CHEST Guidelines-Diffuse-Cystic-Lung-Disease_2024

Diffuse-Cystic-Lung-Disease_2024_chest

Pdf Summary

Diffuse cystic lung diseases (DCLDs) are a varied group of disorders characterized by multiple thin-walled cysts in the lungs, discernible through imaging and distinct from typical fibrotic interstitial lung diseases (ILDs) in their clinical presentation, causes, and treatment. Key DCLDs include lymphangioleiomyomatosis (LAM), pulmonary Langerhans cell histiocytosis (PLCH), Birt-Hogg-Dubé syndrome (BHD), and lymphoid interstitial pneumonia (LIP). These conditions require precise diagnosis due to implications for management and treatment options. <br /><br />Chest CT scans are the most effective non-invasive method for identifying DCLDs, highlighting characteristic cystic patterns. LAM primarily affects women and is linked to tuberous sclerosis, with treatments like sirolimus targeting its root molecular pathways. BHD, an inherited syndrome leading to risk of pneumothorax and renal tumors, manifests as lung cysts alongside characteristic skin lesions and can be confirmed genetically. PLCH is strongly associated with smoking, presenting cysts primarily in the upper lung zones, with cessation being a primary intervention. LIP, associated with autoimmune disorders like Sjögren's syndrome, features lung cysts and often requires histological confirmation for diagnosis.<br /><br />Management across DCLDs varies, emphasizing personalized approaches based on specific disease features. For instance, patients with LAM may benefit from mTOR inhibitors, while BHD management focuses on reducing pneumothorax risk and monitoring for renal cancer. Despite their collective rarity, awareness and understanding of DCLDs are crucial, given their shared features with other diseases and potential for significant progression if untreated. Advances in imaging and molecular understanding continue to inform better diagnosis and treatment, with ongoing research needed to enhance patient outcomes further.

Keywords

Diffuse cystic lung diseases

DCLDs

lymphangioleiomyomatosis

pulmonary Langerhans cell histiocytosis

Birt-Hogg-Dubé syndrome

lymphoid interstitial pneumonia

chest CT scans

mTOR inhibitors

personalized management

imaging advances