Lymphangioleiomyomatosis
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
