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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

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: Current concepts in pathogenesis, diagnosis, and treatment.
Barnes H, Troy L, Lee CT, Sperling A, Strek M, Glaspole I
Allergy 2022 Feb;77(2):442-453. Epub 2021 Jul 29 doi: 10.1111/all.15017. PMID: 34293188
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

Etiology

Combined Pulmonary Fibrosis and Emphysema: A Narrative Review.
Nemoto M, Koo CW, Scanlon PD, Ryu JH
Mayo Clin Proc 2023 Nov;98(11):1685-1696. doi: 10.1016/j.mayocp.2023.05.002. PMID: 37923525
Usual interstitial pneumonia as a stand-alone diagnostic entity: the case for a paradigm shift?
Selman M, Pardo A, Wells AU
Lancet Respir Med 2023 Feb;11(2):188-196. Epub 2023 Jan 11 doi: 10.1016/S2213-2600(22)00475-1. PMID: 36640788
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
An update on interstitial lung disease.
Mudawi D, Heyes K, Hastings R, Rivera-Ortega P, Chaudhuri N
Br J Hosp Med (Lond) 2021 Jul 2;82(7):1-14. Epub 2021 Jul 20 doi: 10.12968/hmed.2020.0556. PMID: 34338019
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

Diagnosis

Occupational interstitial lung diseases.
Spagnolo P, Ryerson CJ, Guler S, Feary J, Churg A, Fontenot AP, Piciucchi S, Udwadia Z, Corte TJ, Wuyts WA, Johannson KA, Cottin V
J Intern Med 2023 Dec;294(6):798-815. Epub 2023 Aug 7 doi: 10.1111/joim.13707. PMID: 37535448
Hypersensitivity pneumonitis: new concepts and classifications.
Churg A
Mod Pathol 2022 Jan;35(Suppl 1):15-27. Epub 2021 Sep 16 doi: 10.1038/s41379-021-00866-y. PMID: 34531525
Hypersensitivity pneumonitis: Current concepts in pathogenesis, diagnosis, and treatment.
Barnes H, Troy L, Lee CT, Sperling A, Strek M, Glaspole I
Allergy 2022 Feb;77(2):442-453. Epub 2021 Jul 29 doi: 10.1111/all.15017. PMID: 34293188
An update on interstitial lung disease.
Mudawi D, Heyes K, Hastings R, Rivera-Ortega P, Chaudhuri N
Br J Hosp Med (Lond) 2021 Jul 2;82(7):1-14. Epub 2021 Jul 20 doi: 10.12968/hmed.2020.0556. PMID: 34338019
Diagnosis and Evaluation of Hypersensitivity Pneumonitis: CHEST Guideline and Expert Panel Report.
Fernández Pérez ER, Travis WD, Lynch DA, Brown KK, Johannson KA, Selman M, Ryu JH, Wells AU, Tony Huang YC, Pereira CAC, Scholand MB, Villar A, Inase N, Evans RB, Mette SA, Frazer-Green L
Chest 2021 Aug;160(2):e97-e156. Epub 2021 Apr 20 doi: 10.1016/j.chest.2021.03.066. PMID: 33861992

Therapy

Hypersensitivity Pneumonitis: Challenges of a Complex Disease.
Calaras D, David A, Vasarmidi E, Antoniou K, Corlateanu A
Can Respir J 2024;2024:4919951. Epub 2024 Jan 18 doi: 10.1155/2024/4919951. PMID: 38283656Free PMC Article
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
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
European protocols for the diagnosis and initial treatment of interstitial lung disease in children.
Bush A, Cunningham S, de Blic J, Barbato A, Clement A, Epaud R, Hengst M, Kiper N, Nicholson AG, Wetzke M, Snijders D, Schwerk N, Griese M; chILD-EU Collaboration
Thorax 2015 Nov;70(11):1078-84. Epub 2015 Jul 1 doi: 10.1136/thoraxjnl-2015-207349. PMID: 26135832

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
Hypersensitivity pneumonitis.
Watts MM, Grammer LC
Allergy Asthma Proc 2019 Nov 1;40(6):425-428. doi: 10.2500/aap.2019.40.4263. PMID: 31690386
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

Prevalence of occupational hypersensitivity pneumonitis: a systematic review and meta-analysis.
Zhang X, Igor B, Elena D, Olga R, Glazachev O
Int J Environ Health Res 2024 Nov;34(11):3891-3908. Epub 2024 Mar 27 doi: 10.1080/09603123.2024.2333021. PMID: 38544315
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
Transbronchial Biopsy and Cryobiopsy in the Diagnosis of Hypersensitivity Pneumonitis among Patients with Interstitial Lung Disease.
Chami HA, Diaz-Mendoza J, Chua A, Duggal A, Jenkins AR, Knight SL, Patolia S, Tamae-Kakazu M, Raghu G, Wilson KC
Ann Am Thorac Soc 2021 Jan;18(1):148-161. doi: 10.1513/AnnalsATS.202005-421OC. PMID: 32810411
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

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