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Cardiology1020 papers

Interstitial lung disease

Last edited: 27 days ago

Overview

Interstitial lung disease (ILD) encompasses a diverse group of pulmonary disorders characterized by inflammation and fibrosis affecting lung parenchyma, leading to impaired gas exchange and respiratory compromise 14112131.

Diagnosis

  • Clinical Presentation: Symptoms include dyspnea, cough, and fatigue 14.
  • Imaging: High-resolution computed tomography (HRCT) is crucial for identifying characteristic patterns such as reticular opacities, honeycombing, and ground-glass opacities 62252.
  • Functional Tests: Pulmonary function tests (PFTs) reveal restrictive pattern and reduced diffusing capacity 14.
  • Histopathology: Transbronchial cryobiopsy (TBCB) under conscious sedation can provide definitive diagnosis when imaging is inconclusive 556869.
  • Serological Markers: Specific autoantibodies may indicate underlying connective tissue disease 81436.
  • Multidisciplinary Approach: Involves pulmonology, radiology, and rheumatology for comprehensive evaluation 1533.
  • Management

  • First-Line Treatments:
  • - Anti-fibrotic Agents: Nintedanib and pirfenidone are recommended for progressive fibrosing ILD, including those associated with autoimmune diseases 4043444563. - Rheumatologic Management: For ILD associated with systemic autoimmune diseases, targeted immunosuppressive therapy (e.g., rituximab, mycophenolate mofetil) may be used 4041444570.
  • Adjunctive Therapies:
  • - Supportive Care: Oxygen therapy, pulmonary rehabilitation, and management of comorbidities 565. - Pulmonary Hypertension: Phosphodiesterase 5 inhibitors for ILD-associated pulmonary hypertension 50.

    Special Populations

  • Pediatrics: Early detection and multidisciplinary care are crucial; nintedanib and other antifibrotic therapies are being explored 2242.
  • Elderly: Frailty assessment is important; management focuses on minimizing complications and maintaining quality of life 1.
  • Comorbidities: Management requires consideration of coexisting conditions like heart failure and connective tissue diseases 2231.
  • Key Recommendations

  • Screening and Monitoring in Autoimmune Diseases: Regular screening for ILD in patients with systemic autoimmune rheumatic diseases using PFTs and HRCT; monitor with serum biomarkers (Evidence: Strong 2326)
  • Use of Anti-fibrotics: Initiate nintedanib or pirfenidone in patients with progressive fibrosing ILD to slow disease progression (Evidence: Strong 40434463)
  • Multidisciplinary Care: Implement multidisciplinary team meetings (MDT) for comprehensive management of ILD (Evidence: Moderate 15)
  • Patient-Centered Care: Incorporate patient values and preferences in ILD management decisions (Evidence: Expert opinion 44)
  • References

    Showing 100 most recent of 233 indexed papers.

