Types of Vasculitis
Vasculitis can be broadly divided into 2 categories: primary or secondary vasculitis. There are over 20 types of primary vasculitis; they are thought to be autoimmune related but the exact cause is generally unknown (1). The etiology of secondary vasculitis includes infection, medication, and other diseases such as lupus and rheumatoid arthritis (1).
The classification of primary vasculitis was updated in 2012 at the International Chapel Hill Consensus Conference (CHCC). CHCC categorized primary vasculitis based on the predominate type of blood vessel involved in the disease: large vessel vasculitis, medium vessel vasculitis, and small vessel vasculitis (2). The definitions of the 3 types of blood vessel are can be found in Figure 1. A key concept is that these 3 categories of primary vasculitis can affect blood vessels of any size (2). For example, ANAC-associated small vessel vasculitis predominately involves small vessels, but they may also affect medium sized vessels as well, as seen in Figure 2.
CHCC’s vasculitis classification system is listed in Table 1. For the description of major primary vasculitis, please visit http://www.nhlbi.nih.gov/health/health-topics/topics/vas/types.html
Figure 1. Types of blood vessels: large vessels include major arteries and veins (A); medium vessels include main visceral arteries and veins; small vessels include arterioles, venules and capillaries.
The classification of primary vasculitis was updated in 2012 at the International Chapel Hill Consensus Conference (CHCC). CHCC categorized primary vasculitis based on the predominate type of blood vessel involved in the disease: large vessel vasculitis, medium vessel vasculitis, and small vessel vasculitis (2). The definitions of the 3 types of blood vessel are can be found in Figure 1. A key concept is that these 3 categories of primary vasculitis can affect blood vessels of any size (2). For example, ANAC-associated small vessel vasculitis predominately involves small vessels, but they may also affect medium sized vessels as well, as seen in Figure 2.
CHCC’s vasculitis classification system is listed in Table 1. For the description of major primary vasculitis, please visit http://www.nhlbi.nih.gov/health/health-topics/topics/vas/types.html
Figure 1. Types of blood vessels: large vessels include major arteries and veins (A); medium vessels include main visceral arteries and veins; small vessels include arterioles, venules and capillaries.
Figure 2. Blood vessel involvement of large vessel vasculitis, medium vessel vasculitis, and small vessel vasculitis. Figure adapted from (2).
Table 1. Major types of primary and secondary vasculitis. Adapted from (2).
PRIMARY VASCULITIS
Large vessel vasculitis (LVV) Takayasu arteritis (TAK) Giant cell arteritis (GCA) Medium vessel vasculitis (MVV) Polyarteritis nodosa (PAN) Kawasaki disease (KD) Small vessel vasculitis (SVV) Antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis (AAV) Microscopic polyangiitis (MPA) Granulomatosis with polyangiitis (Wegener’s) (GPA) Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) Immune complex SVV Anti–glomerular basement membrane (anti-GBM) disease Cryoglobulinemic vasculitis (CV) IgA vasculitis (Henoch-Scho¨nlein) (IgAV) Hypocomplementemic urticarial vasculitis (HUV) (anti-C1q vasculitis) Variable vessel vasculitis (VVV) Behc¸et’s disease (BD) Cogan’s syndrome (CS) Single-organ vasculitis (SOV) Cutaneous leukocytoclastic angiitis Cutaneous arteritis Primary central nervous system vasculitis Isolated aortitis Others |
SECONDARY VASCULITIS
Vasculitis associated with systemic disease Lupus vasculitis Rheumatoid vasculitis Sarcoid vasculitis Others Vasculitis associated with probable etiology Hepatitis C virus–associated cryoglobulinemic vasculitis Hepatitis B virus–associated vasculitis Syphilis-associated aortitis Drug-associated immune complex vasculitis Drug-associated ANCA-associated vasculitis Cancer-associated vasculitis Others |
References
- Vasculitis [Internet]. Atlanta: American College of Rheumatology, 2013 [cited 2013 March 30]. Available from: http://www.rheumatology.org/practice/clinical/patients/diseases_and_conditions/vasculitis.asp
- Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, et al. 2012 Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2013 Jan;65(1):1-11.