Pulmonary Lymphangiomyomatosis

Relation of pulmonary lymphangio-leiomyomatosis to use of the oral contraceptive pill and fertility in the UK: a national case control study.

“…The patients had a mean age of 34 (9) years at onset of symptoms and a median (range) time of 2 (0-29) years from onset of symptoms to diagnosis. Compared with control subjects, cases did not differ in the use of the oral contraceptive pill (odds ratio (OR) 0 39, 95% CI 0 09 to 1.68), diagnosis of fibroids (OR [3][4][5][6][7][8][9][10][11][12] The aetiology of pulmonary lymphangioleiomyomatosis is unknown, but sex hormones have been assumed to be important since the disease develops exclusively in women and almost invariably women of reproductive age. '3"6 Treatment has usually invoved antioestrogen measures in the form of oophorectomy347 or treatment with tamoxifen,8-'0 medroxyprogesterone,8 '-and luteinising hormone releasing hormone analogues.…”

Mutation analysis of the TSC1 and TSC2 genes in Japanese patients with pulmonary lymphangioleiomyomatosis

“…Pulmonary lymphangioleiomyomatosis (LAM) is a rare disease that almost exclusively affects women of reproductive age and is characterized by hamartomatous proliferation of abnormal smooth muscle cells (LAM cells) along the alveolar wall, vessels and lymphatics in the lungs, and occasionally mediastinal or retroperitoneal lymph nodes (Corrin et al 1975;Silverstein et al 1974). Clinical manifestations of pulmonary LAM include recurrent spontaneous pneumothorax, progressive dyspnea on exertion, chylothorax, and hemoptysis (Corrin et al 1975;Silverstein et al 1974).…”

“…Clinical manifestations of pulmonary LAM include recurrent spontaneous pneumothorax, progressive dyspnea on exertion, chylothorax, and hemoptysis (Corrin et al 1975;Silverstein et al 1974). The most common features of pulmonary function in patients with pulmonary LAM is the impairment of diffusing capacity and airflow obstruction (Chu et al 1999;Corrin et al 1975;Johnson and Tattersfield 1999;Silverstein et al 1974;Urban et al 1999). LAM has a diverse clinical course and some patients retain a good pulmonary function for a long period without antihormone therapy, although others show progressive deterioration leading to respiratory failure and cor pulmonale .…”

The changing face of a rare disease: lymphangioleiomyomatosis

“…Early retrospective studies based on small numbers reported a 5-and 10-year mortality of 40 and 80%, respectively [101,102]. More recent studies showed that LAM has a slower progression and a better prognosis than previously thought, even if the disease course varies widely in individual patients.…”