
  The cohort consisted of eight women and two men with a mean age of 48.4 yr. Perinuclear antineutrophil cytoplasmic antibody was detected by indirect immunofluorescence in nine patients. Four of the nine patients and the single remaining patient were found to have myeloperoxidase-antineutrophil cytoplasmic antibodies by enzyme-linked immunosorbent assay. Clinical presentation included proteinuria, hematuria, and acute renal insufficiency, with mean creatinine of 7.1 mg/dl. All biopsies exhibited prominent necrosis and crescents with absent or rare subendothelial deposits and were interpreted as lupus nephritis and antineutrophil cytoplasmic antibody-associated glomerulonephritis. All patients received cyclophosphamide and prednisone. Three patients died of infectious complications. Among the remaining seven patients, five achieved a complete or near-complete remission, one had a remission with subsequent relapse, and one had no response to therapy.
  
  
  CONCLUSIONS
  Antineutrophil cytoplasmic antibody-associated necrotizing and crescentic glomerulonephritis may occur superimposed on lupus nephritis. In patients with lupus nephritis and biopsy findings of prominent necrosis and crescent formation in the absence of significant endocapillary proliferation or subendothelial deposits, antineutrophil cytoplasmic antibody testing by enzyme-linked immunosorbent assay is recommended.
authors.affiliation: Department of Pathology, Columbia University, College of Physicians & Surgeons, 630 West 168th Street, VC14-224, New York, NY 10032, USA. sn386@columbia.edu
authors.forename: Samih H
authors.initials: SH
authors.lastname: Nasr
authors.forename: Vivette D
authors.initials: VD
authors.lastname: D'Agati
authors.forename: Hye-Ran
authors.initials: HR
authors.lastname: Park
authors.forename: Paul L
authors.initials: PL
authors.lastname: Sterman
authors.forename: Juan D
authors.initials: JD
authors.lastname: Goyzueta
authors.forename: Robert M
authors.initials: RM
authors.lastname: Dressler
authors.forename: Shawn M
authors.initials: SM
authors.lastname: Hazlett
authors.forename: Robert N
authors.initials: RN
authors.lastname: Pursell
authors.forename: Christopher
authors.initials: C
authors.lastname: Caputo
authors.forename: Glen S
authors.initials: GS
authors.lastname: Markowitz
mesh_terms: D000328:Adult; D000368:Aged; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood*; D003520:Cyclophosphamide / Q000627:therapeutic use; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D019084:Fluorescent Antibody Technique, Indirect; D005500:Follow-Up Studies; D005921:Glomerulonephritis / Q000188:drug therapy / Q000276:immunology* / Q000401:mortality / Q000473:pathology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007668:Kidney / Q000276:immunology* / Q000473:pathology; D008181:Lupus Nephritis / Q000188:drug therapy / Q000276:immunology* / Q000401:mortality / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009336:Necrosis; D009195:Peroxidase / Q000276:immunology*; D011239:Prednisolone / Q000627:therapeutic use; D012008:Recurrence; D016896:Treatment Outcome
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D011239:Prednisolone; D009195:Peroxidase