pulmonary disorders
Pulmonary Langerhans Cell Histiocytosis
Key Points
In pulmonary Langerhans cell histiocytosis (PLCH), monoclonal Langerhans cells proliferate in alveolar interstitium and bronchioles. Consider PLCH in patients age 20 to 40 who smoke and in whom chest x-ray shows bilaterally symmetric nodular opacities in the middle and upper lung fields with cystic changes. Confirm the diagnosis with high-resolution CT or, if results are inconclusive, lung biopsy. Recommend smoking cessation. Consider corticosteroids and cytotoxic drugs and, if smoking has ceased, lung transplantation. 