Lymphangioleiomyomatosis

Editor-In-Chief: Prab R Tumpati, MD
Obesity, Sleep & Internal medicine
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Lymphangioleiomyomatosis
Synonyms	LAM
Pronounce	N/A
Specialty	N/A
Symptoms	Shortness of breath, chest pain, cough, pneumothorax, chylothorax
Complications	Respiratory failure, pulmonary hypertension, kidney angiomyolipoma
Onset	Typically in women of childbearing age
Duration	Chronic
Types	N/A
Causes	Genetic mutation in the TSC1 or TSC2 genes
Risks	Female gender, tuberous sclerosis complex
Diagnosis	CT scan, lung biopsy, pulmonary function test
Differential diagnosis	Asthma, chronic obstructive pulmonary disease, interstitial lung disease
Prevention	N/A
Treatment	Sirolimus, everolimus, lung transplantation
Medication	Bronchodilators, hormonal therapy
Prognosis	Variable, can lead to progressive lung disease
Frequency	Rare, estimated 3-5 per million women
Deaths	Can be fatal if untreated

Lymphangioleiomyomatosis - low magnification

Lymphangioleiomyomatosis CT scan

File:Diagnostic Algorithm for Lymphangioleiomyomatosis.jpg

Diagnostic Algorithm for Lymphangioleiomyomatosis

Lymphangioleiomyomatosis (LAM) is a rare lung disease that typically affects women of childbearing age. In LAM, muscle cells that line the lungs' airways and blood vessels begin to multiply abnormally. These LAM cells form clusters and can cause blockages, leading to a variety of symptoms.

Symptoms

The symptoms of LAM can vary greatly from person to person. Some people may have no symptoms at all, while others may experience:

• Shortness of breath, especially during physical activity
• Chest pain
• Frequent cough, sometimes with blood (hemoptysis)
• Fatigue
• Wheezing

Causes

The exact cause of LAM is unknown. However, it is believed to be related to mutations in the TSC1 or TSC2 genes. These genes are involved in controlling cell growth, and mutations can lead to uncontrolled growth and division of cells.

Diagnosis

Diagnosis of LAM can be challenging, as its symptoms are similar to those of other lung diseases. However, certain tests can help in the diagnosis:

• High-resolution computed tomography (HRCT) scan
• Lung function test
• Biopsy
• Blood test

Treatment

There is currently no cure for LAM, but treatments can help manage symptoms and slow the progression of the disease. These may include:

• Bronchodilators
• Oxygen therapy
• Lung transplantation
• Medications such as sirolimus

Prognosis

The prognosis for individuals with LAM varies. Some people may remain stable for many years, while others may experience a rapid progression of the disease.

See also

• Tuberous sclerosis
• Pulmonary fibrosis
• Pulmonary hypertension

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