    1 Chen Y, Zhu X, Wei J, Gu X, Fei J, Liu Q. Analysis of risk factors for frailty in older inpatients with interstitial lung disease. BMC pulmonary medicine 2026. link 2 Yu Z, Zhu H, Liao X. Antibody-drug conjugates-related interstitial lung diseases: data mining of the FAERS database. Expert opinion on drug safety 2026. link 3 Jeong J, Kim H, Lee S, Hwang J, Smith L, Woo HG et al.. Global Disproportionality Analysis of Adverse Event Reports on Interstitial Lung Diseases for Cancer-Targeted Therapies, 1968-2024. Respirology (Carlton, Vic.) 2026. link 4 Jose A, Sood N, Elwing JM, Akkanti B, Bajwa A, Bernardo R et al.. Pulmonary Hypertension Associated With Interstitial Lung Diseases. Chest 2026. link 5 Broncano J, Shifren A, Royuela J, Bhalla S, Martín de León R, Sánchez-González J et al.. Role of MRI in Interstitial Lung Diseases. Chest 2026. link 6 Baratella E, Borghesi A, Calandriello L, Cortese G, Della Casa G, Giraudo C et al.. Quantification of progressive pulmonary fibrosis by visual scoring of HRCT images: recommendations from Italian chest radiology experts. La Radiologia medica 2025. link 7 Ng MHV, Wettesinghe PV, Ryerson CJ, Khor YH. Clinical care in interstitial lung disease: a critical appraisal of clinical guidance documents. European respiratory review : an official journal of the European Respiratory Society 2025. link 8 Morais A, Duarte AC, Fernandes MO, Borba A, Ruano C, Marques ID et al.. Early detection of interstitial lung disease in rheumatic diseases: A joint statement from the Portuguese Pulmonology Society, the Portuguese Rheumatology Society, and the Portuguese Radiology and Nuclear Medicine Society. Pulmonology 2025. link 9 He D, Guler SA, Shannon CP, Ryerson CJ, Tebbutt SJ. Transcriptomics of interstitial lung disease: a systematic review and meta-analysis. The European respiratory journal 2025. link 10 Bundgaard-Nielsen M, Johnsen RH, Mortensen J, Shaker SB, Nielsen CTH. Radio-labelled fibroblast activation protein inhibitors in interstitial lung diseases - a systematic review. Autoimmunity reviews 2025. link 11 Ryerson CJ, Adegunsoye A, Piciucchi S, Hariri LP, Khor YH, Wijsenbeek MS et al.. Update of the international multidisciplinary classification of the interstitial pneumonias: an ERS/ATS statement. The European respiratory journal 2025. link 12 Ma L, Yang L, Su S, Chen W. A Comparative Bibliometric Analysis of Pediatric Interstitial Lung Disease Treatment: Global Trends, Advances, and Future Directions (2004-2024). Pediatric pulmonology 2025. link 13 Irene King S, Neumeier A, Graney B, Kreider M, Peterson R, Huie T. Defining Interstitial Lung Disease Education in Pulmonary Fellowship: A Mixed Methods Study. Chest 2025. link 14 Fassio A, Sebastiani M, Pollastri F, Cozzini F, Crotti C, Ughi N et al.. Updated systematic literature review and meta-analysis to inform the Italian Society of Rheumatology Recommendations on the treatment of rheumatoid arthritis-associated interstitial lung disease. Autoimmunity reviews 2025. link 15 Bauer CJ, Skowasch D, Kreuter M, Hamer OW, Behr J, Gläser S et al.. National survey on the presence of multidisciplinary meetings for interstitial lung diseases (ILD-MDM) in hospitals in Germany. Zeitschrift fur Rheumatologie 2025. link 16 Minagi A, Nawa H, Goda M, Niimura T, Miyata K, Hamano H et al.. Evaluation of Interstitial Lung Disease Complications Caused by Biologic Agents Using a Spontaneous Adverse Drug Reaction Reporting Database. Pharmacology research & perspectives 2025. link 17 Fang N, Wang X, Xu Z, Zhan H, Wang Y, Zhao B et al.. Immuno-OncologyThe adverse event of interstitial lung disease in patients treated with antibody-drug conjugates. The oncologist 2025. link 18 Liu Y, Feng R. Real-World Safety Characteristic of Iguratimod: A Pharmacovigilance Study Based on the Japanese Adverse Drug Event Report (JADER) Database. Biological & pharmaceutical bulletin 2025. link 19 Yavordios S, Beltramo G, Freppel R, Beau Salinas F, Le Beller C, Bihan K et al.. Diffuse lung diseases ascribed to drugs: a nationwide observational study over 37 years using the French Pharmacovigilance Database. The European respiratory journal 2025. link 20 Gong Z, Wang Q, Huang Y, Xu H. Signal stratification of antineoplastic drugs associated with interstitial lung disease: A multi-method signal detection analysis using the FAERS database. Medicine 2025. link 21 Buschulte K, Kabitz HJ, Hagmeyer L, Hammerl P, Esselmann A, Wiederhold C et al.. Baseline characteristics from the EXCITING-ILD registry. BMJ open respiratory research 2025. link 22 Lu RL, Huang Q, Yu TT, Liu DZ, Hong XP. The global burden of heart failure attributable to interstitial lung diseases: insights from the global burden of disease study 2021. BMC cardiovascular disorders 2025. link 23 Johnson