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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.
 
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.

Professional guidelines

PubMed

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
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
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

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
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
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
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
Kligerman SJ, Henry T, Lin CT, Franks TJ, Galvin JR
Radiographics 2015 Sep-Oct;35(5):1360-80. Epub 2015 Aug 14 doi: 10.1148/rg.2015140308. PMID: 26274445

Diagnosis

Marinescu DC, Raghu G, Remy-Jardin M, Travis WD, Adegunsoye A, Beasley MB, Chung JH, Churg A, Cottin V, Egashira R, Fernández Pérez ER, Inoue Y, Johannson KA, Kazerooni EA, Khor YH, Lynch DA, Müller NL, Myers JL, Nicholson AG, Rajan S, Saito-Koyama R, Troy L, Walsh SLF, Wells AU, Wijsenbeek MS, Wright JL, Ryerson CJ
Chest 2022 Sep;162(3):614-629. Epub 2022 Jun 20 doi: 10.1016/j.chest.2022.06.013. PMID: 35738345
Churg A
Mod Pathol 2022 Jan;35(Suppl 1):15-27. Epub 2021 Sep 16 doi: 10.1038/s41379-021-00866-y. PMID: 34531525
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
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
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

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
Waxman AB, Elia D, Adir Y, Humbert M, Harari S
Eur Respir Rev 2022 Sep 30;31(165) Epub 2022 Jul 12 doi: 10.1183/16000617.0220-2021. PMID: 35831007Free PMC Article
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
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
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

Cottin V, Si-Mohamed S, Diesler R, Bonniaud P, Valenzuela C
Curr Opin Pulm Med 2022 Sep 1;28(5):432-440. Epub 2022 Jul 18 doi: 10.1097/MCP.0000000000000907. PMID: 35855575
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
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
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
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

Maher TM
JAMA 2024 May 21;331(19):1655-1665. doi: 10.1001/jama.2024.3669. PMID: 38648021
Waxman AB, Elia D, Adir Y, Humbert M, Harari S
Eur Respir Rev 2022 Sep 30;31(165) Epub 2022 Jul 12 doi: 10.1183/16000617.0220-2021. PMID: 35831007Free PMC Article
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
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
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

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
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
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
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
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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