ArticleLung2026
Clinical Characteristics and Outcomes of Patients with Lymphangioleiomyomatosis.
Article in Lung, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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15 authors.
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Abstract
backgroundLymphangioleiomyomatosis (LAM) is a rare, progressive cystic lung disease that affects women of reproductive age. Although registries from Western countries have provided key insights, comprehensive nationwide data from Asian countries are scarce. This study aimed to characterise the clinical, functional and outcome features of Korean patients with LAM from a nationwide multicentre registry.
methodsWe analyzed data from patients with LAM enrolled in the Korean Nationwide Rare Interstitial Lung Disease Registry in 2024. Data on clinical and functional characteristics were collected from 20 centers. Longitudinal changes in pulmonary function were assessed using linear mixed-effects models. Individual annual forced expiratory volume in 1 s (FEV
resultsEighty-six women (mean age, 47.3 years) were included; 50% were asymptomatic at diagnosis and 61.6% had complications, including pneumothorax (29.1%) and renal angiomyolipoma (25.6%). Baseline mean forced vital capacity (FVC) and FEV
conclusionThis study provides a nationwide characterization of LAM in Korea, showing preserved lung function at diagnosis and frequent mTOR inhibitor use.
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