DIAGNOSIS

SYMPTOMS1,3,4

  • Dyspnea
  • Cough
  • Chest pain
  • Hemoptysis
  • Spontaneous pneumothorax is common (36%)

LAM is often confused with other obstructive lung diseases - patients are often diagnosed with asthma, emphysema, or COPD before LAM is suspected.3

DIAGNOSIS

Imaging — CXR and HRCT3

  • Multiple, small, diffusely distributed cysts

Lung Biopsy3

  • If HRCT is nondiagnostic
  • Abnormal proliferation of LAM cells with cystic changes

PFTs1,3

  • Obstructive or mixed obstructive and restrictive pattern
  • Increased total lung capacity (TLC) and residual volume (RV)
  • Reduced partial pressure of oxygen (PaO2) and DLCO

See also

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Interstitial lung disease in systemic sclerosis with a focus on chest CT

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