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Diffuse Lung Disease |

A Patient With Autoimmune Hepatitis and Interstitial Lung Disease

Juan Enghelmayer, MD; Tulio Papucci, MD; Julio Silio, MD; Luciano Melatini, MD
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Hospital de Clínicas José de San Martín, Buenos Aires, Argentina


Chest. 2013;144(4_MeetingAbstracts):438A. doi:10.1378/chest.1701233
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Abstract

SESSION TITLE: Interstitial Lung Disease Global Case Reports

SESSION TYPE: Global Case Report

PRESENTED ON: Tuesday, October 29, 2013 at 01:30 PM - 02:30 PM

INTRODUCTION: Autoimmune diseases are a heterogeneous group of disorders that share a immunologic damage and may affect various organs. It is well known that autoimmune diseases can compromise directly or indirectly to the lung, for example in the context of scleroderma, lupus, rheumatoid arthritis, dermatomyositis, sarcoidosis, Sjogren, but little has been published about the association between autoimmune hepatitis and interstitial lung diseases (ILD).

CASE PRESENTATION: We present a 57 year old woman with a history of hypothyroidism and current smoker (40 p / a) who complains of dry cough of 3 months duration and dyspnea. Physical examination showed isolated crackles in both lung bases. Pulse Oximetry breathing room air was 96%. No joint pain. Blood tests demonstrated elevated aminotransferase levels ( GOT:121 and GPT: 197) , Alkaline Phosphatase: 338, Total Bilirubin: 0.73 Direct Bilirubin: 0.11, anti-DNA antibody 95 UI/ml , ANA 1/80 with nucleolar pattern, Anti-smooth muscle antibodie: 1/1280. Spirometry showed FVC of 85% of predcited, with moderate to severe fall in the DLCO (53% of predicted), and important desaturation during exercise. Hepatic ultrasonography: mild diffuse, heterogeneous and lobulated hepatomegaly. Homogeneous splenomegaly (152 x 59 x 67 mm) ; intra-and extrahepatic biliary tract preserved. With preserved hepatic artery flow. Portal hypertension was not observed. Liver biopsy was performed and it demonstrated moderate inflammatory infiltrate and some plasmacytes with extensive hepatitis. At acinar level was observed isolated necroinflammatory foci. Tricomic Technique: thick fibrous bands that delimit regenerative nodules. Histological findings along with patient serology were compatible with autoimmune etiology.The Chest CT showed: diffuse interstitial opacities with bilateral ground glass, thin and thick reticular pattern, few bronchiolectasis but honeycombing was not observed. It was interpreted as Hepatitis plus ILD. The patient refused lung biopsy.

DISCUSSION: Chronic liver disease may involve the lung through abnormal communications between the portal and pulmonary veins or by changes in the lungs caused by similar biochemical abnormalities to those in liver parenchyma. There are very few reports in the medical literature describing the association between autoimmune liver disease and pulmonary interstical involvement. It is important to determine whether this type of autoimmune liver disease shares a common pathophysiological mechanism with ILD, because it has diagnostic and therapeutic implications (eg the decision to perform surgical lung biopsy) and may have prognostic significance.

CONCLUSIONS: We should consider autoimmune hepatitis and ILD as related diseases with probable common pathophysiological mechanisms.

Reference #1: Koulaouzidis A, et al. Lynphocytic interstitial pneumonitis (LIP)-the liver and the lung. Ann Hepatol. 2006 Jul-Sep;5(3):170-1.

Reference #2: Mieli-Vergani G, Vergani D. Autoimmune hepatitis: A comprehensive review.Autoimmun. Nat Rev Gastroenterol Hepatol. 2011 Jun;8(6):320-9.

Reference #3: Autoimmune hepatitis: a review. Gossard AA, Lindor KD.J Gastroenterol. 2012 May;47(5):498-503. doi: 10.1007/s00535-012-0586-z. Epub 2012 Apr 17.

DISCLOSURE: The following authors have nothing to disclose: Juan Enghelmayer, Tulio Papucci, Julio Silio, Luciano Melatini

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