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Advanced Lymphangioleiomyomatosis (LAM)

Rare cystic lung disease in women progressing to respiratory failure and chylous complications.

Written by: Saygı Hospital Health Guide Editorial Board
Published:

This content is for general information; please consult your physician for diagnosis and treatment.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Göğüs Hastalıkları department. Book Appointment →

What is Advanced Lymphangioleiomyomatosis (LAM)?

Lymphangioleiomyomatosis is a rare neoplastic disorder driven by mutations in the TSC1 or TSC2 genes, characterized by proliferation of abnormal smooth muscle-like cells leading to cystic lung destruction, chylous effusions and angiomyolipomas of the kidneys.

Advanced disease presents with progressive dyspnea, recurrent pneumothorax, chylothorax and severely impaired diffusing capacity, sometimes requiring lung transplantation.

mTOR inhibitors such as sirolimus stabilize lung function and reduce chylous complications and angiomyolipoma size.

Symptoms

Progressive exertional dyspnea
Recurrent spontaneous pneumothorax
Chylous pleural effusion
Persistent dry cough
Hemoptysis
Abdominal mass from large angiomyolipoma
Fatigue and reduced exercise tolerance

Risk Factors

Female sex of reproductive or postmenopausal age
Tuberous sclerosis complex
Estrogen exposure
Family history of TSC
Pregnancy may worsen disease in some patients
Smoking aggravates lung function decline
Air travel may increase pneumothorax risk

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • New pneumothorax in a young woman
  • Recurrent chylothorax
  • Sudden severe dyspnea
  • Hemoptysis
  • Pregnancy planning with known LAM
  • Worsening pulmonary function tests
  • Suspected progression of angiomyolipoma

Treatment Methods

01
High-resolution CT and pulmonary function evaluation
02
Serum VEGF-D measurement and renal imaging
03
Sirolimus or other mTOR inhibitor therapy
04
Management of pneumothorax with pleurodesis when recurrent
05
Treatment of chylothorax with dietary and surgical measures
06
Lung transplantation for end-stage disease
07
Long-term multidisciplinary follow-up at expert centers

Which Department to Visit?

You can visit our Göğüs Hastalıkları department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

Learn About Göğüs Hastalıkları Department

Let us help you

You can make an appointment with our specialists or contact us for your concerns.

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Related Health Topics

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Asthma

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Asthma is characterized by wheezing, coughing and shortness of breath attacks; with proper treatment it can be kept under control.

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Pleural effusion is the accumulation of excess fluid in the pleural space, resulting from imbalances in fluid production and removal, and represents a manifestation of diverse cardiopulmonary, infectious, and malignant disorders.

Pneumothorax

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Pneumothorax is the presence of air in the pleural space resulting in partial or complete lung collapse, classified as spontaneous (primary/secondary), traumatic, or iatrogenic, with tension pneumothorax representing a life-threatening emergency.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.