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Interstitial lung disease

MedGen UID:
60064
•Concept ID:
C0206062
•
Disease or Syndrome
Synonyms: Diffuse Parenchymal Lung Disease; Diffuse Parenchymal Lung Diseases; Interstitial Lung Disease; Interstitial Lung Diseases; Lung Disease, Interstitial; Lung Diseases, Interstitial
SNOMED CT: Diffuse parenchymal lung disease (233703007); Interstitial lung disease (233703007); ILD - Interstitial lung disease (233703007)

Definition

A category of lung diseases characterized by varying degrees of inflammation and fibrosis of the interstitial lung tissue. Causes include occupational lung exposures and drugs. In a minority of cases there is no clear cause and such cases are termed idiopathic interstitial pneumonia. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVInterstitial lung disease
Follow this link to review classifications for Interstitial lung disease in Orphanet.

Professional guidelines

PubMed

Diagnosis and Management of Myositis-Associated Lung Disease.
Hallowell RW, Danoff SK
Chest 2023 Jun;163(6):1476-1491. Epub 2023 Feb 9 doi: 10.1016/j.chest.2023.01.031. PMID: 36764512
Dermatomyositis: Diagnosis and treatment.
Waldman R, DeWane ME, Lu J
J Am Acad Dermatol 2020 Feb;82(2):283-296. Epub 2019 Jul 4 doi: 10.1016/j.jaad.2019.05.105. PMID: 31279813
Diagnosis of Idiopathic Pulmonary Fibrosis. An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline.
Raghu G, Remy-Jardin M, Myers JL, Richeldi L, Ryerson CJ, Lederer DJ, Behr J, Cottin V, Danoff SK, Morell F, Flaherty KR, Wells A, Martinez FJ, Azuma A, Bice TJ, Bouros D, Brown KK, Collard HR, Duggal A, Galvin L, Inoue Y, Jenkins RG, Johkoh T, Kazerooni EA, Kitaichi M, Knight SL, Mansour G, Nicholson AG, Pipavath SNJ, Buendía-Roldán I, Selman M, Travis WD, Walsh S, Wilson KC; American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Latin American Thoracic Society
Am J Respir Crit Care Med 2018 Sep 1;198(5):e44-e68. doi: 10.1164/rccm.201807-1255ST. PMID: 30168753

Recent clinical studies

Etiology

Noninvasive diagnostic modalities and prediction models for detecting pulmonary hypertension associated with interstitial lung disease: a narrative review.
Arvanitaki A, Diller GP, Gatzoulis MA, McCabe C, Price LC, Wort SJ
Eur Respir Rev 2024 Oct;33(174) Epub 2024 Oct 9 doi: 10.1183/16000617.0092-2024. PMID: 39384306Free PMC Article
Janus kinase inhibitors in rheumatoid arthritis-associated interstitial lung disease: A systematic review and meta-analysis.
Narváez J, Aguilar-Coll M, Roig-Kim M, Maymó-Paituvi P, Palacios-Olid J, Nolla JM, LLop D
Autoimmun Rev 2024 Oct;23(10):103636. Epub 2024 Sep 11 doi: 10.1016/j.autrev.2024.103636. PMID: 39270812
Cardiovascular disease in connective tissue disease-associated interstitial lung disease: A systematic review and meta-analysis of observational studies.
Hu Z, Wang H, Huang J, Yang G, Luo W, Zhong J, Zheng X, Wei X, Luo X, Xiong A
Autoimmun Rev 2024 Oct;23(10):103614. Epub 2024 Aug 31 doi: 10.1016/j.autrev.2024.103614. PMID: 39222675
Clinical characteristics and prognostic analysis of idiopathic inflammatory myopathy with positive anti-aminoacyl-tRNA synthetase antibodies: A single center experience.
Zhang D, Wang H, Zhou X, Yang J, Liu Y, Wang W, Jiang P, Fan B
Immun Inflamm Dis 2023 Nov;11(11):e1085. doi: 10.1002/iid3.1085. PMID: 38018600Free PMC Article
Nailfold capillaroscopy findings of interstitial pneumonia with autoimmune features.
Lee SH, Min HK, Kim SH, Kim YW, Yoo KH, Kim HJ, Kim IA, Kim HR
Korean J Intern Med 2023 Nov;38(6):903-911. Epub 2023 Jul 25 doi: 10.3904/kjim.2022.358. PMID: 37488834Free PMC Article

