2504 Successful Budesonide Monotherapy for Autoimmune Hepatitis/Hepatic Sarcoidosis Overlap Syndrome in a Patient With Drug Reaction With Eosinophilia and Systemic Symptoms (DRESS) From Azathioprine

Abstract

INTRODUCTION: Drug reaction with eosinophilia and systemic symptoms (DRESS) is considered a severe idiosyncratic drug hypersensitivity which can be fatal if not promptly diagnosed and treated. Here, we describe a case of hepatic sarcoidosis and autoimmune hepatitis (AIH) overlap syndrome who was found to have DRESS from taking azathioprine (AZA). CASE DESCRIPTION/METHODS: A 63-year-old African-American female with a diagnosis of AIH and hepatic sarcoidosis overlap based on liver biopsy (chronic hepatitis with moderate activity (grade 2/4), mild fibrosis (stage 1/4), non-caseating granulomata, and interface hepatitis with dense lymphoplasmacytic infiltration) (Figure 1), positive ANA (1:160), and elevated IgG (2140) was started on treatment with AZA and budesonide (intolerance to prednisone). She had normal TPMT activity. Patient stopped taking AZA few days later because of feeling “jittery.” She presented to the ED five weeks later with lip swelling and desquamating rash and was discharged with antihistamines. She returned to the ED two days later with worsening rash, fever, and dyspnea. Labs showed leukocytosis, eosinophilia (56.1%, 13,400/mm3), acute hepatitis, acute kidney injury, and respiratory distress with concern for DRESS. Her initial LFTs were ALT 96, AST 65, and Alk Phos 633, which were increased from her baseline. Imaging ruled out biliary obstruction and bone marrow biopsy was negative for myeloproliferative disorders such as hypereosinophilic syndrome. She was diagnosed with DRESS secondary to AZA and was treated with a short course of prednisone with transition to budesonide monotherapy. Eosinophilia and other organ dysfunction resolved. Her most recent LFTs are ALT 37, AST 42, and Alk Phos 277. DISCUSSION: While AZA hypersensitivity syndrome is a well-known disorder presenting with drug eruption and flu-like symptoms, to our knowledge this is the first case of DRESS secondary to AZA. Additionally, this occurred in the setting of hepatic sarcoidosis and AIH overlap syndrome which itself is a rare entity. DRESS is characterized by a long latency time between exposure and disease (2-8 weeks) and presents with end organ dysfunction requiring steroid treatment. Based on the European Registry of Severe Cutaneous Adverse Reactions (RegiSCAR) scoring system, our patient's score was 7, where a score of 6 or higher is considered definite for DRESS. This case also stresses the value of budesonide monotherapy for treating AIH and hepatic sarcoidosis overlap when other first line medications cannot be used.