Reversion of severe hepatopulmonary syndrome in a non cirrhotic patient after corticosteroid treatment for granulomatous hepatitis: a case report and review of the literature

Abstract

Hepatopulmonary syndrome (HPS) is defined as a clinical triad including liver disease, abnormal pulmonary gas exchange and evidence of intrapulmonary vascular dilatations. We report a 61-year-old male presented with fatigue, long-lasting fever, loss of weight, signs of portal hypertension, hepatosplenomegaly, cholestasis and progressive dyspnoea over the last year. Clinical, laboratory and histological findings confirmed the diagnosis of granulomatous hepatitis. HPS due to hepatic granuloma-induced portal hypertension was proved to be the cause of severe hypoxemia of the patient as confirmed by contrast-enhanced echocardiography. Reversion of HPS after corticosteroid therapy was confirmed by a new contrast-enhanced echocardiography along with the normalization of cholestatic enzymes and improvement of the patient's conditions. This is the first case of complete reversion of HPS in a non-cirrhotic patient with hepatic granuloma, indicating that intrapulmonary shunt in liver diseases is a functional phenomenon and HPS can be developed even in miscellaneous liver involvement as in this case.

Figure 1

Liver histopathology shows an epithelioid granuloma without central necrosis. The granuloma is consisted of epithelioid macrophages and some chronic inflammatory cells. There is also a multinucleated giant cell (Hematoxylin and eosin staining x 40).

Figure 2

Contrast-enhanced echocardiography image of the patient before (A, B) and after (C, D) corticosteroid treatment for granulomatous hepatitis. A, C: Microbubble appearance in the right heart chamber (RA and RV) after the bolus infusion of agitated saline; B: Microbubble opacification in the left heart chambers (LA and LV) is not detected after six to eight heart beats; D: Microbubble opacification in the left heart chambers (LA and LV)is not detected after six to eight heart beats.