Diagnosis

Treatment patterns and patient journey in progressive pulmonary fibrosis: a cross-sectional survey.
Chaudhuri N, Spagnolo P, Valenzuela C, Amatto VC, Carter OT, Lee L, Small M, Kreuter M
Respir Res 2024 Oct 9;25(1):364. doi: 10.1186/s12931-024-02995-9. PMID: 39385232Free PMC Article
Noninvasive diagnostic modalities and prediction models for detecting pulmonary hypertension associated with interstitial lung disease: a narrative review.
Arvanitaki A, Diller GP, Gatzoulis MA, McCabe C, Price LC, Wort SJ
Eur Respir Rev 2024 Oct;33(174) Epub 2024 Oct 9 doi: 10.1183/16000617.0092-2024. PMID: 39384306Free PMC Article
Covert airflow obstruction dominates the overt ones in interstitial lung disease: An appraisal.
Bhattacharyya P, Karmakar S, Sengupta S, Paul M, Kar A, Dey D, Ghosh S, Sen S
Indian J Med Res 2024 Jul;160(1):70-77. doi: 10.25259/ijmr_114_24. PMID: 39382508Free PMC Article
Clinical characteristics and prognostic analysis of idiopathic inflammatory myopathy with positive anti-aminoacyl-tRNA synthetase antibodies: A single center experience.
Zhang D, Wang H, Zhou X, Yang J, Liu Y, Wang W, Jiang P, Fan B
Immun Inflamm Dis 2023 Nov;11(11):e1085. doi: 10.1002/iid3.1085. PMID: 38018600Free PMC Article
Nailfold capillaroscopy findings of interstitial pneumonia with autoimmune features.
Lee SH, Min HK, Kim SH, Kim YW, Yoo KH, Kim HJ, Kim IA, Kim HR
Korean J Intern Med 2023 Nov;38(6):903-911. Epub 2023 Jul 25 doi: 10.3904/kjim.2022.358. PMID: 37488834Free PMC Article

Therapy

State-of-the-art evidence in the treatment of systemic sclerosis.
Pope JE, Denton CP, Johnson SR, Fernandez-Codina A, Hudson M, Nevskaya T
Nat Rev Rheumatol 2023 Apr;19(4):212-226. Epub 2023 Feb 27 doi: 10.1038/s41584-023-00909-5. PMID: 36849541Free PMC Article
Inhaled Treprostinil in Pulmonary Hypertension Due to Interstitial Lung Disease.
Waxman A, Restrepo-Jaramillo R, Thenappan T, Ravichandran A, Engel P, Bajwa A, Allen R, Feldman J, Argula R, Smith P, Rollins K, Deng C, Peterson L, Bell H, Tapson V, Nathan SD
N Engl J Med 2021 Jan 28;384(4):325-334. Epub 2021 Jan 13 doi: 10.1056/NEJMoa2008470. PMID: 33440084
Interstitial lung disease in Sjögren's syndrome: a clinical review.
Luppi F, Sebastiani M, Silva M, Sverzellati N, Cavazza A, Salvarani C, Manfredi A
Clin Exp Rheumatol 2020 Jul-Aug;38 Suppl 126(4):291-300. Epub 2020 Oct 23 PMID: 33095142
Trastuzumab Deruxtecan in Previously Treated HER2-Positive Breast Cancer.
Modi S, Saura C, Yamashita T, Park YH, Kim SB, Tamura K, Andre F, Iwata H, Ito Y, Tsurutani J, Sohn J, Denduluri N, Perrin C, Aogi K, Tokunaga E, Im SA, Lee KS, Hurvitz SA, Cortes J, Lee C, Chen S, Zhang L, Shahidi J, Yver A, Krop I; DESTINY-Breast01 Investigators
N Engl J Med 2020 Feb 13;382(7):610-621. Epub 2019 Dec 11 doi: 10.1056/NEJMoa1914510. PMID: 31825192Free PMC Article
Nintedanib for Systemic Sclerosis-Associated Interstitial Lung Disease.
Distler O, Highland KB, Gahlemann M, Azuma A, Fischer A, Mayes MD, Raghu G, Sauter W, Girard M, Alves M, Clerisme-Beaty E, Stowasser S, Tetzlaff K, Kuwana M, Maher TM; SENSCIS Trial Investigators
N Engl J Med 2019 Jun 27;380(26):2518-2528. Epub 2019 May 20 doi: 10.1056/NEJMoa1903076. PMID: 31112379

