P2524 - A Unique Presentation of Autoimmune Hepatitis as Acute Hypoxic Respiratory Failure

Tooba Laeeq, MD¹, Mark Hsu, MD², Zahra Dossaji, DO^2
1University of Nevada, Las Vegas, Henderson, NV; ²Kirk Kerkorian School of Medicine at UNLV, Las Vegas, NV

Introduction: Autoimmune hepatitis (AIH) is a chronic liver disease with dynamic and heterogeneous disease manifestations. Diagnosis is confirmed with elevated aminotransferases, serum antibody positivity, compatible histology, and responsiveness to steroids after exclusion of other causes. Clinical presentations of AIH can be vague, generalized, and sometimes manifestations of extrahepatic processes. Herein we present a case of AIH presenting primarily as respiratory failure with incidental findings of elevated aminotransferases.

Case Description/Methods: A 23-year-old female with no known medical history presented with dry cough, shortness of breath and right upper quadrant abdominal pain for one month. Labs on admission revealed WBC 9 k/mm3, AST 156 IU/L, and ALT 179 U/L, with increased oxygen requirements at 3L nasal cannula (baseline room air). CT imaging of the lungs revealed bilateral ground glass opacities with reverse halo sign and pleural plaques. Extensive infectious work-up including HIV, legionella, coccidioides, cryptococcus, mycoplasma, histoplasma, blastomycosis, and aspergillus was unremarkable. ANA, AMA, LKM, RF, PR3-ANCA, anti-GBM, and cryoglobulin were all negative. Mixed Connective tissue diseases, scleroderma, rheumatoid arthritis were ruled out using auto-immune superpanel. ASMA was strongly positive (1:92), and atypical p-ANCA was positive 1:320. Liver biopsy was performed showing non-specific chronic inflammation. Steroids were initiated with subsequent improvement in oxygen requirements and downtrend in aminotransferases (AST 38 and ALT 85 at discharge).

Discussion: AIH has a wide variety of extrahepatic manifestations; recent literature has suggested a potential involvement of the lungs through communications between the portal and pulmonary veins. Rarely, pulmonary symptoms may even be the only presenting complaint. The most sensitive and specific test for evaluation of pulmonary disease is high resolution CT scan, which may reveal non-specific interstitial or nodular changes in the pulmonary parenchyma, lung vessels, and/or pleura. As pulmonary manifestations of AIH tend to be insidious, early recognition is paramount in preventing irreversible changes secondary to pulmonary manifestations increasing mortality and morbidity in AIH.


Disclosures:


Tooba Laeeq, MD¹, Mark Hsu, MD², Zahra Dossaji, DO². P2524 - A Unique Presentation of Autoimmune Hepatitis as Acute Hypoxic Respiratory Failure, ACG 2023 Annual Scientific Meeting Abstracts. Vancouver, BC, Canada: American College of Gastroenterology.