Clinical features of systemic vasculitides

The features of a systemic vasculitis may be nonspecific. Although all vasculitic syndromes are rare, suspect a systemic vasculitis in anyone presenting with an unexplained persistent inflammatory state (eg fever, weight loss, night sweats, fatigue, malaise, raised inflammatory markers). Specific clinical features may suggest a particular vasculitic syndrome (see Clinical features of systemic vasculitides), but diagnosis can be complex and up to 40% of people do not fit the classical description for any of the specific vasculitides.

Table 1. Clinical features of systemic vasculitides
[NB1]
Jennette, 2013 Sharma, 2011
Systemic vasculitis	Key clinical features
Large-vessel vasculitides (often affect the aorta or its major branches)
giant cell arteritis	age over 50 years, especially females over 70 years predominantly cranial artery vasculitis jaw claudication, severe headache, scalp tenderness diplopia or visual loss associated with polymyalgia rheumatica systemic symptoms such as weight loss, fever and malaise
Takayasu arteritis	early adulthood, especially females under 50 years aortitis and other large-vessel vasculitis upper-limb claudication absent pulses, arterial bruits, hypertension
Medium-vessel vasculitides (predominantly affect the main visceral arteries and their branches)
Kawasaki disease	almost exclusively in children predominantly coronary artery vasculitis mucocutaneous and lymph node involvement; see Criteria for the diagnosis of Kawasaki disease
polyarteritis nodosa	predominantly in middle-aged adults medium-vessel vasculitis (eg renal and mesenteric arteries) cutaneous features (eg cutaneous and subcutaneous nodules, palpable purpura, livedo reticularis) peripheral polyneuropathy intestinal and liver ischaemia renal vasculopathy, chronic kidney failure
Small-vessel vasculitides (predominantly affect small intraparenchymal arteries, capillaries, venules or arterioles)
ANCA-associated vasculitides
eosinophilic granulomatosis with polyangiitis (formerly known as Churg–Strauss vasculitis)	adult-onset asthma pulmonary eosinophilia, pulmonary infiltrates and diffuse interstitial lung changes blood eosinophilia mononeuritis multiplex palpable purpura, skin infarcts, nodules over pressure areas granulomatous infiltration of the myocardium or coronary vasculitis in up to 50%, myocardial infarction, congestive cardiac failure focal, segmental necrotising glomerulonephritis associated with pANCA (MPO antibodies) in approximately 50%
granulomatosis with polyangiitis (formerly known as Wegener granulomatosis)	ear, nose, throat and lower respiratory tract necrosis and granuloma formation sinusitis, nasal and oral disease, subglottic stenosis otitis media, hearing loss, ear pain pulmonary infiltrates and nodules, haemoptysis, pleuritis glomerulonephritis in 75%, chronic kidney failure ophthalmic extension, orbital mass, necrotising scleritis associated with cANCA (PR3 antibodies)
microscopic polyangiitis	acute, rapidly progressive glomerulonephritis pulmonary haemorrhage palpable purpura associated with pANCA (MPO antibodies)
Immune-complex vasculitis
IgA vasculitis (formerly known as Henoch–Schönlein purpura)	occurs predominantly in children nonthrombocytopenic purpura colicky abdominal pain large-joint arthritis renal vasculopathy, nephropathy
cryoglobulinaemic vasculitis	often secondary to infection, malignancy or connective tissue disease most common features are palpable purpura, arthralgia, arthritis Raynaud phenomenon severe disease may involve kidneys, gastrointestinal system, lungs, and central nervous system
Goodpasture syndrome (anti–glomerular basement membrane disease) [NB2]	very rare autoimmune variant affects adults aged 20 to 30 years; acquired form affects adults aged over 60 years lung (pulmonary haemorrhage) and kidney (glomerulonephritis) involvement
other conditions associated with small-vessel vasculitis	viral infection–associated inflammatory connective tissue disease–associated drug-associated leukocytoclastic
Note: ANCA = antineutrophil cytoplasmic antibody; cANCA = cytoplasmic ANCA; IgA = immunoglobulin A; MPO = myeloperoxidase; pANCA = perinuclear ANCA; PR3 = proteinase 3 NB1: This table concentrates on the systemic vasculitides discussed in this topic; other specific systemic vasculitides have been defined, including vasculitis associated with other inflammatory syndromes (eg Behçet syndrome, which is discussed in a separate topic). NB2: Goodpasture syndrome (anti–glomerular basement membrane disease) is not covered further in Rheumatology guidelines.