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Clinical Challenges and Future Directions of Interstitial Lung Disease

A special issue of Journal of Clinical Medicine (ISSN 2077-0383). This special issue belongs to the section "Respiratory Medicine".

Deadline for manuscript submissions: 20 September 2026 | Viewed by 474

Special Issue Editor


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Guest Editor
Division of Respirology, Neurology and Rheumatology, Department of Medicine, Kurume University School of Medicine, 67 Asahi-machi, Kurume, Fukuoka 830-0011, Japan
Interests: lung pathology; pulmonary pathology; interstitial pneumonia; tuberculosis; interstitial lung disease
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Special Issue Information

Dear Colleagues,

Interstitial lung diseases (ILDs) represent a heterogeneous group of disorders characterized by varying degrees of inflammation and fibrosis in the lung parenchyma. Despite advancements in our understanding, several key clinical challenges remain. Early and accurate diagnosis is often difficult due to non-specific symptoms and overlapping radiologic and histopathologic features. In this context, multidisciplinary discussion (MDD) involving pulmonologists, radiologists, and pathologists plays a pivotal role in achieving diagnostic consensus. Optimizing treatment strategies—particularly for progressive pulmonary fibrosis (PPF)—continues to be a major challenge. Furthermore, non-pharmacological interventions such as pulmonary rehabilitation and long-term oxygen therapy are recognized as vital components of care, yet their implementation and standardization are inconsistent across clinical settings. Additionally, end-of-life care and the timely integration of palliative approaches are essential but often underutilized aspects of ILD management.

This Special Issue welcomes original research and review articles that provide solid evidence on the diagnosis, treatment, and patient-centered management of ILDs. Submissions focusing on efforts to promote early detection, improve diagnostic processes, refine therapeutic decision making, and enhance supportive and palliative care are particularly encouraged. Through this Special Issue, we aim to contribute to more effective and compassionate care for patients with ILDs.

Dr. Yoshiaki Zaizen
Guest Editor

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Keywords

  • interstitial lung disease
  • progressive pulmonary fibrosis
  • early detection
  • clinical diagnosis
  • multidisciplinary discussion
  • treatment
  • non-pharmacological therapy
  • end-of-life care
  • patient-centered management

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Published Papers (1 paper)

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Research

11 pages, 1291 KB  
Article
Combined HDL–BMI Phenotyping Provides Incremental Prognostic Value in Idiopathic Pulmonary Fibrosis
by Qinxue Shen, Xiaoli Ouyang, Yuexin Tan, Qing Zhang, Feng Hu, Shengyang He and Hong Peng
J. Clin. Med. 2026, 15(7), 2525; https://doi.org/10.3390/jcm15072525 - 26 Mar 2026
Viewed by 239
Abstract
Background/Objectives: Risk stratification in idiopathic pulmonary fibrosis (IPF) remains primarily based on physiological indices, yet increasing evidence suggests that systemic metabolic and nutritional vulnerability may influence outcomes in chronic interstitial lung disease. Methods: In this longitudinal, single-center cohort, 211 patients with [...] Read more.
Background/Objectives: Risk stratification in idiopathic pulmonary fibrosis (IPF) remains primarily based on physiological indices, yet increasing evidence suggests that systemic metabolic and nutritional vulnerability may influence outcomes in chronic interstitial lung disease. Methods: In this longitudinal, single-center cohort, 211 patients with IPF were followed from diagnosis until death or last follow-up. Baseline lipid profiles and body mass index (BMI) were assessed. A metabolic–nutritional phenotype was constructed using high-density lipoprotein cholesterol (HDL) and BMI. Survival was analyzed using Kaplan–Meier and multivariable Cox models adjusted for GAP stage. Incremental prognostic value beyond the GAP index was evaluated using Harrell’s C-index and time-dependent ROC analysis. Results: During a median follow-up of 29 months, 134 patients (63.5%) died. Lower HDL levels were associated with increased mortality in unadjusted analysis (HR = 1.45, 95% CI 1.03–2.04) but were not independently predictive after adjustment. In contrast, the combined HDL–BMI phenotype independently stratified mortality risk. Compared with HDL ≤ 1.0 mmol/L and BMI ≤ 24 kg/m2, patients with HDL > 1.0 mmol/L and BMI > 24 kg/m2 had significantly lower mortality (adjusted HR = 0.48, 95% CI 0.29–0.80), with stronger associations among those aged ≥ 65 years (adjusted HR = 0.37, 95% CI 0.18–0.74). The addition of HDL–BMI improved discrimination beyond GAP (C-index: 0.585 vs. 0.618; 36-month AUC: 0.633 vs. 0.675; NRI: 0.243). Conclusions: The coexistence of HDL ≤ 1.0 mmol/L and BMI ≤ 24 kg/m2 identified a subgroup with poorer survival in IPF. This combined metabolic–nutritional phenotype improved mortality risk stratification beyond the GAP stage. Full article
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