Diffuse Cystic Lung Disease – Updates and Opportunities for Advancement

Purpose of Review To provide an updated, comprehensive overview of diffuse cystic lung diseases (DCLDs), highlighting diagnostic and therapeutic advancements and identifying opportunities for future investigations. Recent Findings We review the six main subcategories of DCLD – Lymphangioleiomyomatosis, Pulmonary Langerhans Cell Histiocytosis, Birt-Hogg-Dubé, Lymphoid Interstitial Pneumonia, Cystic Amyloidosis, and Light Chain Deposition Disease – along with other disease processes that may manifest as DCLD, including certain infections, malignancies, and genetic conditions. We outline the unique presentations, pathogenesis, diagnostic features, and management of these conditions. We also review recent updates in DCLD research, which primarily focus on exploring new diagnostic mechanisms and therapies for these rare conditions. Summary DCLD is a unique disease category that warrants further attention to strengthen clinicians’ confidence in caring for affected individuals and to expand diagnostic and treatment modalities available to this population.