A hypogammaglobulinaemic man with respiratory failure.

Accepted 10 March 1998 A 65-year-old man with a long-standing clinical history of bronchiectasis and sarcoid-like skin lesions had been diagnosed as having common variable immunodeficiency (CVID) 4 years previously, and began treatment with regular intravenous immunoglobulin (IVIG) to good effect. Seven months before admission he developed a Coombs' positive haemolytic anaemia (haemoglobin 9.3 g/l) with splenomegaly and lymphadenopathy. He received three units ofblood, his IVIG dose was increased, but he required oral prednisolone 40 mg/day to control his anaemia. A month before admission his visual acuity deteriorated in the right eye, and investigations revealed a granulomatous choroiditis, inaccessible to biopsy. Whilst this was being investigated, he developed dyspnoea at rest, with a cough productive of white sputum, and no haemoptysis. On examination he was unwell, cyanosed, plethoric and with bruised skin. Chest examination showed dullness at both bases, associated with inspiratory and expiratory crackles. Investigations showed him to be hypoxic, with a markedly reduced diffusion capacity (DLco 28% predicted, KC0 38% predicted). Only normal respiratory flora, and no acid/alcohol-fast bacteria were isolated from sputum cultures. Imaging with high resolution computed tomography (CT) showed ground-glass opacification in both lung fields with multifocal consolidation in the right upper lobe. Bronchoscopy of this area was normal; lavage showed a minor inflammatory cell infiltrate with no malignancy. The lavage culture was negative for bacteria, fungus, acid/alcohol-fast bacteria, and Pneumocystis carinii, and cytological examination was normal. There was no improvement on intravenous antibiotics, so an open lung biopsy was performed. Characteristic lesions were found (figure).