Lymphangioleiomyomatosis
Lymphangioleiomyomatosis is a rare lung disease most commonly affecting women of childbearing age, which occurs sporadically or associated with tuberous sclerosis complex. Pathological findings are interstitial proliferation of abnormal smooth muscle, which obstructs lymphatics and causes multiple thin-walled pulmonary cysts.
Patients with lymphangioleiomyomatosis present with progressive dyspnoea, recurrent pneumothoraces, chylous collections, or haemoptysis. Extrapulmonary masses may also be found. Elevated serum vascular endothelial growth factor-D levels have been demonstrated in this condition. Lymphangioleiomyomatosis is thought to be accelerated by oestrogen and is believed to progress during pregnancy. However, successful pregnancies have been reported in women with mild disease. Treatment is usually supportive with oxygen, management of airflow obstruction, and treatment of pneumothorax if it occurs. Recently, genetic findings have suggested a benefit of mechanistic target of rapamycin inhibitors such as sirolimus.
More medical literature on Medic.Studio
More on the topic Lymphangioleiomyomatosis:
-
Infectious diseases -
Internal diseases -
Obstetrics and Gynaecology -
Pediatrics -
Veterinary medicine -
-
Conflictology -
Ecology -
Economy -
Finance -
History -
Law -
Medicine -
Philosophy -
Religious studies -