A Multicenter Registry for Rare Interstitial Lung Diseases in Korea: Baseline Characteristics and Clinical Outcomes.

ABSTRACT:

Background

Rare interstitial lung diseases (ILDs) are a heterogeneous group of diffuse parenchymal lung disorders with low prevalence and diverse etiologies that pose diagnostic and therapeutic challenges. We aimed to establish a nationwide, multicenter registry of rare ILDs in South Korea to characterize their clinical features and outcomes.

Methods

We conducted a multicenter, observational cohort study involving 304 patients with rare ILDs including Birt-Hogg-Dubé syndrome (BHD), lymphangioleiomyomatosis (LAM), pulmonary alveolar proteinosis (PAP), pulmonary Langerhans cell histiocytosis (PLCH), pleuroparenchymal fibroelastosis (PPFE), and hypersensitivity pneumonitis (HP) enrolled across 20 centers in Korea between September 2023 and December 2024. Data on clinical charac

SUBMITTER: Oh JH 

PROVIDER: S-EPMC13646835 | biostudies-literature | 2026 Oct

REPOSITORIES: biostudies-literature