Article Text

Download PDFPDF
Concise Report
An open-label trial of abatacept (CTLA4-IG) in non-severe relapsing granulomatosis with polyangiitis (Wegener's)
  1. Carol A Langford1,
  2. Paul A Monach2,
  3. Ulrich Specks3,
  4. Philip Seo4,
  5. David Cuthbertson5,
  6. Carol A McAlear6,
  7. Steven R Ytterberg3,
  8. Gary S Hoffman1,
  9. Jeffrey P Krischer5,
  10. Peter A Merkel2,6,
  11. for the Vasculitis Clinical Research Consortium
  1. 1Cleveland Clinic, Cleveland, Ohio, USA
  2. 2Boston University, Boston, Massachusetts, USA
  3. 3Mayo Clinic, Rochester, Minnesota, USA
  4. 4John Hopkins University, Baltimore, Maryland, USA
  5. 5University of South Florida, Tampa, Florida, USA
  6. 6University of Pennsylvania, Philadelphia, Pennsylvania, USA
  1. Correspondence to Dr Carol A Langford, Center for Vasculitis Care and Research, Cleveland Clinic, 9500 Euclid Avenue, A50 Cleveland, OH 44195, USA; langfoc{at}


Objectives 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.

  • Systemic vasculitis
  • Granulomatosis with polyangiitis
  • Treatment

Statistics from

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.