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A rare case of desquamating interstitial pneumonia, in a 15-year-old boy, is reported. Symptoms consisted of dry cough, progressive dyspnoea and chest pain. The chest X-ray revealed bilateral basal opacities. There were hypoxaemia and restrictive ventilatory changes. Bacteriological, virological and serological tests were all negative. An open lung biopsy established the diagnosis and corticosteroids were given as the drug of first choice. Other drugs, such as cytostatic ones, should only be given if steroids are ineffective. Arterial blood gases proved to be the most sensitive indicator of improvement, while the chest X-ray remained abnormal for several months.
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