Large Vessel Vasculitis

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Takayasu's Arteritis

The ACR classification criteria describe the characteristic features of sub-clavian or aortic bruit, age <40 years at onset, decreased brachial artery pulse, blood pressure difference of >10 mm Hg between arms, claudication of extremities, and arteriographic evidence of narrowing or occlusion of aorta, its primary branches, or large arteries in the proximal, upper or lower extremities (21). In Japan almost a fifth of cases occur in 10 to 19 year olds

Table 2 Nomenclature of Systemic Vasculitis

Annual incidence/106 population (Ref.)

Large vessel vasculitis

Giant cell (temporal)

Granulomatous arteritis of the aorta

178 in adults


and its major branches, with a

>50 years old

predilection for the extracranial


branches of the carotid artery


Granulomatous inflammation of the aorta and its major branches

2.6 in adults (15)

Medium vessel vasculitis


Necrotising inflammation of medium-sized or small arteries without glomerulonephritis or vasculitis in arterioles, capillaries or venules

9 in adults (16)


Arteritis involving large, medium-

900 in Japanese

sized and small arteries, and

<5 years old

associated with mucocutaneous


lymph node syndrome

55 in U.K. children (18)

Small vessel vasculitis


Granulomatous inflammation involving the respiratory tract, and necrotising vasculitis affecting small to medium-sized vessels (e.g. capillaries, venules, arterioles and arteries)

6 in adults (19)


Eosinophil-rich and granulomatous inflammation involving the respiratory tract, necrotising vasculitis affecting small to medium sized vessels, and associated with asthma and eosinophilia


Necrotising vasculitis, with few or no immune deposits, affecting small vessels (i.e. capillaries, venules or arterioles)


Vasculitis, with IgA-dominant

12 in adults (20)

immune deposits, affecting small

204 in children

vessels (i.e. capillaries, venules or




Table 2 Nomenclature of Systemic Vasculitis (Continued)

Annual incidence/106 population (Ref.)

Essential cryoglobulinemia vasculitis

Vasculitis, with cryoglobulin immune deposits, affecting small vessels (i.e., capillaries, venules, or arterioles) and associated cryoglobulins in serum

Cutaneous leukocytoclastic vasculitis

Isolated cutaneous leukocytoclastic angiitis without systemic vasculitis or glomerulonephritis

Note: Large vessel refers to the aorta and the largest branches directed towards the body; medium vessel refers to the main visceral arteries (e.g., renal, hepatic, coronary, and mesenteric arteries); small vessel refers to venules, capillaries, arterioles, and the intraparenchymal distal arterial radicals that connect with arterioles. a Strongly associated with antineutrophil cytoplasmic antibodies.

Abbreviations: CSS, Churg-Strauss syndrome; HSP, Henoch-Schonlein purpura; KD, Kawasaki disease; MPA, microscopic polyangiitis; PAN, polyarteritis nodosa; TA, Takayasu's arteritis; WG, Wegener's granulomatosis. Source: Adapted from Ref. 13.

(22) and in the series used to develop the ACR classification criteria, 29% of patients were in this age bracket. In Japanese series a female to male ratio of 9:1 is reported.

The clinical presentation is classically that of an initial non-specific inflammatory disease, before going onto the "pulseless" vascular phase (23). However, the lack of constitutional symptoms and presenting features of hypertension and cardiac failure has been highlighted (24,25). There are no specific laboratory tests. Vascular imaging and histopathological examination may be extremely helpful and indeed essential to diagnosis. It should be noted that a considerable delay in diagnosis has been described in the adolescent population compared to adults (26,27), illustrating the importance of pediatric and adult rheumatologists being alert to this diagnosis. The value of early diagnosis in underscored by the observation that prognosis closely correlates to the initiation of therapy from onset of symptoms (24). Therapy consists of high dose corticosteroids (1 mg/kg/day of prednisolone) (25), tapering once in remission. In the absence of achieving or maintenance of remission, further immunosuppression should be considered, with the best evidence available for the use of methotrexate (28) and cyclophosphamide (29). The use of other immunosuppressive drugs, e.g., azathioprine and the use of immunosuppressive agents in addition to corticosteroids at diagnosis remains controversial. Revascularization of symptomatic occluded vessels

Table 3 Causes of Secondary Vasculitis and Mimics of Vasculitis






Mycobacterial Spirochetal


Autoimmune rheumatic disease

Malignancy Drugs

Thromboembolic Others

Invasive infective arteritis secondary to organisms such as staphylococcus, streptococcus, salmonella, and neiserria Hepatitis B-associated PAN Hepatitis C-associated cryoglobulinaemia CMV, infectious mononucleosis, and rubella may be associated with a small vessel vasculitis HIV may be associated with a small to medium-sized vessel vasculitis

Aspergillus may be associated with a medium vessel vasculitis and coccidiomycosis with a large vessel vasculitis in immunocompromised patients Tuberculosis may be associated with a vasculitis of any vessel size but typically affects small vessels and venules in particular.

Leprosy may cause a vasculopathy, perivasculitis, and, only rarely, a vasculitis. Syphilis is typically associated with a large to medium vessel arteritis and gonorrhoea with a small vessel vasculitis.

Lyme disease is rarely associated with a small vessel vasculitis.

Rocky Mountain spotted fever, epidemic typhus, and scrub typhus may produce a small vessel vasculitis. Inflammatory arthritis (especially rheumatoid factor positive and systemic JIA) Dermatomyositis Systemic lupus erythematosus Sjogren's syndrome

A small-to-medium vessel has been reported in many malignancies. Prescription medications rarely cause a small vessel vasculitis.

Substance abuse causing predominantly a central nervous system vasculitis has been described with amphetamines, heroin, methylphenidate, etc. Bacterial endocarditis Cardiac myxoma Antiphospholipid syndrome

Inflammatory bowel disease, serum sickness, transplantand radiation-associated vasculitis

Abbreviations: JIA, juvenile idiopathic arthritis; PAN, polyarteritis nodosa.

may be undertaken. The reported five-year mortality varies considerably between case series with reports of 94% in North America (25) and 35% in Mexico (24). Considerable morbidity occurs with difficulty in activities of daily living in 74%, and permanent disability in 47% (25). Pregnancy in patients with Takayasu's arteritis is usually successful (25).

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