Two patients with angioimmunoblastic lymphadenopathy (AIL), in whom the disease process involved the lungs, are reported. One patient died of respiratory failure. Details of the radiologic appearance, abnormalities of lung function and lung histology are presented together with response to steroid therapy in 1 patient. Life-threatening involvement of the lung parenchyma can be a mode of presentation of this disease and can contribute to the morbidity and mortality of the disorder.