Official reprint from UpToDate®
www.uptodate.com ©2017 UpToDate, Inc. and/or its affiliates. All Rights Reserved.

Overview of the management of vasculitis in adults

Peter A Merkel, MD, MPH
Section Editor
Eric L Matteson, MD, MPH
Deputy Editor
Monica Ramirez Curtis, MD, MPH


The vasculitides are defined by the presence of leukocytes in the vessel wall with reactive damage to mural structures. In general, affected vessels vary in size, type, and location in association with the specific vasculitic disorder. These conditions are often serious and sometimes fatal diseases that require prompt recognition and therapy.

Symptomatic involvement may occur in single or multiple organs. Specific clinical manifestations may suggest a particular vasculitic disorder, but significant overlap is observed among the vasculitides. The wide clinical spectrum of disease in the vasculitides presents particular challenges to management of these complex disorders.

This topic review will provide an overview of the approach to the management of the vasculitides. Treatment recommendations for specific forms of vasculitis are reviewed for each disorder separately (see 'Disease-specific treatments' below), and an overview of the classification and clinical manifestations of the different vasculitides and the approach to the patient with suspected vasculitis is presented separately. (See "Overview of and approach to the vasculitides in adults".)


The framework for the treatment of vasculitis is similar to that used for many other systemic autoimmune rheumatic disorders, while the specific therapeutic regimen depends upon the nature and severity of the particular disorder (see 'Disease-specific treatments' below). The management scheme for vasculitis generally includes the following components:

Remission induction – The goal of initial treatment is to induce remission of the disease. Initial management usually involves use of medium to high doses of glucocorticoids, with the use of an additional immunosuppressive agent in some forms of disease. The initial presentation of vasculitis is often rapid, and delay in diagnosis or failure to recognize the extent of disease and arrest the disease process can lead to substantial morbidity and, in some forms of disease, mortality. The initial treatment phase may thus be more intensive than the subsequent phases of treatment, involving the use of higher doses or drugs with greater risk of toxicity.

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:

Subscribers log in here

Literature review current through: Nov 2017. | This topic last updated: Sep 18, 2017.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2017 UpToDate, Inc.
  1. Mahr AD, Jover JA, Spiera RF, et al. Adjunctive methotrexate for treatment of giant cell arteritis: an individual patient data meta-analysis. Arthritis Rheum 2007; 56:2789.
  2. Villiger PM, Adler S, Kuchen S, et al. Tocilizumab for induction and maintenance of remission in giant cell arteritis: a phase 2, randomised, double-blind, placebo-controlled trial. Lancet 2016; 387:1921.
  3. Stone JH, Tuckwell K, Dimonaco S, et al. Trial of Tocilizumab in Giant-Cell Arteritis. N Engl J Med 2017; 377:317.
  4. Jayne D, Rasmussen N, Andrassy K, et al. A randomized trial of maintenance therapy for vasculitis associated with antineutrophil cytoplasmic autoantibodies. N Engl J Med 2003; 349:36.
  5. Pagnoux C, Mahr A, Hamidou MA, et al. Azathioprine or methotrexate maintenance for ANCA-associated vasculitis. N Engl J Med 2008; 359:2790.
  6. Guillevin L, Pagnoux C, Karras A, et al. Rituximab versus azathioprine for maintenance in ANCA-associated vasculitis. N Engl J Med 2014; 371:1771.
  7. Jones RB, Tervaert JW, Hauser T, et al. Rituximab versus cyclophosphamide in ANCA-associated renal vasculitis. N Engl J Med 2010; 363:211.
  8. Stone JH, Merkel PA, Spiera R, et al. Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N Engl J Med 2010; 363:221.
  9. Specks U, Merkel PA, Seo P, et al. Efficacy of remission-induction regimens for ANCA-associated vasculitis. N Engl J Med 2013; 369:417.
  10. De Groot K, Rasmussen N, Bacon PA, et al. Randomized trial of cyclophosphamide versus methotrexate for induction of remission in early systemic antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheum 2005; 52:2461.
  11. Villa-Forte A, Clark TM, Gomes M, et al. Substitution of methotrexate for cyclophosphamide in Wegener granulomatosis: a 12-year single-practice experience. Medicine (Baltimore) 2007; 86:269.
  12. Mukhtyar C, Guillevin L, Cid MC, et al. EULAR recommendations for the management of large vessel vasculitis. Ann Rheum Dis 2009; 68:318.
  13. Mukhtyar C, Guillevin L, Cid MC, et al. EULAR recommendations for the management of primary small and medium vessel vasculitis. Ann Rheum Dis 2009; 68:310.
  14. Hoffman GS. L52. Vasculitis treatment: is it time to change the standard of care for ANCA-associated vasculitis? Presse Med 2013; 42:643.
  15. Wechsler ME, Akuthota P, Jayne D, et al. Mepolizumab or Placebo for Eosinophilic Granulomatosis with Polyangiitis. N Engl J Med 2017; 376:1921.
  16. Grayson PC, Cuthbertson D, Carette S, et al. New features of disease after diagnosis in 6 forms of systemic vasculitis. J Rheumatol 2013; 40:1905.
  17. Luqmani RA, Bacon PA, Moots RJ, et al. Birmingham Vasculitis Activity Score (BVAS) in systemic necrotizing vasculitis. QJM 1994; 87:671.
  18. Stone JH, Hoffman GS, Merkel PA, et al. A disease-specific activity index for Wegener's granulomatosis: modification of the Birmingham Vasculitis Activity Score. International Network for the Study of the Systemic Vasculitides (INSSYS). Arthritis Rheum 2001; 44:912.
  19. Tomasson G, Grayson PC, Mahr AD, et al. Value of ANCA measurements during remission to predict a relapse of ANCA-associated vasculitis--a meta-analysis. Rheumatology (Oxford) 2012; 51:100.
  20. Flossmann O, Berden A, de Groot K, et al. Long-term patient survival in ANCA-associated vasculitis. Ann Rheum Dis 2011; 70:488.
  21. Rhee RL, Hogan SL, Poulton CJ, et al. Trends in Long-Term Outcomes Among Patients With Antineutrophil Cytoplasmic Antibody-Associated Vasculitis With Renal Disease. Arthritis Rheumatol 2016; 68:1711.