title: A historical study of American patients with anti-neutrophil cytoplasmic antibody negative pauci-immune glomerulonephritis.
abstract: Anti-neutrophil cytoplasmic antibodies (ANCA) play an important role in the pathogenesis of ANCA-associated vasculitis. The lack of ANCA antibodies may indicate a variation in clinical presentation and outcomes of this disease. We identified 74 adult patients between 1995 and 2009 with the diagnosis of pauci-immune glomerulonephritis. Demographics, histological features, and treatment outcomes were compared between ANCA-positive and ANCA-negative patients. These factors were correlated with renal function at presentation and follow-up. Of the 74 patients, 57 were ANCA-positive, and 17 were ANCA-negative. Demographics and mean Birmingham Vasculitis Activity Score were similar between ANCA-negative and ANCA-positive patients at presentation. Renal function was significantly worse at presentation in the ANCA-negative patients (eGFR 16.59 vs. 31.89 ml/min/1.73 m(2), p = 0.03). Patients in the ANCA-negative group had a significantly higher interstitial fibrosis score compared to the ANCA-positive group (2.1 vs.1.6, p = 0.04). The median time to remission was shorter in the ANCA-negative patients (51 vs. 78 days, p = 0.01). Long-term renal function and 1-year patient and renal survival were similar between ANCA-negative and ANCA-positive patients. Baseline eGFR, percentage of normal glomeruli, glomerular sclerosis, and tubulointerstitial scarring predicted eGFR at 1 year in both groups similarly. This is the first historical review of American patients with pauci-immune glomerulonephritis, comparing patients with ANCA-negative and ANCA-positive serology. Although ANCA-negative patients present with lower eGFR and more interstitial fibrosis, 1-year and long-term outcomes in both groups are similar.
authors.affiliation: Division of Nephrology, Department of Medicine, Johns Hopkins Hospital, Baltimore, MD, USA. sshah72@jhmi.edu.|Division of Nephrology, Department of Medicine, Johns Hopkins Bayview Medical Center, 301 Mason Lord Drive, Suite 2500, Baltimore, MD, 21224, USA. sshah72@jhmi.edu.
authors.forename: Shivani
authors.initials: S
authors.lastname: Shah
authors.affiliation: Kidney Specialists of Southern Nevada, 100 N Green Valley pkwy, Henderson, NV, USA.
authors.forename: John
authors.initials: J
authors.lastname: Havill
authors.affiliation: Bloomberg School of Public Health, Johns Hopkins University, Baltimore, MD, USA.
authors.forename: M Hafizur
authors.initials: MH
authors.lastname: Rahman
authors.affiliation: Division of Nephrology, Department of Medicine, Johns Hopkins Hospital, Baltimore, MD, USA.
authors.forename: Duvuru
authors.initials: D
authors.lastname: Geetha
mesh_terms: D000328:Adult; D000368:Aged; D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000097:blood / Q000453:epidemiology* / Q000276:immunology*; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood / Q000276:immunology*; D001706:Biopsy; D003404:Creatinine / Q000097:blood; D005260:Female; D005355:Fibrosis / Q000276:immunology; D005919:Glomerular Filtration Rate; D005921:Glomerulonephritis / Q000097:blood / Q000453:epidemiology* / Q000276:immunology*; D006801:Humans; D007668:Kidney / Q000473:pathology; D007677:Kidney Function Tests; D008297:Male; D008875:Middle Aged; D012044:Regression Analysis; D012074:Remission Induction; D012189:Retrospective Studies; D012720:Severity of Illness Index; D013997:Time Factors; D016896:Treatment Outcome; D014481:United States; D014657:Vasculitis / Q000276:immunology
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D003404:Creatinine
keywords: ANCA negative; GPA; MPA; Vasculitis