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New trends in cystic lung diseases

Presented By
Prof. Cormac McCarthy, University College Dublin, Ireland
Conference
ERS 2022
Doi
https://doi.org/10.55788/1527fb06
The progress of 3 common diffuse cystic lung diseases (DCLDs) was discussed, including lymphangioleiomyomatosis (LAM), pulmonary Langerhans cell histiocytosis (PLCH), and Birt-Hogg-Dubé syndrome (BHD). LAM cells were found that could serve as a diagnostic marker and VEGF-D has proven to be a distinctive biomarker for LAM. BRAF mutations in patients seem an indicator for PLCH and smoking cessation is an important intervention. FLCN mutations may offer a lead into the pathogenic mechanisms to be targeted in BHD. Finally, future research should focus on the common pathogenesis in DCLDs. “DCLDs are pathophysiologically distinct entities that share a common phenotypic manifestation of thin-walled cysts on chest radiology,” explained Prof. Cormac McCarthy (University College Dublin, Ireland) at the start of his presentation titled ‘New trends in cystic lung diseases’ [1]. The thin-walled cysts are usually less than 2 mm thick and are ...


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