Overview of the management of vasculitis in adults
- Peter A Merkel, MD, MPH
Peter A Merkel, MD, MPH
- Section Editor — Vasculitis
- Chief, Division of Rheumatology
- University of Pennsylvania
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".)
APPROACH TO TREATMENT
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.
- 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.
- 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.
- Pagnoux C, Mahr A, Hamidou MA, et al. Azathioprine or methotrexate maintenance for ANCA-associated vasculitis. N Engl J Med 2008; 359:2790.
- Guillevin L, Pagnoux C, Karras A, et al. Rituximab versus azathioprine for maintenance in ANCA-associated vasculitis. N Engl J Med 2014; 371:1771.
- 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.
- 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.
- Jones RB, Tervaert JW, Hauser T, et al. Rituximab versus cyclophosphamide in ANCA-associated renal vasculitis. N Engl J Med 2010; 363:211.
- Stone JH, Merkel PA, Spiera R, et al. Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N Engl J Med 2010; 363:221.
- Mukhtyar C, Guillevin L, Cid MC, et al. EULAR recommendations for the management of large vessel vasculitis. Ann Rheum Dis 2009; 68:318.
- 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.
- Hoffman GS. L52. Vasculitis treatment: is it time to change the standard of care for ANCA-associated vasculitis? Presse Med 2013; 42:643.
- 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.
- Luqmani RA, Bacon PA, Moots RJ, et al. Birmingham Vasculitis Activity Score (BVAS) in systemic necrotizing vasculitis. QJM 1994; 87:671.
- 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.
- 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.
- Flossmann O, Berden A, de Groot K, et al. Long-term patient survival in ANCA-associated vasculitis. Ann Rheum Dis 2011; 70:488.