Discussion
Diagnosis With Brief Discussion
- Diagnosis
- Langerhans cell histiocytosis
- Radiologic Findings
- Brief Review
- PLCH is a part of spectrum of disease now collectively known as Langerhans' cell histiocytosis. The classification has been simplified and is based on the presence of single or multiorgan involvement. In the majority of patients PLCH is isolated to the lungs with only 5% to 15% demonstrating multiorgan involvement including bone lesions, skin lesions, and diabetes insipidus. Patients most commonly present with dypnea and cough, although up to 25% are asymptomatic at the time of disease discovery. PLCH primarily affects individuals in the 3rd to 5th decade of life, however, the relative frequency of males and females. The pathogenesis remains unknown, however, 90% or more of PLCH patients are cigarette smokers. The early imaging features are small nodules ranging from 1 to 10mm, bilateral, symmetric distributions. Nodules have irregular borders, predominenty distributed in the upper, middle lung zones with sparing of lung bases near costophrenic angle. As the diseases progresses, reticulonodular abnormalities are noted. Further progression results in a predominance of cystic changes. As cysts become more numerous, nodules tend to occur less frequently. The cystic manifestation can mimic that of bullous emphysema or lymphangioleiomyomatosis, with multifocal contiguous cysts measuring up to 2-3 cm in diameter. The end stage of PLCH is characterized by coarse reticular areas of opacity that tend to occur in the upper, middle lung zones that may progress to honeycomb lung. Lung volumes are normal or increased in most patients with PLCH. Pneumothorax is a recognized complication.
- References
- 1. Abbott GF, Rosado-de-Christenson ML, Franks TJ, Frazier AA, Galvin JR. From the archives of the AFIP Pulmonary langerhans cell histiocytosis. Radiographics. 2004 May-Jun;24(3):821-41
2. Galvin, Jeffrey R, Franks, Teri J. Smoking related lung diseases. J Thorac Imag. 2009; 24(4):274-284
- Keywords
- Lung, Interstitial lung disease, LCH, smoking related ILD,