A rare case of common variable immunodeficiency and hepatopulmonary syndrome

Abstract

Abstract Hepatopulmonary syndrome (HPS) is the triad of liver disease, hypoxia and intrapulmonary shunt. In this case report, we describe an immunocompromised female with a background of common variable immunodeficiency (CVID) who presented with haemoptysis and dyspnoea. Investigations demonstrated significant hypoxaemia and intrapulmonary right‐to‐left shunting. Further evidence revealed advanced liver cirrhosis, and a diagnosis of HPS was made. This extremely rare association of CVID and HPS is one of the few cases reported in the literature.

Keywords

• chronic bronchiectasis
• clinical allergy and immunology
• immunodeficiency
• pulmonary circulation and pulmonary hypertension
• rare lung diseases