![]() However, with the introduction of the HBV vaccination, the incidence of PAN has been greatly reduced. Historically, PAN has been found to be frequently associated with hepatitis B (HBV) infection, occurring at a rate of 30% in that population. PAN typically involves the medium and small arteries with arterial inflammation leading to vessel narrowing and subsequent formation of aneurysms and microaneurysms. Polyarteritis nodosa (PAN) is a rare inflammatory vasculitis that affects both men and women equally with a prevalence of 5–10 patients per million. In patients with recent diagnosis of autoimmune hepatitis, there should be a modest suspicion for concomitant polyarteritis nodosa if symptoms and signs of multisystem vasculitis develop. Since her hospitalization, she has been maintained on low-dose prednisone and mycophenolate mofetil. Azathioprine was discontinued and she was treated with pulse steroids followed by a prednisone taper, cyclophosphamide, and intravenous immune globulin with overall improvement in her symptomatology. Surgical pathology of the small bowel resection revealed ischemic necrosis, medium and small vessel vasculitis with microvascular thrombi consistent with polyarteritis nodosa. She subsequently developed repeated episodes of ischemic acute bowel necrosis that required multiple surgeries and extensive small bowel resections. Physical examination was notable for erythema nodosum, anterior uveitis, retinal vasculitis, and frosted branch angiitis (frosted branch angiitis (a widespread florid translucent perivascular exudate). Three months later, she presented to the emergency department with fever, bilateral ankle pain, rash, oral ulcers, and poor vision. Her liver biopsy showed mild portal fibrosis and her liver chemistries improved with prednisone and azathioprine. Case presentationĪ 45-year-old woman was diagnosed with autoimmune hepatitis after initially presenting with a two-month history of fatigue, nausea, and anorexia and a three-week history of scleral icterus. Here we report the case of an adult patient with autoimmune hepatitis who, shortly after diagnosis, developed life-threatening polyarteritis nodosa. Polyarteritis nodosa is a type of vasculitis affecting medium- and small-sized arteries that has been associated with hepatitis B but does not have an established relationship with autoimmune hepatitis.
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