Vasculitis - Medium and Variable Vessel Sizes

Vasculitides are characterized by blood vessel inflammation with possible necrosis, ischemia, and organ damage. The vessels and organs affected vary by the specific vasculitic disorder. General symptoms and signs are due to systemic inflammation, which can cause fever, arthritis, arthralgia, fatigue, and weight loss. Skin lesions are associated with small and medium vessel vasculitides. Treatments include corticosteroids and immunosuppressants. Polyarteritis nodosa is characterized by necrotizing inflammation that most commonly involves the medium muscular arteries, especially at their branch points. The resulting ischemia can affect multiple organ systems: – Nervous system involvement often produces mononeuritis multiplex or asymmetric polyneuropathy; sensory and motor deficits of the median, ulnar, and fibular nerves are common. – Renal involvement can lead to hypertension, oliguria, and even renal failure. – Gastrointestinal involvement can produce pain and malabsorption; when larger vessels, such as the celiac trunk or its branches are affected, fatal aneurysms can develop. – Skin manifestations can take various forms, including livedo reticularis (which presents as a purplish lace-like pattern), ulcers, subcutaneous nodules, or even gangrene. – Heart failure can occur due to coronary artery obstruction. Polyarteritis nodosa can be systemic or cutaneous. Some secondary forms are related to hepatitis B and C infections, or to rheumatoid arthritis. Most commonly affects men over 50 years of age. Kawasaki disease (aka, mucocutaneous lymph node syndrome) is characterized by proliferative inflammation, and tends to involve the medium muscular arteries. It has a predilection for the coronary arteries, which are affected in approximately 20% of cases Key cause of acquired heart disease in children. Large coronary aneurysms can be fatal, as they can cause myocarditis, cardiac tamponade, or, as we've illustrated here, coronary artery thrombosis. Typically presents as a self-limiting, acute febrile illness in children: – Fever – Conjunctivitis – Erythematous macular rash – Edema and desquamation, particularly of the limbs – Cervical lymphadenopathy – A red, inflamed throat – Strawberry tongue with dry, fissured lips. Behçet disease is a chronic, relapsing vasculitis that affects vessels of all sizes. Mucosal inflammation is a common manifestation of the disorder, and often causes oral and genital lesions; ocular inflammation can produce uveitis or hypopyon. Cutaneous manifestations vary, and include pus-filled bumps, palpable purpura, erythema nodosum, and superficial thrombophlebitis. Typical age of onset is around 20 years old. Behçet disease is equally common in men and women, though it's often worse in men. Characterized by inflammation and occlusive thrombosis of the medium and small arteries and veins. Most commonly affects the extremities; signs and symptoms typically begin distally and move proximally. Ischemia from vessel occlusion often produces numbness, coldness, or tingling in the extremities, with claudication, then, pain at rest. Affected limbs are often cold, sweaty, and cyanotic, and ulcers form that progress to gangrenous. Smoking is a major cause of Buerger disease, and smoking cessation is key to remission. Histopathology reveals neutrophil infiltration and granulomatous formation resulting in vessel occlusion with relative sparing of the vessel wall. Arteriography may show a classic "corkscrew" appearance. See: Corkscrew Collaterals in Thromboangitis Obliterans (Buerger's Disease).