Autoimmune Hepatitis and Sarcoidosis

• Main Authors: Shoaib Z Junejo Resident, Sandeep Tuli Attendent
• Format: Article
• Language: English
• Published: Thieme Medical and Scientific Publishers Pvt. Ltd. 2017-12-01
• Series: International Journal of Recent Surgical and Medical Sciences
• Subjects: angiotensin-converting enzyme; antismooth muscle antibody; autoimmune hepatitis; liver function test
• Online Access: http://www.thieme-connect.de/DOI/DOI?10.5005/jp-journals-10053-0057
• ISSN: 2455-7420; 2455-0949

Background: Sarcoidosis is a systemic disorder of unknown etiology. It affects the organ systems in the body, and is characterized by the presence of noncaseating granulomas in the involved organs. The most common manifestation is bilateral hilar adenopathy along with skin, joint, and eye lesions. Here, we discuss an unusual manifestation of sarcoidosis along with autoimmune hepatitis (AIH). Case report: We report the case of a 33-year-old African American female presented with two-day history of progressive jaundice and deranged Liver function test (LFTs) the autoimmune work up was positive for anti-nuclear antibody (ANA) 1:640 and anti-smooth muscle antibody (SMA) 1:160, The serum Angiotensin converting enzyme (ACE) level was elevated 299 U/L; a right upper quadrant ultrasound revealed a mildly enlarged liver, subsequent CT chest and CT abdomen enlarged paratracheal and subcarinal adenopathy periportal edema in the liver and numerous small hypodense nodules in the spleen. Subsequent liver biopsy showed chronic, non-granulomatous, portal and parenchymal inflammation that was compatible with Autoimmune Hepatitis. Bronchoscopy with transbroncial biopsy revealed chronic, non-caseating granulomatous inflammation that was consistence with sarcoidosis. Thus the patient was diagnosed with sarcoidosis and AIH. The patient was started on Prednisone 60 mg daily, with subsequent reduction to 15 mg. The serum transaminase levels returned to normal level within three months. Conclusion: This case underscores the significance of association of Sarcoidosis that presented with Autoimmune Hepatitis. Physicians should be aware of this possibility so they may efficiently pick up the diagnosis after relevant investigations and start appropriate management. Timely intervention is key to treat the acute presentation and to prevent the further complications.