SR, Bernstein EJ, Bolster MB, Chung JH, Danoff SK, George MD et al.. 2023 American College of Rheumatology (ACR)/American College of Chest Physicians (CHEST) Guideline for the Screening and Monitoring of Interstitial Lung Disease in People with Systemic Autoimmune Rheumatic Diseases. Arthritis & rheumatology (Hoboken, N.J.) 2024. link 24 Johnson SR, Bernstein EJ, Bolster MB, Chung JH, Danoff SK, George MD et al.. 2023 American College of Rheumatology (ACR)/American College of Chest Physicians (CHEST) Guideline for the Treatment of Interstitial Lung Disease in People with Systemic Autoimmune Rheumatic Diseases. Arthritis care & research 2024. link 25 Johnson SR, Bernstein EJ, Bolster MB, Chung JH, Danoff SK, George MD et al.. 2023 American College of Rheumatology (ACR)/American College of Chest Physicians (CHEST) Guideline for the Treatment of Interstitial Lung Disease in People with Systemic Autoimmune Rheumatic Diseases. Arthritis & rheumatology (Hoboken, N.J.) 2024. link 26 Johnson SR, Bernstein EJ, Bolster MB, Chung JH, Danoff SK, George MD et al.. 2023 American College of Rheumatology (ACR)/American College of Chest Physicians (CHEST) Guideline for the Screening and Monitoring of Interstitial Lung Disease in People with Systemic Autoimmune Rheumatic Diseases. Arthritis care & research 2024. link 27 Yang MM, Lee S, Neely J, Hinchcliff M, Wolters PJ, Sirota M. Gene expression meta-analysis reveals aging and cellular senescence signatures in scleroderma-associated interstitial lung disease. Frontiers in immunology 2024. link 28 Puiu R, Motoc NS, Lucaciu S, Ruta MV, Rajnoveanu RM, Todea DA et al.. The Role of Lung Microbiome in Fibrotic Interstitial Lung Disease-A Systematic Review. Biomolecules 2024. link 29 Isshiki T, Naiel S, Vierhout M, Otsubo K, Ali P, Tsubouchi K et al.. Therapeutic strategies to target connective tissue growth factor in fibrotic lung diseases. Pharmacology & therapeutics 2024. link 30 Narváez J, Aguilar-Coll M, Roig-Kim M, Maymó-Paituvi P, Palacios-Olid J, Nolla JM et al.. Janus kinase inhibitors in rheumatoid arthritis-associated interstitial lung disease: A systematic review and meta-analysis. Autoimmunity reviews 2024. link 31 Hinds DM, Nevel RJ, Liptzin DR, Barber AT. Pediatric Pulmonology 2023 year in review: Rare and diffuse lung disease. Pediatric pulmonology 2024. link 32 Drzewicka K, Zasłona Z. Metabolism-driven glycosylation represents therapeutic opportunities in interstitial lung diseases. Frontiers in immunology 2024. link 33 Pohunek P, Manali E, Vijverberg S, Carlens J, Chua F, Epaud R et al.. ERS statement on transition of care in childhood interstitial lung diseases. The European respiratory journal 2024. link 34 Mirza RD, Bolster MB, Johnson SR, Allen A, Bernstein EJ, Chung JH et al.. Assessing Patient Values and Preferences to Inform the 2023 American College of Rheumatology/American College of Chest Physicians Interstitial Lung Disease Guidelines. Arthritis care & research 2024. link 35 Salton F, Biolo M, Trotta L, Mondini L, Andrisano AG, Reccardini N et al.. A novel technique for conducting flexible bronchoscopy cryobiopsy under conscious sedation: An observational study. Medicine 2024. link 36 Lin W, Xu J, Liao Y, Lin X, Yang J, Zhuang W. Assessing safety concerns of interstitial lung disease associated with antibody-drug conjugates: a real-world pharmacovigilance evaluation of the FDA adverse event reporting system. International journal of clinical pharmacy 2024. link 37 Shi J, Liu X, Wu L, Jiang Y, Zhang Y, Wang Y. Interstitial lung disease with antibody-drug conjugates: a real-world pharmacovigilance study based on the FAERS database during the period 2014-2023. Therapeutic advances in respiratory disease 2024. link 38 Panagiotidou E, Βoutou A, Fouka E, Papakosta D, Chatzopoulos E, Sourla E et al.. Phenotyping exercise limitation of patients with Interstitial Fibrosing Lung Disease: the importance of exercise hemodynamics. Pulmonology 2024. link 39 Patel V, Patel J, Gan J, Rahiminejad M, Preston R, Mak SM et al.. Reporting of coronary artery calcification on chest CT studies in patients with interstitial lung disease. Clinical radiology 2024. link 40 Mankikian J, Caille A, Reynaud-Gaubert M, Agier MS, Bermudez J, Bonniaud P et al.. Rituximab and mycophenolate mofetil combination in patients with interstitial lung disease (EVER-ILD): a double-blind, randomised, placebo-controlled trial. The European respiratory journal 2023. link 41 Shah Gupta R, Koteci A, Morgan A, George PM, Quint JK. Incidence and prevalence of interstitial lung diseases worldwide: a systematic literature review. BMJ open respiratory research 2023. link 42 Deterding R, Young LR, DeBoer EM, Warburton D, Cunningham S, Schwerk N et al.. Nintedanib in children and adolescents with