Pulmonary Langerhans Cell Histiocytosis

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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

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