[A review of pulmonary histiocytosis X]

The article contains a review and two case reports of pulmonary histiocytosis-X. This is a rare disease entity comprising about 3% of all chronic interstitial lung diseases. Diagnosis is confirmed by histological and electronmicroscopic demonstration of the typical histiocytosis-X cell. The course of the disease varies. About 25% of the patients show spontaneous remission, in 40% the changes remain stationary, while 35% progress and eventually die from respiratory insufficiency or cor pulmonale. Treatment with glucocorticoids and cytostatics should be initiated at high disease activity, with progressive X-ray changes and decreasing pulmonary function.