lymphangioleiomyomatosis
Also known as Lymphangiomyomatosis, Lymphangiomyomatosis (morphologic abnormality), lung lymphangioleiomyomatosis, pulmonary lymphangioleiomyomatosis (disorder), pulmonary lymphangioleiomyomatosis, Lymphangioleiomyomatosis; LAM, Lung Lymphangiomyomatosis
Lymphangioleiomyomatosis (LAM) is a rare, progressive and systemic disease that typically results in cystic lung destruction. It predominantly affects women, especially during childbearing years. The term sporadic LAM is used for patients with LAM not associated with tuberous sclerosis complex (TSC), while TSC-LAM refers to LAM that is associated with TSC.
Key facts
- Medical condition(new).name
- Lymphangioleiomyomatosis (LAM)
- Medical condition(new).synonyms
- lymphangiomyomatosis, LAM
- Medical condition(new).image
- Lymphangioleiomyomatosis.jpg
- Medical condition(new).caption
- Figure A shows the location of the lungs and airways in the body. The inset image shows a cross-section of a healthy lung. Figure B shows a view of the lungs with LAM and a collapsed lung (pneumothorax). The inset image shows a cross-section of a lung with LAM.
via Wikipedia infobox
Research
2,339 papers- Lymphangioleiomyomatosis.Presse medicale (Paris, France : 1983) · 2023
- Lymphangioleiomyomatosis.Seminars in respiratory and critical care medicine · 2020
- Lymphangioleiomyomatosis: A Review.Archives of pathology & laboratory medicine · 2025
- Lymphangioleiomyomatosis.
Article
27 sectionsContents
- Signs and symptoms
- Genetics
- Pathophysiology
- Diagnosis
- Chest radiograph
- Computed tomography
- Ventilation-perfusion scans
- Positron emission tomography
- Abdominal imaging
- Central nervous system imaging
- Pulmonary function studies
- Pathology
- Treatment
- Treatment of pneumothoraces
- Treatment of chylothorax
- Treatment of renal angiomyolipoma
- Treatment of lymphangioleiomyoma
- Medication
- Other management
- Prognosis
- Epidemiology
- Pregnancy
- Society
- In popular culture
- See also
- References
- External links
Lymphangioleiomyomatosis (LAM) is a rare, progressive and systemic disease that typically results in cystic lung destruction. It predominantly affects women, especially during childbearing years. The term sporadic LAM is used for patients with LAM not associated with tuberous sclerosis complex (TSC), while TSC-LAM refers to LAM that is associated with TSC.
==Signs and symptoms== The average age of onset is the early-to-mid-30s. Exertional dyspnea (shortness of breath) and spontaneous pneumothorax (lung collapse) have been reported as the initial presentation of the disease in 49% and 46% of patients, respectively.