Snapshot A patient presents with a fever, diffuse myalgias, abdominal pain, a peripheral neuropathy, as well as hypertension and a recent weight loss of 10 pounds. The patient has a past medical history of hepatitis B and C. Angiography demonstrates a "string of pearls appearance" in the renal artery, as well as in several other organ systems with the exception of the pulmonary arteries. Polyarteritis Nodosa Introduction immune complex-mediated transmural vasculitis with fibrinoid necrosis involves renal, coronary, and mesenteric arteries does not involve the pulmonary arteries lesions of different ages (the rationale being that circulating immune complexes deposit at different times) Symptoms flu-like symptoms fever, malaise, and myalgia GI symptoms weight loss, abdominal pain, melena hypertension neurologic dysfunction cutaneous eruptions Findings hepatitis B (HBsAg) seropositivity in 30% of patients multiple aneurysms and constrictions on arteriogram segmental ischemic necrosis with lesions of different ages increased inflammatory markers like ESR and CRP Treatment corticosteroids cyclophosphamide Snapshot A 7-year-old male presents with conjunctival injection and a rash that has persisted for quite some time. On physical exam, the patient has a bright red tongue, adenopathy, and hepatosplenomegaly as well as a fever that has persisted for the past 7 days. Kawasaki Disease (Mucocutaneous Lymph Node Syndrome) Introduction acute, self-limiting necrotizing vasculitis coronary arteries often affected (thrombosis and aneurysm) seen in infants and children leading cause of death from acquired heart disease in children association with Asian ethnicity Presentation fever conjunctivitis cervical lymphadenitis desquamative skin rash with changes in lips/oral mucosa ("strawberry tongue") myocardial infarction (if coronary arteries affected) crash and burn conjunctivitis rash adenopathy strawberry tongue hands (peeling skin) fever Treatment IV immunoglobulin aspirin vaccinations (do not want the patient to get a viral infection due to aspirin treatment) Snapshot A 69-year-old gentleman presents with painful discoloration and ulceration of his fingers. The patient's symptoms have been slowly worsening over the past several months. This patient has smoked 3 packs of cigarettes per day for the past 40 years. Thromboangiitis Obliterans (Buerger Disease) Introduction vasculitis with digital vessel thrombosis seen in heavy smokers, most commonly in men between ages 25 and 50 thought to be caused by a hypersensitivity reaction to components of tobacco smoke Presentation intermittent claudication painful ulceration due to the involvement of nerve and vessel Raynaud's phenomenon may lead to gangrene and autoamputation of digits Treatment smoking cessation cilostazol iloprost calcium channel blockers (nifedipine) Snapshot A 49-year-old woman with abnormally smooth skin on her face and hands presents with a chief concern of episodes of intense pain in her hands. She states that her hands at times will turn pale, then will flush red after a short period of time. She describes the episodes as very painful and unpleasant. Raynaud's Disease Introduction vasculitis affecting vessels of fingers and toes ↑↑ vasomotor reaction to cold/stress seen in young women Presentation digital color changes (white to blue to red) chronic cases may progress to ulceration and gangrene Diagnosis nail fold capillaroscopy Treatment calcium channel blockers (nifedipine, amlodipine) sildenafil (phosphodiesterase inhibitor) Raynaud's Phenomenon Introduction vasculitis affecting vessels of fingers and toes the phenomenon as result of an underlying disease e.g., CREST syndrome and systemic sclerosis affects adults (no gender specificity) Presentation digital color changes chronic cases may progress to ulceration and gangrene Treatment lifestyle and general avoid triggers such as cold and stress cessation of smoking avoid medications that can precipitate attacks such as beta-blockers and ergotamines medical calcium channel blockers (nifedipine) first-line topical nitroglycerin second-line