RESUMO
INTRODUCTION: The association between interstitial pneumonia and hepatopulmonary syndrome (HPS) has rarely been described. OBSERVATION: We report the cases of two patients, 62 and 64 years old, who presented with idiopathic interstitial pneumonia (IIP) and severe, disproportionate hypoxemia, PaO(2) 42 and 47 mmHg, respectively. This hypoxemia lead to search for associated disease. The diagnosis of right-to-left shunt was established by the 99mTc-MAA perfusion lung imaging. A contrast cardiac echography confirmed the intrapulmonary shunt. Both patients were found to have hepatic cirrhosis, classed as Child Grade A6 and C10. In this context of chronic hepatopathy, the diagnosis of SHP associated with IIP was established. Liver transplantation, the only way to cure the lung consequences of the HPS, was planned. CONCLUSION: These two cases highlight the importance of searching for right-to-left shunt in patients who have lung disease with severe disproportionate hypoxemia. Liver transplantation is the only way to cure the lung consequences of the hepathopathy. The rare association between ILD and HPS raises problems when deciding to go forward to transplantation in the absence of a histological diagnosis of the lung disease and where the prognosis of the lung disease is unclear.