A 52-year-old man with a personal history of 10 cigarettes/day ex-smoking and diagnosed with a bicuspid aortic valve with normal systolic function. A year earlier he had undergone surgery for acute sigmoid diverticulitis with perforation and peritonitis and 4 months after the operation he developed right femoral-popliteal deep vein thrombosis (DVT), and had been treated with acenocoumarol since then. The study performed 3 months after the episode to rule out hypercoagulability syndrome showed a prothrombin time (PT) of 12.67 sec (VN 9-12 sec), an activated partial thromboplastin time (APTT) of 66.23 sec (VN 26-39 sec), platelets 140. 000 /dL, Ig G anticardiolipin antibodies 127 GPL/ml (VN 3.2-16.5) and IgM anticardiolipin antibodies 9.2 GPL/ml (VN 3.9-41). Serum levels of antithrombin III, protein S and protein C were normal.
He consulted for asthenia and intermittent fever of 38ºC since one month before, which in the 4 days prior to admission had begun with haemoptotic expectoration, dyspnoea on moderate exertion and the fever had become persistent. Physical examination showed a good general condition, with only a temperature of 38ºC and no other relevant alterations except for the colostomy.
The blood test showed haemoglobin 14.5 g/dl, red blood cells 4,650,000/mm3, mean corpuscular volume 89.7 µmc, leukocyte count 8170/dl and leukocyte count was normal. ESR was 50 mm in the first hour. INR was 2.5, adjusted for acenocoumarol. Biochemistry, ions and transaminases were normal. Urinary sediment showed no abnormalities. Rheumatoid factor, cryoglobulins, antinuclear antibodies (ANA), anti-neutrophil cytoplasmic antibodies (ANCA), anti-glomerular basement membrane, anti-peroxidase, anti-histone, anti-Ro, anti-SSA and anti-SM were normal or negative. Arterial blood gases FiO2 0.21: pH 7.53, pO2 65 mmHg, pCO2 39 mmHg. The electrocardiogram was normal and the echocardiogram confirmed a bicuspid aortic valve without other alterations. Chest X-ray showed bilateral alveolar infiltrates with normal cardiac silhouette. Blood cultures and sputum culture for bacteria and mycobacteria were negative.
Ventilation/perfusion scintigraphy and computed tomography angiography showed no evidence of pulmonary thromboembolism, so anticoagulant treatment was discontinued and treatment with amoxicillin/clavulanic acid was started, with clinical improvement and disappearance of fever, but haemoptysis sputum and infiltrates persisted in the chest X-ray. Bronchoscopy was performed and no endoscopic abnormalities were found. Bronchoaspirate culture showed no growth for bacteria, fungi or mycobacteria and cytology showed no malignant cells. Spirometry, lung volumes and diffusion were normal.
After discharge from hospital, the patient continued to present haemoptotic sputum. A new high-resolution computed axial tomography (HRCT) scan showed bilateral pulmonary infiltrates and ground glass. A new bronchoscopy showed no intrabronchial alterations and bronchoalveolar lavage (BAL) showed 512 cells/ml: neutrophilic polymorphonuclear 1%, eosinophilic polymorphonuclear 1% and histiocytes 98%. Prussian blue staining showed 80% haemosiderophages.

Peripheral blood levels of anticardiolipin and lupus anticoagulant antibodies remained elevated. Based on the findings described above, the patient was diagnosed with primary antiphospholipid syndrome and secondary pulmonary haemorrhage, and treatment was restarted with acenocoumarol and acetylsalicylic acid. During one year of follow-up he presented occasional haemoptotic sputum, but the chest X-ray and pulmonary function (spirometry, diffusion and arterial gases) remained normal and only the CT scan showed some areas of ground glass.