Case 1

A 70 year old woman was referred by her general physician to the respiratory clinic with a few days’ history of haemoptysis without any associated chest pain, fever, or dyspnoea. The only medical history of note was a successful pulmonary venous isolation procedure for paroxysmal atrial fibrillation in the previous week. She was a lifelong non-smoker and was previously fit and well. Physical examination and routine blood tests were unremarkable. The electrocardiogram showed sinus rhythm. A small (2 cm) opacity was seen on the chest radiography in the left mid-zone. Computed tomography of the thorax and abdomen showed only numerous ill-defined patchy lesions with ground-glass shadowing in the left upper lobe, without any evidence of malignancy. Bronchoscopy showed altered blood in the left upper lobe bronchus, and lavage specimens were negative for malignancy and infection, including tuberculosis. She was treated empirically for an atypical pneumonia.

Haemoptysis recurred six weeks later. Further tests including autoantibody screen, aspergillus precipitins, and complement status had negative results. On a repeat scan of the thorax, patchy ground-glass shadowing persisted in the left upper lobe. Bronchoscopy was repeated, with transbronchial biopsies taken from the left upper lobe. Histological examination showed focal occlusion of blood vessels, with recanalisation and presence of haemosiderin-laden macrophages suggestive of alveolar haemorrhage. The overall appearances were characteristic of veno-occlusive pulmonary disease. Review of the original thoracic scan showed significant impairment of contrast opacification at the left upper pulmonary venous ostium, suggestive of severe pulmonary venous stenosis (fig 1⇓). The stenosis was confirmed by pulmonary venous angiography and relieved by percutaneous balloon venoplasty. The patient was symptom free after 12 months of follow-up.