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Emergency and Acute Medicine: Vasculitis
Vasculitis refers to a heterogeneous group of disorders characterized by inflammation of blood vessel walls, leading to ischemia, necrosis, aneurysm formation, or hemorrhage. The underlying mechanisms include immune complex deposition, complement activation, cell-mediated hypersensitivity, and granulomatous inflammation. Because vessels of varying sizes and organs may be involved, vasculitis often presents as a multisystem disease with diverse clinical manifestations.
Vasculitis may be primary (idiopathic) or secondary to infections, drugs, autoimmune diseases, or malignancy. Classification is increasingly based on antineutrophil cytoplasmic antibodies (ANCA), though traditional classification divides vasculitis by vessel size. Large-vessel vasculitides include Giant cell arteritis, typically affecting patients older than 50 years, and Takayasu arteritis, which affects younger individuals. Medium-vessel diseases include Polyarteritis nodosa and Kawasaki disease, the latter primarily affecting children and involving coronary arteries. Small-vessel vasculitides include Granulomatosis with polyangiitis, microscopic polyangiitis, and eosinophilic granulomatosis (Churg–Strauss), often associated with pulmonary and renal involvement.
Clinically, patients often present initially with constitutional symptoms such as fever, fatigue, weight loss, and diffuse myalgias before organ-specific manifestations appear. Signs of arterial insufficiency depend on the vascular territory involved. Neurologic symptoms may include headache, transient ischemic attacks, stroke, or vision loss. Renal involvement may manifest as hypertension, hematuria, or renal failure. Cutaneous findings are common and include palpable purpura, ulcers, nodules, and livedo reticularis. Respiratory symptoms such as sinusitis, epistaxis, and pulmonary hemorrhage may be seen in small-vessel vasculitis. Gastrointestinal ischemia may present with abdominal pain or bleeding.
Diagnosis requires a high index of suspicion, particularly in patients with unexplained multisystem disease. Laboratory evaluation typically reveals elevated inflammatory markers (ESR, CRP), anemia, leukocytosis, and sometimes eosinophilia. Urinalysis may show proteinuria or hematuria, suggesting renal involvement. Autoimmune testing including ANA and ANCA can help guide diagnosis. Imaging studies such as CT, MRI, or angiography may demonstrate vessel abnormalities, while definitive diagnosis often requires tissue biopsy.
Management depends on the specific type and severity of vasculitis and is usually guided by a specialist. Initial emergency care focuses on stabilizing life-threatening complications such as stroke, renal failure, or pulmonary hemorrhage. Corticosteroids are the cornerstone of treatment for many vasculitides, particularly giant cell arteritis, where prompt treatment is critical to prevent vision loss. Immunosuppressive agents such as cyclophosphamide, methotrexate, or azathioprine are used in more severe or refractory cases. Intravenous immunoglobulin (IVIG) is used in conditions like Kawasaki disease, often combined with aspirin to reduce coronary complications.
Patients with severe disease or evidence of end-organ damage require hospital admission. Those with milder symptoms may be managed as outpatients but require urgent referral to a rheumatologist for further evaluation and initiation of definitive therapy. Close follow-up is essential, as untreated vasculitis can lead to irreversible organ damage or death.
Key clinical considerations include recognizing vasculitis as a cause of unexplained systemic illness, avoiding premature immunosuppressive therapy without specialist input, and ruling out mimics such as Infective endocarditis. Early diagnosis and targeted treatment are critical to improving outcomes in these potentially life-threatening conditions.
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