Pulmonary arterial hypertension in a patient with common variable immunodeficiency and unilateral bronchiectasis: Successful treatment with iloprost.

The present study describes a case of severe pulmonary arterial hypertension (PAH) associated with unilateral lung destruction due to bronchiectasis in a patient with common variable immunodeficiency (CVID). Initially, the patient's treatment included antibiotics, oral anticoagulants, diuretics, and immunoglobulin replacement therapy. However, the patient's condition improved significantly only after inhaled iloprost was administered. Three months later, his PAH was almost reversed. The hemodynamic response of our patient suggests that inhaled iloprost may have a role in the treatment of sustained PAH related to unilateral lung destruction.