Overview of and approach to the vasculitides 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 inflammatory leukocytes in vessel walls with reactive damage to mural structures. Both loss of vessel integrity leading to bleeding, and compromise of the lumen may result in downstream tissue ischemia and necrosis. In general, affected vessels vary in size, type, and location in association with the specific type of vasculitis. Vasculitis may occur as a primary process or may be secondary to another underlying disease. The exact pathogenetic mechanisms underlying these diseases are unknown.
The vasculitides are often serious and sometimes fatal diseases that require prompt recognition and therapy. Symptomatic involvement of affected organs may either occur in isolation or in combination with multiple organs. The distribution of affected organs may suggest a particular type of vasculitis.
This topic will review the nomenclature of the different vasculitides and provide an overview of the approach to the patient with suspected vasculitis. An overview of the treatment of these disorders and detailed discussions of the individual disorders are presented separately. (See "Overview of the management of vasculitis in adults".)
The disease names and definitions of the vasculitides continue to evolve as our understanding of the pathogenesis advances. The international Chapel Hill Consensus Conference (CHCC) has developed one of the most widely used nomenclature systems which specifies the names and definitions for most forms of vasculitis [1,2]. The CHCC nomenclature system has changed over the past few decades, and definitions that were put forth by the CHCC in 1994 have since been revised in the 2012 CHCC (table 1 and table 2).
Among the notable changes in the 2012 CHCC was the preferential use and adoption of new names for several diseases, consistent with the trend of replacing eponyms with disease names that reflect an increased pathophysiologic understanding of these conditions. Among the name changes are: eosinophilic granulomatosis with polyangiitis (Churg-Strauss), abbreviated EGPA, in place of Churg-Strauss syndrome; granulomatosis with polyangiitis (Wegener's), abbreviated GPA, in place of Wegener’s granulomatosis; immunoglobulin A (IgA) vasculitis (Henoch-Schönlein), abbreviated as IgAV, in place of Henoch-Schönlein purpura (HSP); anti-C1q vasculitis as an alternative name for hypocomplementemic urticarial vasculitis, abbreviated HUV; and use of the term “cryoglobulinemic vasculitis” in place of “essential cryoglobulinemic vasculitis.” Furthermore, the 2012 CHCC formally adopted the term antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) for the group of three disorders that include microscopic polyangiitis (MPA), GPA, and EGPA, with additional categories also named to describe variable-vessel vasculitis and secondary forms of vasculitis. This nomenclature system is not meant to substitute for classification criteria, which include clinical observations that classify a specific patient into a category for a research purposes. (See 'Classification criteria' below.)
- Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum 2013; 65:1.
- Jennette JC, Falk RJ, Andrassy K, et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum 1994; 37:187.
- Gomard-Mennesson E, Landron C, Dauphin C, et al. Kawasaki disease in adults: report of 10 cases. Medicine (Baltimore) 2010; 89:149.
- Watts RA, Suppiah R, Merkel PA, Luqmani R. Systemic vasculitis--is it time to reclassify? Rheumatology (Oxford) 2011; 50:643.
- Hunder GG, Arend WP, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of vasculitis. Introduction. Arthritis Rheum 1990; 33:1065.
- Leavitt RY, Fauci AS, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of Wegener's granulomatosis. Arthritis Rheum 1990; 33:1101.
- Masi AT, Hunder GG, Lie JT, et al. The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum 1990; 33:1094.
- Lightfoot RW Jr, Michel BA, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of polyarteritis nodosa. Arthritis Rheum 1990; 33:1088.
- Arend WP, Michel BA, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of Takayasu arteritis. Arthritis Rheum 1990; 33:1129.
- Hunder GG, Bloch DA, Michel BA, et al. The American College of Rheumatology 1990 criteria for the classification of giant cell arteritis. Arthritis Rheum 1990; 33:1122.
- Rao JK, Allen NB, Pincus T. Limitations of the 1990 American College of Rheumatology classification criteria in the diagnosis of vasculitis. Ann Intern Med 1998; 129:345.
- Watts R, Lane S, Hanslik T, et al. Development and validation of a consensus methodology for the classification of the ANCA-associated vasculitides and polyarteritis nodosa for epidemiological studies. Ann Rheum Dis 2007; 66:222.
- Craven A, Robson J, Ponte C, et al. ACR/EULAR-endorsed study to develop Diagnostic and Classification Criteria for Vasculitis (DCVAS). Clin Exp Nephrol 2013; 17:619.
- Luqmani RA, Suppiah R, Grayson PC, et al. Nomenclature and classification of vasculitis - update on the ACR/EULAR diagnosis and classification of vasculitis study (DCVAS). Clin Exp Immunol 2011; 164 Suppl 1:11.
- Niles JL, Böttinger EP, Saurina GR, et al. The syndrome of lung hemorrhage and nephritis is usually an ANCA-associated condition. Arch Intern Med 1996; 156:440.
- Grayson PC, Tomasson G, Cuthbertson D, et al. Association of vascular physical examination findings and arteriographic lesions in large vessel vasculitis. J Rheumatol 2012; 39:303.
- Bateman H, Rehman A, Valeriano-Marcet J. Vasculitis-like Syndromes. Curr Rheumatol Rep 2009; 11:422.
- Molloy ES, Langford CA. Vasculitis mimics. Curr Opin Rheumatol 2008; 20:29.
- MAJOR CATEGORIES OF VASCULITIS
- Large-vessel vasculitis
- - Takayasu arteritis
- - Giant cell arteritis
- Medium-vessel vasculitis
- - Polyarteritis nodosa
- - Kawasaki disease
- Small-vessel vasculitis
- - ANCA-associated vasculitis
- Microscopic polyangiitis
- Granulomatosis with polyangiitis (Wegener’s)
- Eosinophilic granulomatosis with polyangiitis (Churg-Strauss)
- - Immune complex small-vessel vasculitis
- Anti-glomerular basement membrane disease
- Cryoglobulinemic vasculitis
- IgA vasculitis (Henoch-Schönlein purpura)
- Hypocomplementemic urticarial vasculitis (anti-C1q vasculitis)
- Variable-vessel vasculitis
- - Behçet’s syndrome
- - Cogan's syndrome
- Single-organ vasculitis
- - Primary central nervous system vasculitis
- Vasculitis associated with systemic disease
- Vasculitis associated with probable etiology
- CLASSIFICATION CRITERIA
- CLINICAL FEATURES SUGGESTIVE OF SYSTEMIC VASCULITIS
- Diagnostic approach
- - History
- - Physical examination
- - Laboratory tests
- - Additional tests
- - Biopsy
- - Vascular imaging
- DIFFERENTIAL DIAGNOSIS
- INFORMATION FOR PATIENTS
- SUMMARY AND RECOMMENDATIONS