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Hypersensitivity pneumonitis, familial

MedGen UID:
326724
Concept ID:
C1840386
Disease or Syndrome
Synonyms: Familial hypersensitivity pneumonitis; Hypersensitivity pneumonitis familial; Hypersensitivity Pneumonitis, Familial
 
Monarch Initiative: MONDO:0007776
OMIM®: 145300

Definition

An instance of hypersensitivity pneumonitis that is caused by an inherited modification of the individual's genome. [from MONDO]

Clinical features

From HPO
Hypersensitivity pneumonitis
MedGen UID:
1446
Concept ID:
C0002390
Disease or Syndrome
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

Recent clinical studies

Etiology

Okamoto T, Miyazaki Y, Tomita M, Tamaoka M, Inase N
Respiration 2013;85(5):384-90. Epub 2012 Jun 9 doi: 10.1159/000338123. PMID: 22688445
Allen DH, Basten A, Williams GV, Woolcock AJ
Am J Med 1975 Oct;59(4):505-14. doi: 10.1016/0002-9343(75)90258-2. PMID: 1080953

Diagnosis

Okamoto T, Miyazaki Y, Tomita M, Tamaoka M, Inase N
Respiration 2013;85(5):384-90. Epub 2012 Jun 9 doi: 10.1159/000338123. PMID: 22688445
Johnson CL, Bernstein IL, Gallagher JS, Bonventre PF, Brooks SM
Am Rev Respir Dis 1980 Aug;122(2):339-48. doi: 10.1164/arrd.1980.122.2.339. PMID: 6774642
Allen DH, Basten A, Williams GV, Woolcock AJ
Am J Med 1975 Oct;59(4):505-14. doi: 10.1016/0002-9343(75)90258-2. PMID: 1080953

Prognosis

Okamoto T, Miyazaki Y, Tomita M, Tamaoka M, Inase N
Respiration 2013;85(5):384-90. Epub 2012 Jun 9 doi: 10.1159/000338123. PMID: 22688445

Clinical prediction guides

Okamoto T, Miyazaki Y, Tomita M, Tamaoka M, Inase N
Respiration 2013;85(5):384-90. Epub 2012 Jun 9 doi: 10.1159/000338123. PMID: 22688445
Johnson CL, Bernstein IL, Gallagher JS, Bonventre PF, Brooks SM
Am Rev Respir Dis 1980 Aug;122(2):339-48. doi: 10.1164/arrd.1980.122.2.339. PMID: 6774642

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