Pulmonale Angiodysplasie mit tödlicher Lungenblutung

For 6 weeks a 52-year-old woman had complained of increasing fatigue, blood-streaked vomitus, retrosternal burning and pain between the shoulder blades. Physical examination showed no abnormalities. Blood sedimentation rate was increased to 15/40 mm and the iron concentration was slightly reduced. Computed tomography demonstrated densities in the left upper lobe of the lung and both lower lobes. Scintigraphy revealed a perfusion defect in the left apex of the lung while bronchoscopy demonstrated acute bronchitis in the left upper lobe. Further haemoptysis occurred 3 months later, but several bronchoscopies failed to elucidate their cause. Three days later another haemoptysis caused respiratory arrest. After resuscitation the bleeding was localized to the right main bronchus, and the right upper and middle lobes were resected. The patient died the next day from a massive haemoptysis. Post-mortem examination showed angiodysplasia in all lobes of the lung. The branches of the pulmonary artery were dilated, their wall was irregular and the muscular tunica media reduced. The elastic lamellae were fragmented and there were cell-rich intimal pads. These changes most closely resembled fibromuscular dysplasia.