A rare case of Polyarteritis Nodosa associated with autoimmune hepatitis: a case report

Abstract

Background: 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. Here we report the case of an adult patient with autoimmune hepatitis who, shortly after diagnosis, developed life-threatening polyarteritis nodosa.

Case presentation: A 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. Her liver biopsy showed mild portal fibrosis and her liver chemistries improved with prednisone and azathioprine. Three months later, she presented to the emergency department with fever, bilateral ankle pain, rash, oral ulcers, and poor vision. Physical examination was notable for erythema nodosum, anterior uveitis, retinal vasculitis, and frosted branch angiitis (frosted branch angiitis (a widespread florid translucent perivascular exudate). She subsequently developed repeated episodes of ischemic acute bowel necrosis that required multiple surgeries and extensive small bowel resections. Surgical pathology of the small bowel resection revealed ischemic necrosis, medium and small vessel vasculitis with microvascular thrombi consistent with polyarteritis nodosa. 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. Since her hospitalization, she has been maintained on low-dose prednisone and mycophenolate mofetil.

Conclusions: 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.

Keywords: Autoimmune hepatitis; Azathioprine; Cyclophosphamide; Polyarteritis nodosa.

Fig. 1

a Liver needle biopsy showing marked interface activity with lymphoplasmacytic inflammation and apoptotic hepatocytes (H&E stain × 400 magnification). b Small bowel with extensive ischemic necrosis and fibrinopurulent exudate on the mucosal surface (H&E stain × 200 magnification). c Medium sized muscular blood vessel with fibrinoid necrosis and neutrophilic inflammation of the vessel wall (H&E stain × 100 magnification). d Small capillary with thrombotic microangiopathy and acute inflammation with leukocytoclastic-like inflammatory response (H&E stain × 400 magnification)