When to suspect and how to diagnose pulmonary lymphangioleiomyomatosis
- 1 September 1996
- journal article
- Published by Wiley in Respirology
- Vol. 1 (3), 207-212
- https://doi.org/10.1111/j.1440-1843.1996.tb00034.x
Abstract
The objective of this study was to present clinical and radiological data of eight women with histologically proven lymphangioleiomyomatosis (LAM) diagnosed between 1984 and 1994, and to suggest a diagnostic strategy when LAM is suspected. A review of case reports, including results of biopsies, lung function and radiological procedures was undertaken. The mean age of the women at start of symptoms was 36 years, and the mean age at time of diagnosis 42 years. The most frequent presenting complaint was dyspnea, either in conjunction with pneumothorax (3), chylothorax (2) or on exertion (2). All patients had airflow limitation and markedly reduced gas transfer. Five patients had 16 episodes of pneumothorax. In seven patients multiple cysts were observed on the surface of the lung during thoracotomy while computerized tomography (CT) scans revealed numerous cysts evenly distributed throughout the lung parenchyma. The procedures that confirmed the diagnosis included transbronchial lung biopsy (4), open lung biopsy (2), thoracoscopy (1), thoracotomy (3) and autopsy (1). Three specimens had to be revised before the histological diagnosis was confirmed. It was concluded that the important clues to a diagnosis of LAM are recurrent episodes of pneumothoraces in fertile women, progressive air-flow limitation, markedly reduced gas transfer and characteristic findings on thoracic CT scans. A specific request to the pathologist to stain lung tissue specimens for smooth muscle cells is mandatory.Keywords
This publication has 18 references indexed in Scilit:
- Pulmonary lymphangioleiomyomatosis: a report of 46 patients including a clinicopathologic study of prognostic factors.American Journal of Respiratory and Critical Care Medicine, 1995
- CT of the lung in patients with pulmonary emphysema: diagnosis, quantification, and correlation with pathologic and physiologic findings.American Journal of Roentgenology, 1994
- Pulmonary Mechanics in LymphangioleiomyomatosisAmerican Review of Respiratory Disease, 1991
- Computed Tomography of Chronic Diffuse Infiltrative Lung Disease: Part 1American Review of Respiratory Disease, 1990
- LymphangioleiomyomatosisThe New England Journal of Medicine, 1990
- Lymphangiomyomatosis: CT, chest radiographic, and functional correlations.Radiology, 1990
- Pulmonary lymphangioleiomyomatosis: CT findingsAmerican Journal of Roentgenology, 1989
- Pulmonary Lymphangiomyomatosis: With Particular Reference to Steroid-Receptor Assay Studies and Pathologic CorrelationMayo Clinic Proceedings, 1984
- Industrial Bronchitis and the Function of the LungsArchives of environmental health, 1962
- A case of chylous effusion due to leiomyosarcomaBritish Journal of Tuberculosis, 1937