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Hypersensitivity pneumonitis

MedGen UID:
1446
•Concept ID:
C0002390
•
Disease or Syndrome
Synonyms: Allergic interstitial pneumonitis; Allergic pneumonitis; Extrinsic allergic alveolitis; Extrinsic allergic pneumonia hypersensitivity Pneumonitis
SNOMED CT: Extrinsic allergic alveolitis (37471005); Extrinsic allergic bronchiolo-alveolitis (37471005); Hypersensitivity pneumonia (37471005); Allergic alveolitis (37471005); Allergic pneumonitis (37471005); Allergic interstitial pneumonitis (37471005); Hypersensitivity pneumonitis (37471005); Bagpipe lung (37471005); EAA - Extrinsic allergic alveolitis (37471005)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
HPO: HP:0006516
Monarch Initiative: MONDO:0017853
Orphanet: ORPHA31740

Definition

Hypersensitivity pneumonitis involves inhalation of an antigen. This leads to an exaggerated immune response and a following inflammation of the alveoli within the lungs. The main feature of chronic hypersensitivity pneumonitis on lung biopsies is expansion of the interstitium by lymphocytes accompanied by an occasional multinucleated giant cell or loose granuloma. After exposure to the provoking antigen, following symptoms can be seen [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVHypersensitivity pneumonitis
Follow this link to review classifications for Hypersensitivity pneumonitis in Orphanet.

Conditions with this feature

Hypersensitivity pneumonitis, familial
MedGen UID:
326724
•Concept ID:
C1840386
•
Disease or Syndrome
An instance of hypersensitivity pneumonitis that is caused by an inherited modification of the individual's genome.
See: Condition Record
Hypersensitivity pneumonitis, familial

Professional guidelines

PubMed

Immunomodulatory treatment of interstitial lung disease.
van den Bosch L, Luppi F, Ferrara G, Mura M
Ther Adv Respir Dis 2022 Jan-Dec;16:17534666221117002. doi: 10.1177/17534666221117002. PMID: 35938712Free PMC Article
Diagnosis, course and management of hypersensitivity pneumonitis.
Hamblin M, Prosch H, Vašáková M
Eur Respir Rev 2022 Mar 31;31(163) Epub 2022 Feb 9 doi: 10.1183/16000617.0169-2021. PMID: 35140104Free PMC Article
Diagnosis of Hypersensitivity Pneumonitis in Adults. An Official ATS/JRS/ALAT Clinical Practice Guideline.
Raghu G, Remy-Jardin M, Ryerson CJ, Myers JL, Kreuter M, Vasakova M, Bargagli E, Chung JH, Collins BF, Bendstrup E, Chami HA, Chua AT, Corte TJ, Dalphin JC, Danoff SK, Diaz-Mendoza J, Duggal A, Egashira R, Ewing T, Gulati M, Inoue Y, Jenkins AR, Johannson KA, Johkoh T, Tamae-Kakazu M, Kitaichi M, Knight SL, Koschel D, Lederer DJ, Mageto Y, Maier LA, Matiz C, Morell F, Nicholson AG, Patolia S, Pereira CA, Renzoni EA, Salisbury ML, Selman M, Walsh SLF, Wuyts WA, Wilson KC
Am J Respir Crit Care Med 2020 Aug 1;202(3):e36-e69. doi: 10.1164/rccm.202005-2032ST. PMID: 32706311Free PMC Article

Recent clinical studies

Therapy

Immunomodulatory treatment of interstitial lung disease.
van den Bosch L, Luppi F, Ferrara G, Mura M
Ther Adv Respir Dis 2022 Jan-Dec;16:17534666221117002. doi: 10.1177/17534666221117002. PMID: 35938712Free PMC Article
Proteomic biomarkers of progressive fibrosing interstitial lung disease: a multicentre cohort analysis.
Bowman WS, Newton CA, Linderholm AL, Neely ML, Pugashetti JV, Kaul B, Vo V, Echt GA, Leon W, Shah RJ, Huang Y, Garcia CK, Wolters PJ, Oldham JM
Lancet Respir Med 2022 Jun;10(6):593-602. Epub 2022 Jan 18 doi: 10.1016/S2213-2600(21)00503-8. PMID: 35063079Free PMC Article
Hypersensitivity pneumonitis.
Costabel U, Miyazaki Y, Pardo A, Koschel D, Bonella F, Spagnolo P, Guzman J, Ryerson CJ, Selman M
Nat Rev Dis Primers 2020 Aug 6;6(1):65. doi: 10.1038/s41572-020-0191-z. PMID: 32764620
Nintedanib in patients with progressive fibrosing interstitial lung diseases-subgroup analyses by interstitial lung disease diagnosis in the INBUILD trial: a randomised, double-blind, placebo-controlled, parallel-group trial.
Wells AU, Flaherty KR, Brown KK, Inoue Y, Devaraj A, Richeldi L, Moua T, Crestani B, Wuyts WA, Stowasser S, Quaresma M, Goeldner RG, Schlenker-Herceg R, Kolb M; INBUILD trial investigators
Lancet Respir Med 2020 May;8(5):453-460. Epub 2020 Mar 5 doi: 10.1016/S2213-2600(20)30036-9. PMID: 32145830
Potential of nintedanib in treatment of progressive fibrosing interstitial lung diseases.
Wollin L, Distler JHW, Redente EF, Riches DWH, Stowasser S, Schlenker-Herceg R, Maher TM, Kolb M
Eur Respir J 2019 Sep;54(3) Epub 2019 Sep 19 doi: 10.1183/13993003.00161-2019. PMID: 31285305Free PMC Article

