1. Pulmonary lymphangioleiomyomatosis: literature update.
- Author
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Valentín-Mendoza S, Nieves-Nieves J, Fernández-Medero R, Fernández-Gonzales R, Adorno-Fontánez J, and Adorno-Fontánez E
- Subjects
- Diagnostic Imaging methods, Dyspnea etiology, Female, Humans, Lung Transplantation, Male, Metalloproteases physiology, Neoplasm Proteins physiology, Pneumothorax etiology, Pneumothorax surgery, Sex Distribution, Tuberous Sclerosis Complex 1 Protein, Tuberous Sclerosis Complex 2 Protein, Tumor Suppressor Proteins deficiency, Tumor Suppressor Proteins genetics, Tumor Suppressor Proteins physiology, Estrogens physiology, Lung Neoplasms diagnosis, Lung Neoplasms epidemiology, Lung Neoplasms genetics, Lung Neoplasms pathology, Lung Neoplasms therapy, Lymphangioleiomyomatosis diagnosis, Lymphangioleiomyomatosis epidemiology, Lymphangioleiomyomatosis genetics, Lymphangioleiomyomatosis pathology, Lymphangioleiomyomatosis therapy, Neoplasms, Hormone-Dependent diagnosis, Neoplasms, Hormone-Dependent epidemiology, Neoplasms, Hormone-Dependent genetics, Neoplasms, Hormone-Dependent pathology, Neoplasms, Hormone-Dependent therapy
- Abstract
Pulmonary lymphangioleiomyomatosis is an uncommon disease of unknown etiology characterized by the proliferation of abnormal smooth muscle cells in the lungs, leading to parenchymal destruction and progressive respiratory failure. The natural history of this disease remains poorly understood, primarily seen in women of childbearing age. The diagnosis can be difficult because symptoms are nonspecific and very similar to other respiratory diseases like asthma, emphysema and bronchitis. Lymphangioleiomyomatosis may not be diagnosed until a pneumothorax, chylothorax, interstitial lung disease or angiomyolipomas are discovered. The recent advances in genetic and molecular research provide new hope to discover the intricate mechanism of disease and evaluate new therapies. Internists, primary care physicians and pulmonologists should be aware of this condition in order to avoid delay in the diagnosis and institute appropriate therapy. The clinical features, pathophysiology, molecular genetics and medical treatment will be reviewed.
- Published
- 2013