RESUMO
15 cases of histiocytosis X and 274 cases of histologically confirmed sarcoidosis were diagnosed during the investigation period from 1969 to 1975. Data of 12 adults with primary pulmonary histiocytosis X were evaluated in extenso. The necessary histological verification of diagnosis was only possible by open lung biopsy. Already in early stage small excavations were found by tomography in half of the cases. Eelvation of serum copper and of the index of leukocyte alkaline phosphatase was striking. In a single case antinuclear antibodies were proven. An intra patient comparison verifies corticosteroids suppressing the disease. On the occasion of a second lung biopsy in one case could be seen that after treatment no more histiocytosis-specific substrate was existing. Exacerbation and relapse during and after continuous long-term therapy were not observed. The features of histiocytosis X and sarcoidosis are set side by side in order to show differences and relations.
