New insights in lymphangioleiomyomatosis and pulmonary Langerhans cell histiocytosis

Abstract

Lymphangioleiomyomatosis (LAM) and pulmonary Langerhans cell histiocytosis (PLCH) are rare diseases that lead to progressive cystic destruction of the lungs. Despite their distinctive characteristics, these diseases share several features. Patients affected by LAM or PLCH have similar radiological cystic patterns, a similar age of onset, and the possibility of extrapulmonary involvement. In this review, the recent advances in the understanding of the molecular pathogenesis, as well as the current and most promising biomarkers and therapeutic approaches, are described.

FIGURE 1

High-resolution chest computed tomography images of a patient with lymphangioleiomyomatosis, showing round-shaped, thin-walled cysts distributed diffusely throughout the lungs without sparing of lung bases (a, b), and a patient with pulmonary Langerhans cell histiocytosis with evidence of micronodules and cystic lesions mainly involving middle and upper lung fields (c, d).