fibrosing interstitial lung diseases. The European respiratory journal 2023. link 43 Matteson EL, Aringer M, Burmester GR, Mueller H, Moros L, Kolb M. Effect of nintedanib in patients with progressive pulmonary fibrosis associated with rheumatoid arthritis: data from the INBUILD trial. Clinical rheumatology 2023. link 44 Inoue Y, Wells AU, Song JW, Xu Z, Kitamura H, Suda T et al.. Nintedanib in Asian patients with progressive fibrosing interstitial lung diseases: Results from the INBUILD trial. Respirology (Carlton, Vic.) 2023. link 45 Joy GM, Arbiv OA, Wong CK, Lok SD, Adderley NA, Dobosz KM et al.. Prevalence, imaging patterns and risk factors of interstitial lung disease in connective tissue disease: a systematic review and meta-analysis. European respiratory review : an official journal of the European Respiratory Society 2023. link 46 Barber AT, Liptzin DR, Gower WA, Hinds DM. Pediatric Pulmonology 2022 year in review: Rare and diffuse lung disease. Pediatric pulmonology 2023. link 47 Lee MT, Lee JW, Lee HJ, Lee JM, Choi JC, Gu KM et al.. Interstitial lung disease following COVID-19 vaccination: a disproportionality analysis using the Global Scale Pharmacovigilance Database (VigiBase). BMJ open respiratory research 2023. link 48 Chan RK, Horrigan M, Goh NSL, Khor YH. Clinical assessment for pulmonary hypertension in interstitial lung disease. Internal medicine journal 2023. link 49 Liang H, Fritz AV, Martin AK. Perioperative Circulatory Support and Management for Lung Transplantation: A Case-Based Review. Seminars in cardiothoracic and vascular anesthesia 2023. link 50 Dawes TJW, McCabe C, Dimopoulos K, Stewart I, Bax S, Harries C et al.. Phosphodiesterase 5 inhibitor treatment and survival in interstitial lung disease pulmonary hypertension: A Bayesian retrospective observational cohort study. Respirology (Carlton, Vic.) 2023. link 51 Sanders JL, Axelsson G, Putman R, Menon A, Dupuis J, Xu H et al.. The relationship between interstitial lung abnormalities, mortality, and multimorbidity: a cohort study. Thorax 2023. link 52 Korevaar DA, Colella S, Fally M, Camuset J, Colby TV, Hagmeyer L et al.. European Respiratory Society guidelines on transbronchial lung cryobiopsy in the diagnosis of interstitial lung diseases. The European respiratory journal 2022. link 53 Flaherty KR, Wells AU, Cottin V, Devaraj A, Inoue Y, Richeldi L et al.. Nintedanib in progressive interstitial lung diseases: data from the whole INBUILD trial. The European respiratory journal 2022. link 54 Matteson EL, Kelly C, Distler JHW, Hoffmann-Vold AM, Seibold JR, Mittoo S et al.. Nintedanib in Patients With Autoimmune Disease-Related Progressive Fibrosing Interstitial Lung Diseases: Subgroup Analysis of the INBUILD Trial. Arthritis & rheumatology (Hoboken, N.J.) 2022. link 55 Piotrowski WJ, Martusewicz-Boros MM, Białas AJ, Barczyk A, Batko B, Błasińska K et al.. Guidelines of the Polish Respiratory Society on the Diagnosis and Treatment of Progressive Fibrosing Interstitial Lung Diseases Other than Idiopathic Pulmonary Fibrosis. Advances in respiratory medicine 2022. link 56 Lemmers JMJ, Vonk MC, van den Ende CHM. Patient-reported outcomes to assess dyspnoea in interstitial lung disease and pulmonary hypertension: a systematic literature review of measurement properties. European respiratory review : an official journal of the European Respiratory Society 2022. link 57 Duman D. Progressive pulmonary fibrosis (PPF). 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Clinicoserological features of antisynthetase syndrome (ASyS)-associated interstitial lung disease presenting to respiratory services: comparison with idiopathic pulmonary fibrosis and ASyS diagnosed in rheumatology services. BMJ open respiratory research 2021. link 67 Rapp CK, Van Dijck I, Laugwitz L, Boon M, Briassoulis G, Ilia S et al.. Expanding the phenotypic spectrum of FINCA (fibrosis, neurodegeneration, and cerebral angiomatosis) syndrome beyond infancy. Clinical genetics 2021. link 68 Deasy KF, Walsh LJ, Kennedy MP, Henry MT. Endobronchial Balloon Blockers: A Retrospective Analysis of Their Implementation for Use in Transbronchial Cryobiopsy Under Conscious Sedation. Lung 2021. link 69 O'Mahony AM, Burke L, Cavazza A, Maher MM, Kennedy MP, Henry MT. Transbronchial lung cryobiopsy (TBLC) in the diagnosis of interstitial lung disease: experience of first 100 cases performed under conscious sedation with flexible bronchoscope. 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    Original source

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      Analysis of risk factors for frailty in older inpatients with interstitial lung disease.Chen Y, Zhu X, Wei J, Gu X, Fei J, Liu Q BMC pulmonary medicine (2026)
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