Lung function studies in a patient with diffuse pulmonary fibroleiomyomas

Diffuse pulmonary fibroleiomyomas were detected by open-lung biopsy in a 33-year-old male patient. Lung function studies revealed severe airflow obstruction, hyperinflation, grossly impaired single-breath-diffusing capacity and a decrease in PaO2 during exercise, i.e. data which are compatible with pulmonary emphysema. However, static lung compliance was within normal limits excluding the existence of pure emphysema. Additional special lung function tests confirmed the assumption that in diffuse fibroleiomyomas of the lung, functional patterns characteristic of obstructive and restrictive lung disease are present that are in agreement with the histological features of this disease.