Pulmonary telangiectasia without hypoxemia

We describe an elderly patient with an unusual presentation of hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber disease) involving the lung. He had recurrent "pneumonia" caused by massive hemorrhage from endobronchial telangiectases. When stable, he was normoxic, had no evidence of right-to-left shunting, and had mild pulmonary arterial hypertension. His pulmonary telangiectases may be isolated to the bronchial circulation. We report hemodynamic data and show the first photographs of endobronchial telangiectases.