pulmonary Langerhans cell granulomatosis

Etiology

cigarette smoking is risk factor

Epidemiology

• more common in women; young males
• more common in smokers (95% of patients)
• more common in whites, rare in blacks
• uncommon disease, 1500 cases reported as of 1998

Pathology

• proliferation of histiocytes with numerous eosinophils interspersed
• pulmonary fibrosis replaces granulomatous changes as the disease progresses
• Langerhans' cells detected by electron microscopy (EM)

Clinical manifestations

• dyspnea in 40%
  • osteolytic rib lesions
  • recurrent pneumothorax
• fever
• fatigue
• weight loss
• spontaneous resolution is not uncommon

Laboratory

• lung biopsy or bronchoalveolar lavage (BAL)
• S100 staining on light microscopy
• no eosinophils in peripheral blood

Diagnostic procedures

Pulmonary function testing:

• restrictive pattern
  • decreased lung volumes
  • normal flow rate
  • decreased diffusion capacity
• generally, relatively good pulmonary function despite extensive chest x-ray abnormalities

Radiology

• chest X-ray
  • involvement is typically diffuse, bilateral & more pronounced in upper 2/3 of lungs
  • honeycomb cyst pattern
  • pneumothorax
• high resolution computed tomography (CT):
  • cystic, nodular changes

Management

• smoking cessation
• supportive
• corticosteroids
• Vinca alkaloids

More general terms

• Langerhans cell histiocytosis (LCH); histiocytosis X

Additional terms

• eosinophilia
• vinca alkaloid