Freda Kennedy1, Rachel Kapelow1,2, Bilge D Kalyon3, Nitzan C Roth4, Arvind Rishi5, Maria-Louise Barilla-LaBarca6,7. 1. Department of Medicine, Northwell Health, 300 Community Drive, Manhasset, NY, 11030, USA. 2. Division of Rheumatology, Department of Medicine, Northwell Health, 865 Northern Boulevard, Suite 302, Great Neck, NY, 11021, USA. 3. Department of Surgery, Northwell Health, 300 Community Drive, Manhasset, NY, 11030, USA. 4. Sandra Atlas Bass Center for Liver Diseases and Division of Hepatology, Department of Medicine, Northwell Health, 400 Community Drive, Manhasset, NY, 11030, USA. 5. Department of Pathology and Laboratory Medicine, 2200 Northern Boulevard, Suite 104, Greenvale, NY, 11548, USA. 6. Department of Medicine, Northwell Health, 300 Community Drive, Manhasset, NY, 11030, USA. mbarilla@northwell.edu. 7. Division of Rheumatology, Department of Medicine, Northwell Health, 865 Northern Boulevard, Suite 302, Great Neck, NY, 11021, USA. mbarilla@northwell.edu.
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.
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.
Authors: Armando De Virgilio; Antonio Greco; Giuseppe Magliulo; Andrea Gallo; Giovanni Ruoppolo; Michela Conte; Salvatore Martellucci; Marco de Vincentiis Journal: Autoimmun Rev Date: 2016-02-13 Impact factor: 9.754