title: An open-label trial of abatacept (CTLA4-IG) in non-severe relapsing granulomatosis with polyangiitis (Wegener's).
abstract: OBJECTIVE
  To determine the safety and efficacy of abatacept in non-severe relapsing granulomatosis with polyangiitis (Wegener's)(GPA).
  
  
  METHODS
  An open-label trial of intravenous abatacept was conducted in 20 patients with non-severe relapsing GPA. Prednisone up to 30 mg daily was permitted within the first 2 months, and patients on methotrexate, azathioprine, or mycophenolate mofetil continued these agents. Patients remained on study until common closing or early termination.
  
  
  RESULTS
  Of the 20 patients, 18 (90%) had disease improvement, 16 (80%) achieved remission (BVAS/WG=0) at a median of 1.9 months, and 14 (70%) reached common closing. Six patients (30%) met criteria for early termination due to increased disease activity; 3 of 6 achieved remission and relapsed at a median of 8.6 months. The median duration of remission before common closing was 14.4 months, with the median duration of time on study for all patients being 12.3 months (range 2-35 months). Eleven of the 15 (73%) patients on prednisone reached 0 mg. Nine severe adverse events occurred in 7 patients, including 7 infections that were successfully treated.
  
  
  CONCLUSIONS
  In this study of patients with non-severe relapsing GPA, abatacept was well tolerated and was associated with a high frequency of disease remission and prednisone discontinuation.
authors: Cleveland Clinic, Cleveland, Ohio, USA.
authors: Carol A
authors: CA
authors: Langford
authors: Boston University, Boston, Massachusetts, USA.
authors: Paul A
authors: PA
authors: Monach
authors: Mayo Clinic, Rochester, Minnesota, USA.
authors: Ulrich
authors: U
authors: Specks
authors: John Hopkins University, Baltimore, Maryland, USA.
authors: Philip
authors: P
authors: Seo
authors: University of South Florida, Tampa, Florida, USA.
authors: David
authors: D
authors: Cuthbertson
authors: University of Pennsylvania, Philadelphia, Pennsylvania, USA.
authors: Carol A
authors: CA
authors: McAlear
authors: Mayo Clinic, Rochester, Minnesota, USA.
authors: Steven R
authors: SR
authors: Ytterberg
authors: Cleveland Clinic, Cleveland, Ohio, USA.
authors: Gary S
authors: GS
authors: Hoffman
authors: University of South Florida, Tampa, Florida, USA.
authors: Jeffrey P
authors: JP
authors: Krischer
authors: Boston University, Boston, Massachusetts, USA University of Pennsylvania, Philadelphia, Pennsylvania, USA.
authors: Peter A
authors: PA
authors: Merkel
mesh_terms: D000069594:Abatacept; D000293:Adolescent; D000328:Adult; D000368:Aged; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D001379:Azathioprine / Q000627:therapeutic use; D015331:Cohort Studies; D004359:Drug Therapy, Combination; D005260:Female; D014890:Granulomatosis with Polyangiitis / Q000188:drug therapy*; D006801:Humans; D018796:Immunoconjugates / Q000627:therapeutic use*; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008297:Male; D008727:Methotrexate / Q000627:therapeutic use; D008875:Middle Aged; D009173:Mycophenolic Acid / Q000031:analogs & derivatives / Q000627:therapeutic use; D011241:Prednisone / Q000627:therapeutic use; D011446:Prospective Studies; D012008:Recurrence; D012074:Remission Induction / Q000379:methods; D012720:Severity of Illness Index; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D000893:Anti-Inflammatory Agents; D018796:Immunoconjugates; D007166:Immunosuppressive Agents; D000069594:Abatacept; D009173:Mycophenolic Acid; D001379:Azathioprine; D011241:Prednisone; D008727:Methotrexate
keywords: Granulomatosis with polyangiitis; Systemic vasculitis; Treatment