What is Lymphangioleiomyomatosis of the Lung?
Lymphangioleiomyomatosis (LAM) is a rare lung disease that primarily affects women between puberty and menopause. In LAM, abnormal cells grow in the lungs and other parts of the body, damaging healthy lung tissue. Over time, these cells form cysts, which impair lung function, reduce oxygen levels, and make breathing increasingly difficult.
Causes of LAM
LAM has two main types, each with different causes:
- TSC-LAM: Occurs in individuals with a genetic condition called tuberous sclerosis complex (TSC).
- Sporadic LAM: Caused by a random genetic mutation that is not hereditary.
Signs and Symptoms
Common symptoms of LAM include:
- Shortness of breath
- Coughing, sometimes with bloody mucus
- Chest pain or tightness
- Excessive fatigue
- Wheezing (a whistling sound while breathing)
- Sudden chest pain and difficulty breathing, which may indicate a collapsed lung
- Swelling, pain, or tenderness in lymph nodes
Diagnosis
The following methods are used to diagnose Lymphangioleiomyomatosis (LAM):
- Medical History and Examination: The doctor reviews symptoms, their progression, and examines the chest and lymph nodes to assess the tumour’s origin and extent.
- Chest X-ray: Identifies changes in the lungs as the disease advances.
- Chest CT Scan: Provides detailed imaging to detect lung cysts.
- Lung Function Test: Evaluates how well the lungs are working.
- Blood Tests: Measures levels of VEGF-D, a protein often elevated in LAM patients.
- Biopsy: A small sample of lung tissue may be taken to confirm the diagnosis.
Treatment Options
While there is no cure for LAM, treatments can manage symptoms and slow disease progression:
- Medications: Sirolimus, for instance, can help reduce lung damage.
- Oxygen Therapy: Helps alleviate breathing difficulties.
- Collapsed Lung Management: Immediate medical intervention is necessary if the lung collapses.
By recognising symptoms early and pursuing appropriate treatment, patients can improve their quality of life and manage the disease more effectively.
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