Prognosis

Interstitial Lung Disease: A Review.
Maher TM
JAMA 2024 May 21;331(19):1655-1665. doi: 10.1001/jama.2024.3669. PMID: 38648021
Diagnosis, course and management of hypersensitivity pneumonitis.
Hamblin M, Prosch H, Vašáková M
Eur Respir Rev 2022 Mar 31;31(163) Epub 2022 Feb 9 doi: 10.1183/16000617.0169-2021. PMID: 35140104Free PMC Article
Hypersensitivity pneumonitis.
Costabel U, Miyazaki Y, Pardo A, Koschel D, Bonella F, Spagnolo P, Guzman J, Ryerson CJ, Selman M
Nat Rev Dis Primers 2020 Aug 6;6(1):65. doi: 10.1038/s41572-020-0191-z. PMID: 32764620
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
Idiopathic non-specific interstitial pneumonia.
Belloli EA, Beckford R, Hadley R, Flaherty KR
Respirology 2016 Feb;21(2):259-68. Epub 2015 Nov 13 doi: 10.1111/resp.12674. PMID: 26564810

Clinical prediction guides

Interstitial Lung Disease: A Review.
Maher TM
JAMA 2024 May 21;331(19):1655-1665. doi: 10.1001/jama.2024.3669. PMID: 38648021
Validation of Proposed Criteria for Progressive Pulmonary Fibrosis.
Pugashetti JV, Adegunsoye A, Wu Z, Lee CT, Srikrishnan A, Ghodrati S, Vo V, Renzoni EA, Wells AU, Garcia CK, Chua F, Newton CA, Molyneaux PL, Oldham JM
Am J Respir Crit Care Med 2023 Jan 1;207(1):69-76. doi: 10.1164/rccm.202201-0124OC. PMID: 35943866Free PMC Article
Proteomic biomarkers of progressive fibrosing interstitial lung disease: a multicentre cohort analysis.
Bowman WS, Newton CA, Linderholm AL, Neely ML, Pugashetti JV, Kaul B, Vo V, Echt GA, Leon W, Shah RJ, Huang Y, Garcia CK, Wolters PJ, Oldham JM
Lancet Respir Med 2022 Jun;10(6):593-602. Epub 2022 Jan 18 doi: 10.1016/S2213-2600(21)00503-8. PMID: 35063079Free PMC Article
Immune Checkpoint Inhibitor Therapy-related Pneumonitis: Patterns and Management.
Kalisz KR, Ramaiya NH, Laukamp KR, Gupta A
Radiographics 2019 Nov-Dec;39(7):1923-1937. Epub 2019 Oct 4 doi: 10.1148/rg.2019190036. PMID: 31584861
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

Recent systematic reviews

Risk factors associated with mortality in hypersensitivity pneumonitis: a meta-analysis.
Dasgupta S, Bhattacharya A, Abhijit RD, Roy Chowdhury S, Chaudhury K
Expert Rev Respir Med 2022 Jul;16(7):801-811. Epub 2022 Jul 25 doi: 10.1080/17476348.2022.2100352. PMID: 35819125
Nintedanib in Progressive Pulmonary Fibrosis: A Systematic Review and Meta-Analysis.
Ghazipura M, Mammen MJ, Herman DD, Hon SM, Bissell BD, Macrea M, Kheir F, Khor YH, Knight SL, Raghu G, Wilson KC, Hossain T
Ann Am Thorac Soc 2022 Jun;19(6):1040-1049. doi: 10.1513/AnnalsATS.202103-343OC. PMID: 35499854
Occupational causes of hypersensitivity pneumonitis: a systematic review and compendium.
Kongsupon N, Walters GI, Sadhra SS
Occup Med (Lond) 2021 Oct 1;71(6-7):255-259. doi: 10.1093/occmed/kqab082. PMID: 34370035Free PMC Article
Bronchoalveolar lavage fluid lymphocytosis in chronic hypersensitivity pneumonitis: a systematic review and meta-analysis.
Adderley N, Humphreys CJ, Barnes H, Ley B, Premji ZA, Johannson KA
Eur Respir J 2020 Aug;56(2) Epub 2020 Aug 6 doi: 10.1183/13993003.00206-2020. PMID: 32265306
Systematic review of respiratory health among dairy workers.
Reynolds SJ, Nonnenmann MW, Basinas I, Davidson M, Elfman L, Gordon J, Kirychuck S, Reed S, Schaeffer JW, Schenker MB, Schlünssen V, Sigsgaard T
J Agromedicine 2013;18(3):219-43. doi: 10.1080/1059924X.2013.797374. PMID: 23844790

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