2396 Idiopathic Granulomatous Hepatitis: A Case of Episodic Fever of Unknown Origin

Abstract

INTRODUCTION: We present a case of a patient with episodes of fever and weight loss, due to a second episode of idiopathic granulomatous hepatitis (IGH), 12 years after the first episode. CASE DESCRIPTION/METHODS: A 63-year-old female with a history of hyperlipidemia and reflux disease was referred to the hepatology clinic with an elevated alkaline phosphatase (ALP) of 326. The rest of her hepatic panel was normal. She reported a 3-month history of intermittent fevers up to 103 degrees Fahrenheit and a 35-pound weight loss over 5 months. She had a similar episode over 10 years ago, with an extensive negative work-up. A liver biopsy was obtained at that time and it showed extensive necrotizing granulomas with portal and lobular involvement. She responded to a short course of prednisone with resolution of symptoms and normalization of ALP. With her present episode, viral serology was obtained and found to be negative. A repeat liver biopsy was done, with evaluation for fungal and mycobacterium components, as well as iron overload. It showed extensive sclerosed and calcified granulomas without any evidence of active alcoholic hepatitis. A diagnosis of relapsing fever of unknown origin due to idiopathic granulomatous hepatitis (IGH) was made and she was started on prednisone 40 mg daily with resolution of her symptoms. DISCUSSION: Hepatic granulomas are found in up to 10% of liver biopsies and may be incidental. The etiology of hepatic granulomas is diverse, and search for cause should include an exhaustive infectious, autoimmune and rheumatology work up, as well as careful medication review. Presentation of IGH is variable and most symptoms reported by patients include subjective fevers, chills and night sweats, weight loss, hepatosplenomegaly, or elevation of alkaline phosphatase (ALP) or gamma-glutamyl transferase (GGT). IGH has been noted to occur mostly in middle-aged women with primarily cholestatic liver enzymes and has a favorable prognosis. Corticosteroids are the main-stay of treatment of IGH, and methotrexate has also been shown to be effective in those with significant relapse, in steroid-refractory cases or in patients who do not tolerate steroids.