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A6506 - Interstitial Lung Disease as an Extrahepatic Manifestation of Autoimmune Hepatitis
Author Block: A. D. Hyslop1, N. Gupta2, K. A. Wikenheiser-Brokamp3; 1Internal Medicine, Univ of Cincinnati, Cincinnati, OH, United States, 2Univ of Cincinnati, Cincinnati, OH, United States, 3Cincinnati Childrens Hosp Medical Ctr, Cincinnati, OH, United States.
Introduction: Interstitial lung disease (ILD) is a common manifestation of patients with underlying connective tissue diseases (CTDs) such as Rheumatoid Arthritis (RA), Systemic Lupus Erythematosus (SLE), or Sjögren Syndrome. Autoimmune hepatitis (AIH) is known to cause a multitude of extrahepatic manifestations, however, the development of ILD as an extrahepatic manifestation of AIH has not been well delineated.
Case Description:
A 45 year-old Caucasian female with a history of type 1 diabetes mellitus, asthma, and breast adenocarcinoma presented to her primary care physician with worsening dyspnea on exertion for one week. Basic laboratory studies, electrocardiogram, and chest x-ray (CXR) were ordered, and the patient was prescribed a 7-day steroid course. Laboratory data revealed elevated liver function tests: AST 407, ALT 517, alkaline phosphatase 464, and a normal total bilirubin of 0.6. CXR showed interstitial pneumonia. She presented to the emergency room the following day with worsening dyspnea. Computed Tomography (CT) scan of the chest, abdomen and pelvis with contrast revealed bilateral upper lobe airspace disease. She was diagnosed with atypical pneumonia and discharged home on levofloxacin. Her symptoms progressed and she underwent a repeat CT chest one month later showing worsening diffuse bilateral multifocal, patchy ground glass opacities with mediastinal and hilar lymphadenopathy. Autoimmune serologies revealed elevated anti-F-actin and anti-smooth muscle antibodies, confirming the diagnosis of AIH. Other autoimmune markers for RA, SLE, and Sjögren syndrome were negative. Pulmonary function test revealed a mild restrictive defect with normal diffusion capacity. She underwent video-assisted thoracoscopic surgery-guided lung biopsy showing a patchy cellular interstitial pneumonia with extensive chronic bronchiolitis and rare foci of organizing pneumonia, most consistent with a histopathological diagnosis of non-specific interstitial pneumonia (NSIP). She was started on steroids and mycophenolate with marked improvement in her dyspnea. Follow up CT chest demonstrated near-complete resolution of the radiologic findings.
Discussion:ILD is often the presenting manifestation of an underlying CTD. NSIP is the most commonly observed radiologic and histopathological pattern of ILD in patients with CTD. The association between AIH and development of ILD has not been well characterized. Case reports and small case series have suggested the possibility of co-existence of AIH and other autoimmune disorders including the development of ILD. Our case provides supporting evidence of a possible link between AIH and the development of extrahepatic, systemic autoimmune conditions including ILD. The patient is being closely monitored for the onset of clinical signs and symptoms of another well-characterized CTD.