- Clinical science
Vasculitides are a heterogeneous group of autoimmune diseases, all characterized by inflammation of blood vessels (vasculitis) and subsequent ischemia and damage to the organs supplied by these vessels. Vasculitis may occur as a primary disease (idiopathic) or as a secondary response to an underlying disease (e.g., hepatitis B infection). While the inflammatory process may be confined to one organ, it may also involve several organ systems. Vasculitis should be considered in patients presenting with palpable purpura, pulmonary infiltrates, unexplained ischemic events, and multisystem disease. The detection of antineutrophil cytoplasmic antibodies (ANCA) in the blood is an important diagnostic marker; however, there are also ANCA-negative vasculitis syndromes. Immunosuppressive treatment is implemented to stop vascular inflammation. Specific (e.g., antiviral drugs) or symptomatic (e.g., NSAID) management may be necessary. If the vasculitis is secondary to an underlying disease, appropriate treatment of the underlying disease should be initiated.
See giant cell arteritis for more information.
- Definition: granulomatous inflammation of the aorta and its major branches, resulting in stenosis of involved blood vessels and subsequent vascular symptoms
- Peak incidence: 15–45 years of age
- Asian heritage
- ♀ > ♂ = 3:1
- Clinical features
- Laboratory findings: ↑ ESR
- Angiography (gold standard): detects vascular stenosis
- Biopsy of affected vessel: granulomatous thickening of aortic arch; plasma cells and lymphocytes in media and adventitia; vascular fibrosis
- A diagnosis of Takayasu arteritis requires three or more of the following diagnostic criteria to be fulfilled. :
- Abnormal arteriography of the aorta or large blood vessels in the extremities that is not due to arteriosclerosis or fibromuscular dysplasia
- Bruit audible over subclavian artery or abdominal aorta
- Decreased brachial artery pulse
- Blood pressure difference > 10 mm Hg between arms
- Claudication of upper or lower extremities while in use
- Age of onset: < 40 years
See Kawasaki disease for more information.
Definition: systemic vasculitis of the small and medium-sized vessels, which leads to tissue ischemia; most commonly involving skin, peripheral nerves, muscles, joints, gastrointestinal tract, and kidneys
- Peak incidence: ∼ 45–65 years
- Sex: ♂ > ♀
- Often associated with hepatitis B or C infections
- Nonspecific symptoms: fever, abdominal, muscle, and joint pain
- Renal involvement; (∼ 60%): hypertension, renal impairment
- Coronary artery involvement; (∼ 35%); increased risk of myocardial infarction
- Skin involvement; (∼ 40%): rash, ulcerations, nodules
- Neurological involvement: polyneuropathy (mononeuritis multiplex), cerebral ischemia (stroke)
- GI involvement: abdominal pain, nausea, vomiting
- Usually spares the lungs
- Blood tests
- Urine analysis: proteinuria, hematuria
- Muscle biopsy: transmural inflammation of the arterial wall with leukocytic infiltration and fibrinoid necrosis
- Angiography: aneurysms and stenosis of small and medium-sized vessels of the involved organs
See thromboangiitis obliterans for more information.
See granulomatosis with polyangiitis for more information.
- Definition: : a multisystem disease characterized by a necrotizing granulomatous vasculitis with eosinophilia; , which most commonly involves the lungs and the skin, but can also affect the renal, cardiovascular, gastrointestinal, central, and peripheral nervous systems.
- Etiology: unknown
- Clinical features
Treatment: immunosuppression with glucocorticoids; possible in combination with cyclophosphamide
- Low dose prednisolone may be required to manage persistent asthma.
- Definition: necrotizing vasculitis of small vessels; , typically with renal and pulmonary involvement; however, multiple organ systems may be affected
- Clinical features: similar to that of , but without involvement of the nasopharynx!
- Treatment: immunosuppression with corticosteroids and cyclophosphamide
(Immunoglobulin A (IgA) vasculitis)
See Henoch-Schönlein purpura for more information.
