| Small vessel vasculitis
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| Types
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Pathology
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Presentation and laboratory characters
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| Churg-Strauss syndrome
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Necrotizing vasculitis of vessels in the respiratory tract with eosinophil infiltration and granulomatous inflammation
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Allergic rhinitis, asthma, eosinophilia, fever, anorexia, malaise
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| Cutaneous leukocytoclastic angiitis
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Involves skin only
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Palpable purpura, cutaneous infarcts and necrosis, urticaria
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| Essential cryoglobulinemia
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Immune deposits in glomeruli and skin
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Recurrent palpable purpura, polyarthralgia, glomerulonephritis; Cryoglobulins elevated in serum; may be seen in Hep C infection, multiple myeloma
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| Henoch-Schonlein purpura
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IgA immune deposits in skin,joints, gut and glomeruli
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Follows an URTI, recent viral or bacterial infections, food allergies, medications; Arthralgia, arthritis, abdominal pain, red or purple palpable purpura, headache, glomerulonephritis, typically in young children; normal coagulation studies
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| Microscopic polyangiitis
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Necrotizing vasculitis with scanty immune deposits in microscopic vessels namely capillaries, venules or arterioles; lungs and kidneys involved
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Palpable purpura, pulmonary hemorrhages, glomerulonephritis
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| Granulomatosis with polyangiitis (GPA) (formerly called Wegener’s granulomatosis)
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Granulomatous inflammation of the upper and lower respiratory tract with glomerulonephritis
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Nasopharyngeal ulcers, sinusitis, pneumonitis with bilateral nodular infiltrates, crescentic glomerulonephritis, otitis media, hemoptysis
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| Medium vessel vasculitis
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| Kawasaki disease or mucocutaneous lymph node syndrome
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Coronary arteritis
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Seen in young children, mostly in boys, fever, conjunctivitis, desquamation of fingers and toes, cervical lymphadenopathy, erythema of lips and oral mucosa, coronary aneurysms, most common acquired heart disease in children
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| Polyarteritis nodosa
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Necrotizing inflammation of vessels; spares the lung; no glomerulonephritis but renal vessels may be stenosed causing renal ischemia
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Fever, hypertension,weight loss, abdominal pain, melena, peripheral neuritis, renal and organ ischemia; can be seen in Hep B infection
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| Raynaud’s disease
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Vasospasm only, no underlying cause; NO vasculitis; affects fingers, toes, ears, tip of nose; in response to cold exposure, stress
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More common in women, typical changes of Raynaud’s phenomenon seen; no underlying disease; seen in healthy individuals
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| Raynaud’s phenomenon
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Recurrent, reversible vasospasm of fingers and toes on cold exposure or stress; caused by underlying autoimmune disorder; vasculitis and fibrinoid changes in vessel walls
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More common in women, seen in autoimmune disorders like systemic sclerosis, mixed connective tissue disease, SLE, frostbite, cryoglobulinemia, ergotamine; three color changes occur - initially white from vasospasm, then blue from cyanosis and finally red from hyperemia; affected areas are clearly demarcated from unaffected areas; seen in extremities, tip of the nose, ears.
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| Thromboangiitis obliterans or Buerger’s disease
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Inflammation of peripheral arteries and veins leading to stenosis, thrombosis and decreased blood supply to extremities
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Seen in young and middle-aged male, cigarette smokers; affects extremities, more commonly lower legs; extreme pain of the arms and legs while at rest, thrombophlebitis; Raynaud’s phenomenon; ulcers
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| Large vessel vasculitis
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| Giant cell or temporal arteritis
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Granulomatous inflammation of aorta and branches especially temporal artery
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Fever, facial pain , headache, tender scalp, Polymyalgia rheumatica, jaw claudication on chewing
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| Takayasu arteritis
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Granulomatous inflammation of the aorta and major branches
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More common in young Asian and Latin American women; lower BP and weaker pulses in the upper limbs, HT, cold and numb fingers, neurological deficit
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Secondary causes of vasculitis are rheumatoid arthritis, systemic lupus erythematosus, scleroderma, hepatitis B and C infection, HIV, HTLV 1, CMV, VZV, Parvovirus B19, lymphoma, and solid organ malignancy.
Infectious vasculitis is seen in Rocky mountain spotted fever, Neisseria meningitidis septicaemia , syphilitic aortitis, by direct invasion of blood vessels or septic embolization.
*Based on indirect immunofluorescence patterns, there are two types of ANCA - p-ANCA or perinuclear and c-ANCA or cytoplasmic. p-ANCA corresponds to myeloperoxidase , while c-ANCA corresponds to proteinase 3. Other disease conditions like infections, IBD or drug-induced vasculitis can also give a positive ANCA.