Prognosis

Incidence and prevalence of interstitial lung diseases worldwide: a systematic literature review.
Shah Gupta R, Koteci A, Morgan A, George PM, Quint JK
BMJ Open Respir Res 2023 Jun;10(1) doi: 10.1136/bmjresp-2022-001291. PMID: 37308252Free PMC Article
Global incidence and prevalence of idiopathic pulmonary fibrosis.
Maher TM, Bendstrup E, Dron L, Langley J, Smith G, Khalid JM, Patel H, Kreuter M
Respir Res 2021 Jul 7;22(1):197. doi: 10.1186/s12931-021-01791-z. PMID: 34233665Free PMC Article
Disease manifestations and burden of illness in patients with acid sphingomyelinase deficiency (ASMD).
McGovern MM, Avetisyan R, Sanson BJ, Lidove O
Orphanet J Rare Dis 2017 Feb 23;12(1):41. doi: 10.1186/s13023-017-0572-x. PMID: 28228103Free PMC Article
Predicting survival across chronic interstitial lung disease: the ILD-GAP model.
Ryerson CJ, Vittinghoff E, Ley B, Lee JS, Mooney JJ, Jones KD, Elicker BM, Wolters PJ, Koth LL, King TE Jr, Collard HR
Chest 2014 Apr;145(4):723-728. doi: 10.1378/chest.13-1474. PMID: 24114524
Clinical course and prediction of survival in idiopathic pulmonary fibrosis.
Ley B, Collard HR, King TE Jr
Am J Respir Crit Care Med 2011 Feb 15;183(4):431-40. Epub 2010 Oct 8 doi: 10.1164/rccm.201006-0894CI. PMID: 20935110

Clinical prediction guides

Interstitial lung disease in Primary Sjögren's syndrome.
Lin W, Xin Z, Zhang J, Liu N, Ren X, Liu M, Su Y, Liu Y, Yang L, Guo S, Yang Y, Li Y, Cao J, Ning X, Li J, Xue H, Niu N, Chen Y, Li F, Sun L, Zhang X, Zhang F, Zhang W
BMC Pulm Med 2022 Feb 27;22(1):73. doi: 10.1186/s12890-022-01868-5. PMID: 35220958Free PMC Article
Presentation, diagnosis and clinical course of the spectrum of progressive-fibrosing interstitial lung diseases.
Cottin V, Hirani NA, Hotchkin DL, Nambiar AM, Ogura T, Otaola M, Skowasch D, Park JS, Poonyagariyagorn HK, Wuyts W, Wells AU
Eur Respir Rev 2018 Dec 31;27(150) Epub 2018 Dec 21 doi: 10.1183/16000617.0076-2018. PMID: 30578335Free PMC Article
Pulmonary manifestations of Sjögren's syndrome.
Flament T, Bigot A, Chaigne B, Henique H, Diot E, Marchand-Adam S
Eur Respir Rev 2016 Jun;25(140):110-23. doi: 10.1183/16000617.0011-2016. PMID: 27246587Free PMC Article
Interstitial lung disease.
Antoniou KM, Margaritopoulos GA, Tomassetti S, Bonella F, Costabel U, Poletti V
Eur Respir Rev 2014 Mar 1;23(131):40-54. doi: 10.1183/09059180.00009113. PMID: 24591661Free PMC Article
Interstitial lung disease in systemic sclerosis: a simple staging system.
Goh NS, Desai SR, Veeraraghavan S, Hansell DM, Copley SJ, Maher TM, Corte TJ, Sander CR, Ratoff J, Devaraj A, Bozovic G, Denton CP, Black CM, du Bois RM, Wells AU
Am J Respir Crit Care Med 2008 Jun 1;177(11):1248-54. Epub 2008 Mar 27 doi: 10.1164/rccm.200706-877OC. PMID: 18369202

Recent systematic reviews

Incidence and prevalence of interstitial lung diseases worldwide: a systematic literature review.
Shah Gupta R, Koteci A, Morgan A, George PM, Quint JK
BMJ Open Respir Res 2023 Jun;10(1) doi: 10.1136/bmjresp-2022-001291. PMID: 37308252Free PMC Article
Prevalence, imaging patterns and risk factors of interstitial lung disease in connective tissue disease: a systematic review and meta-analysis.
Joy GM, Arbiv OA, Wong CK, Lok SD, Adderley NA, Dobosz KM, Johannson KA, Ryerson CJ
Eur Respir Rev 2023 Mar 31;32(167) Epub 2023 Mar 8 doi: 10.1183/16000617.0210-2022. PMID: 36889782Free PMC Article
Management of MDA-5 antibody positive clinically amyopathic dermatomyositis associated interstitial lung disease: A systematic review.
McPherson M, Economidou S, Liampas A, Zis P, Parperis K
Semin Arthritis Rheum 2022 Apr;53:151959. Epub 2022 Jan 31 doi: 10.1016/j.semarthrit.2022.151959. PMID: 35134633
Pulmonary rehabilitation for interstitial lung disease.
Dowman L, Hill CJ, May A, Holland AE
Cochrane Database Syst Rev 2021 Feb 1;2(2):CD006322. doi: 10.1002/14651858.CD006322.pub4. PMID: 34559419Free PMC Article
Rheumatoid arthritis-associated interstitial lung disease: epidemiology, risk/prognostic factors, and treatment landscape.
Fazeli MS, Khaychuk V, Wittstock K, Han X, Crocket G, Lin M, Hill SL, Ferri L
Clin Exp Rheumatol 2021 Sep-Oct;39(5):1108-1118. Epub 2021 Feb 26 doi: 10.55563/clinexprheumatol/h9tc57. PMID: 33635222

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