- Definition: cryoglobulin-mediated vasculitis; characterized by temperature dependent deposition of immune complexes in blood vessel walls and subsequent inflammation of involved vessels and surrounding tissue
Etiology: type II and III cryoglobulinemia (mixed cryoglobulinemia)
- > 90% association with hepatitis C infection
- Other underlying diseases: multiple myeloma, lymphoproliferative disorders, connective tissue diseases, autoimmune diseases (SLE), proliferative glomerulonephritis
- Clinical features
- Laboratory findings:
- Skin or renal biopsy: evaluate inflammatory vascular changes and renal damage; cryoglobulin deposits may be detected in glomeruli.
- Investigation of underlying cause: WBC, autoimmune antibodies, liver function testing, renal function testing, imaging (angiography, CT, chest x-ray)
- Treatment of underlying diseases: hepatitis C infection (IFN-α, ribavirin)
- Immunosuppression: Corticosteroids and/or cyclophosphamide are indicated in severe cases of organ involvement (renal, neurological).
- NSAIDs may be administered to manage arthralgia and myalgia.
- Plasmapheresis with simultaneous corticosteroid therapy to reduce the production of immunoglobulin
- Initial uncontrolled studies of rituximab therapy in HCV-induced cryoglobulinemic vasculitis cases have been effective in controlling clinical symptoms.
- Definition: necrotizing vasculitis of cutaneous small vessels
- Etiology: drug-induced; infections
- Clinical features:
- Skin biopsy
- Investigation of underlying cause
- Systemic vasculitis should be ruled out.
- Treatment: symptomatic; discontinue drug intake; immunosuppression may be required
- Definition: a systemic vasculitis that can affect arteries and veins of all sizes
- Most common from the Mediterranean region to eastern Asia, with the highest prevalence observed in Turkey and Japan
- Strong HLA-B51 association
- Peak incidence: 20–40 years
- Pathophysiology: not completely understood
Clinical features 
Recurrent painful oral ulceration (95–100%): multiple minor aphthous ulcers, major aphthous ulcers, and/or herpetiform ulcers
- Typically the initial presenting symptom
Recurrent genital ulceration
- Single or multiple ulcers that resemble oral aphthous ulcers and heal with scarring
- ♀: vulva (most common), vagina
- ♂: scrotum (most common), penis
Ocular disease (50–80%): uveitis (, ), conjunctivitis, keratitis, papillitis, and/or retinal vasculitis
- Typically bilateral
- More common and more severe among men
- Usually occurs 2–3 years after the onset of oral and/or genital ulceration
- Initial presenting feature of Behçet disease in 10–20% of cases
- Skin lesions (35–85%): , , and/or papulopustular lesions (pseudofolliculitis or acneiform eruptions)
- Arthritis (40–50%): non-erosive, non-deforming, asymmetric mono-/oligoarthritis of knees, ankles, hands, and/or wrists
- Gastrointestinal disease (< 25%): ileocecal ulceration → abdominal pain, anorexia, diarrhea, lower GI bleeding, nausea, vomiting
Neuro-Behçet syndrome (5–10%)
- Parenchymal CNS disease: behavioral changes, ataxia, hemiparesis, sudden hearing loss
- Extra-parenchymal CNS disease: ,
- Recurrent painful oral ulceration (95–100%): multiple minor aphthous ulcers, major aphthous ulcers, and/or herpetiform ulcers
Behçet disease triad: oral ulcers, genital ulcers, and uveitis!
- Diagnostics 
- Diagnostic criteria (International Study Group criteria)
- Differential diagnosis
- Oral ulcers and/or genital ulcers: topical corticosteroids ± topical lidocaine for pain relief
- Skin lesions
- Arthritis: colchicine
Ocular disease, CNS disease, and/or vasculopathy
(e.g., , methotrexate, , , , )
- The treatment regimen for ocular disease should include azathioprine.
- The treatment regimen for pulmonary artery and/or peripheral artery aneurysms should include cyclophosphamide.
- Cyclosporine should not be used in a patient with evidence of CNS disease except when therapy for ocular disease is considered.
- Systemic corticosteroids
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