Predominantly medium-vessel vasculitis

Predominantly medium-vessel vasculitis is a group classification within the Chapel Hill Consensus Conference nomenclature for systemic vasculitides, encompassing inflammatory diseases that primarily affect medium-sized arteries. The two principal diseases in this category are polyarteritis nodosa (PAN) and Kawasaki disease. These conditions are characterized by inflammation and damage to the walls of medium-sized muscular arteries, which can lead to aneurysm formation, vessel stenosis, thrombosis, and organ ischemia or infarction. Polyarteritis nodosa typically affects adults and can involve the kidneys, gastrointestinal tract, peripheral nerves, skin, and musculoskeletal system. Key symptoms include fever, weight loss, myalgia, skin lesions (livedo reticularis, nodules, ulcers), abdominal pain, hypertension, and mononeuritis multiplex. Kawasaki disease predominantly affects children under five years of age and is characterized by prolonged fever, conjunctival injection, oral mucosal changes, rash, extremity changes, and cervical lymphadenopathy, with the most serious complication being coronary artery aneurysms. Treatment varies by specific disease. Polyarteritis nodosa is typically managed with corticosteroids and immunosuppressive agents such as cyclophosphamide for severe cases, or antiviral therapy when associated with hepatitis B virus infection. Kawasaki disease is treated acutely with intravenous immunoglobulin (IVIG) and high-dose aspirin to reduce the risk of coronary artery complications. Early diagnosis and treatment are critical in both conditions to prevent irreversible organ damage and life-threatening complications.

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