Lymphangioleiomyomatosis
Lymphangioleiomyomatosis (pronunciation: lim-fan-je-o-li-o-mi-o-ma-to-sis) is a rare lung disease that primarily affects women of childbearing age. The disease is characterized by an abnormal growth of smooth muscle cells, especially in the lungs, lymphatic system, and kidneys.
Etymology
The term "Lymphangioleiomyomatosis" is derived from the Greek words lymph (λύμφα), meaning "clear water", angio (ἀγγεῖον), meaning "vessel", leiomyo (λείος μυς), meaning "smooth muscle", and matosis (μάτωσις), meaning "condition of".
Symptoms
The symptoms of Lymphangioleiomyomatosis include shortness of breath, cough, chest pain, and frequent lung infections. In some cases, the disease can lead to pneumothorax (collapsed lung) and chylous effusion (accumulation of lymphatic fluid in the pleural space).
Diagnosis
Diagnosis of Lymphangioleiomyomatosis is often challenging due to its rarity and non-specific symptoms. It is usually confirmed through a combination of clinical history, imaging studies such as high-resolution computed tomography (HRCT), and lung biopsy.
Treatment
Treatment for Lymphangioleiomyomatosis is primarily aimed at managing symptoms and preventing complications. This may include bronchodilators for breathlessness, antibiotics for lung infections, and surgical intervention for pneumothorax. The drug sirolimus (also known as rapamycin) has been shown to stabilize lung function and improve quality of life in some patients.
Prognosis
The prognosis of Lymphangioleiomyomatosis varies widely. Some individuals have stable disease for many years, while others may experience a rapid decline in lung function.
See also
- Tuberous sclerosis
- Pulmonary Langerhans cell histiocytosis
- Pulmonary alveolar proteinosis
- Pulmonary hypertension
External links
- Medical encyclopedia article on Lymphangioleiomyomatosis
- Wikipedia's article - Lymphangioleiomyomatosis
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