Pulmonary Langerhans Cell Histiocytosis
DIAGNOSIS
SYMPTOMS1
- Dyspnea
- Nonproductive cough
- Fatigue
- Fever
- Weight loss
- Pleuritic chest pain
Common Patient Presentations
- Asymptomatic and radiographic abnormalities are detected on a routine chest X-ray in 15%-25% of patients1,3
- Respiratory symptoms (e.g., dry cough, dyspnea) on presentation are linked to systemic symptoms (e.g., fever, weight loss) in 67% of patients3
- Spontaneous pneumothorax causes chest pain in 10%-20% of patients1,3
DIAGNOSTICS
Imaging — CXR and HRCT1-3
- CXR shows bilaterally symmetric nodular opacities in the middle and upper lung fields with cystic changes
- Lung bases are often spared
- Diagnosis is confirmed with an HRCT— can differentiate LCH from other cystic lung diseases (e.g., lymphangleiomyomatosis (LAM), emphysema)
Pulmonary Function Tests (PFTs)1,3
- Findings are normal, restrictive, obstructive, or mixed depending on when the test is done during the course of the disease
- Most commonly, the diffusing capacity of the lung for carbon monoxide (DLCO) is reduced
Bronchoscopy/biopsy/bronchoalveolar lavage (BAL)1,3
- Indicated when imaging and PFTs are inconclusive
- Finding >5% of CD1a cells in BAL is highly suggestive of disease
Appearance may mimic chronic obstructive pulmonary disease (COPD) or LAM.1
