Progressive dyspnoea in a 40-yr-old female.

A 40-yr-old female presented with dyspnoea on minimal exertion and a paroxysmal dry cough, keeping her awake for long periods. The shortness of breath was reported to be progressive over the last 18 months. She had never smoked and was not on medication. There was no recent history of travelling and she only had a dog at home. Her medical history was marked with autosomal dominant polycystic kidney disease. On clinical examination, the patient presented a moderate fever measured at 39uC and a regular pulse (90 beats?min); blood pressure was 130/90 mmHg. Fine inspiratory crackles were heard on the left hemithorax and on the right base. A blood sample revealed an inflammatory syndrome with a C-reactive protein (CRP) level of 97 mg?L. Arterial blood gas analysis yielded a severe arterial hypoxaemia with an arterial oxygen tension (Pa,O2) of 4.26 kPa (32 mmHg) and an oxygen saturation of 62%. The results of spirography showed an important volume restriction: total lung capacity 2.55 L (normal 7.97 L); vital capacity 1.40 L (normal 6.09 L); forced expiratory volume in one second (FEV1) 1.07 (normal 4.65 L). A chest radiograph (fig. 1) was performed, followed by a high-resolution computed tomography (HRCT) scan of the chest (fig. 2). The patient was treated with inhaled steroids, inhaled bronchodilatators, oxygen and antibiotics. Broad-spectrum antibiotics were given for 15 days with improvement of the inflammatory syndrome, but with no significant improvement of the dyspnoea. Due to lack of improvement with empiric therapy, surgical lung biopsy was performed on two different lobes (figs 3a and b) from the right lung.