Vasculitis: Vasculitis means inflammation of blood vessels. Most types are caused by immunological or autoimmune mechanisms. Blood vessel damage in vasculitis can occur due to:
The end result is often narrowing of the vessel lumen, which reduces blood flow and causes ischemia in the tissue supplied by that vessel. If the vessel wall becomes weakened, it can lead to aneurysm formation and hemorrhage.
Many forms of vasculitis share general symptoms such as fever, rash, myalgia, malaise, arthralgia, and weight loss. Some types have more characteristic presentations, as listed below.
| Small vessel vasculitis | ||
| Types | Pathology | Presentation and laboratory characters |
| Churg-Strauss syndrome | Necrotizing vasculitis of vessels in the respiratory tract with eosinophil infiltration and granulomatous inflammation | Allergic rhinitis, asthma, eosinophilia, fever, anorexia, malaise |
| Cutaneous leukocytoclastic angiitis | Involves skin only | Palpable purpura, cutaneous infarcts and necrosis, urticaria |
| Essential cryoglobulinemia | Immune deposits in glomeruli and skin | Recurrent palpable purpura, polyarthralgia, glomerulonephritis; cryoglobulins elevated in serum; may be seen in Hep C infection, multiple myeloma |
| Henoch-Schonlein purpura | IgA immune deposits in skin, joints, gut and glomeruli | 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 |
| Microscopic polyangiitis | Necrotizing vasculitis with scanty immune deposits in microscopic vessels namely capillaries, venules or arterioles; lungs and kidneys involved | Palpable purpura, pulmonary hemorrhages, glomerulonephritis |
| Granulomatosis with polyangiitis (GPA) (formerly called Wegener’s granulomatosis) | Granulomatous inflammation of the upper and lower respiratory tract with glomerulonephritis | Nasopharyngeal ulcers, sinusitis, pneumonitis with bilateral nodular infiltrates, crescentic glomerulonephritis, otitis media, hemoptysis |
| Medium vessel vasculitis | ||
| Kawasaki disease or mucocutaneous lymph node syndrome | Coronary arteritis | 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 |
| Polyarteritis nodosa | Necrotizing inflammation of vessels; spares the lung; no glomerulonephritis, but renal vessels may be stenosed causing renal ischemia | Fever, hypertension, weight loss, abdominal pain, melena, peripheral neuritis, renal and organ ischemia; can be seen in Hep B infection |
| Raynaud’s disease | Vasospasm only, with no underlying cause; NO vasculitis; affects fingers, toes, ears, tip of nose; triggered by cold exposure or stress | More common in women; typical changes of Raynaud’s phenomenon; no underlying disease; seen in healthy individuals |
| Raynaud’s phenomenon | Recurrent, reversible vasospasm of fingers and toes on cold exposure or stress; caused by an underlying autoimmune disorder; vasculitis and fibrinoid changes in vessel walls | 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. |
| Thromboangiitis obliterans or Buerger’s disease | Inflammation of peripheral arteries and veins leading to stenosis, thrombosis and decreased blood supply to extremities | 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 |
| Large vessel vasculitis | ||
| Giant cell or temporal arteritis | Granulomatous inflammation of aorta and branches especially temporal artery | Fever, facial pain, headache, tender scalp, polymyalgia rheumatica, jaw claudication on chewing |
| Takayasu arteritis | Granulomatous inflammation of the aorta and major branches | 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 |
Secondary causes of vasculitis include 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, and syphilitic aortitis, due to direct invasion of blood vessels or septic embolization.
| Lab finding | Interpretation |
| Eosinophilia | Churg-Strauss syndrome |
| Raised ESR | Any active vasculitis; Giant cell arteritis |
| Raised liver enzymes | Vasculitis affecting the liver e.g. polyarteritis nodosa |
| ANCA* | c-ANCA Wegener’s granulomatosis, p-ANCA microscopic polyangiitis and Churg-Strauss syndrome |
| X-ray Chest | Nonspecific patchy infiltrates and nodules; pleural effusion; cardiomegaly |
| Angiography | Vascular occlusion, aneurysms; seen in renal and mesenteric vessels in polyarteritis nodosa; in coronary arteries in Takayasu’s arteritis and Kawasaki disease |
| Echocardiography | Coronary artery aneurysms, coronary artery ectasia(Kawasaki disease) |
| CT scan | Nodules, ulcerations in nasal mucosa in Wegener’s granulomatosis |
*Based on indirect immunofluorescence patterns, there are two types of ANCA - p-ANCA (perinuclear) and c-ANCA (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.
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