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Idiopathic chronic eosinophilic pneumonia

MedGen UID:
443938
Concept ID:
C2930941
Disease or Syndrome
Synonyms: Carrington syndrome; Carrington's Disease; Carrington's pulmonary eosinophilia; Chronic eosinophilic pneumonia (CEP); Eosinophilic idiopathic chronic pneumopathy
SNOMED CT: Idiopathic chronic eosinophilic pneumonia (724500003)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0017363
Orphanet: ORPHA2902

Definition

A rare, severe, interstitial lung disease characterized by insidious onset with subacute or chronic non-specific respiratory manifestations (dyspnea, cough, wheezing) often associated with systemic manifestations (fatigue, malaise, weight loss) and a history of asthma (up to half of patients). Eosinophilia is present in most cases, usually in excess of 1000 cells/mm3. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVIdiopathic chronic eosinophilic pneumonia

Professional guidelines

PubMed

Delcros Q, Taillé C, Vallée A, Brun AL, Chenivesse C, Couture P, Daboussi S, Guilleminault L, Juster F, Kahn JE, Lefèvre G, Liozon E, Magnan A, Meireles S, Moulis G, Puech C, Tcherakian C, Traclet J, Uzunhan Y, Cottin V, Groh M; CEREO, OrphaLung, and CRISALIS Networks
J Allergy Clin Immunol Pract 2023 Apr;11(4):1317-1319.e1. Epub 2022 Dec 26 doi: 10.1016/j.jaip.2022.12.022. PMID: 36581069
Giovannini-Chami L, Blanc S, Hadchouel A, Baruchel A, Boukari R, Dubus JC, Fayon M, Le Bourgeois M, Nathan N, Albertini M, Clément A, de Blic J
Pediatr Pulmonol 2016 Feb;51(2):203-16. Epub 2015 Dec 30 doi: 10.1002/ppul.23368. PMID: 26716396

Recent clinical studies

Etiology

Murillo AD, Castrillon AI, Serrano CD, Fernandez-Trujillo L
BMC Pulm Med 2024 Feb 8;24(1):74. doi: 10.1186/s12890-024-02868-3. PMID: 38331769Free PMC Article
Brenard E, Pilette C, Dahlqvist C, Colinet B, Schleich F, Roufosse F, Froidure A
Lung 2020 Apr;198(2):355-360. Epub 2020 Feb 12 doi: 10.1007/s00408-020-00336-3. PMID: 32052155
Giovannini-Chami L, Blanc S, Hadchouel A, Baruchel A, Boukari R, Dubus JC, Fayon M, Le Bourgeois M, Nathan N, Albertini M, Clément A, de Blic J
Pediatr Pulmonol 2016 Feb;51(2):203-16. Epub 2015 Dec 30 doi: 10.1002/ppul.23368. PMID: 26716396
Marchand E, Cordier JF
Orphanet J Rare Dis 2006 Apr 6;1:11. doi: 10.1186/1750-1172-1-11. PMID: 16722612Free PMC Article
Marchand E, Etienne-Mastroianni B, Chanez P, Lauque D, Leclerc P, Cordier JF; Groupe d'Etudes et de Recherche sur les Maladies Orphelines Pulmonaires
Eur Respir J 2003 Jul;22(1):8-13. doi: 10.1183/09031936.03.00085603. PMID: 12882444

Diagnosis

Cottin V
Immunol Allergy Clin North Am 2023 May;43(2):289-322. doi: 10.1016/j.iac.2023.01.002. PMID: 37055090
Giovannini-Chami L, Blanc S, Hadchouel A, Baruchel A, Boukari R, Dubus JC, Fayon M, Le Bourgeois M, Nathan N, Albertini M, Clément A, de Blic J
Pediatr Pulmonol 2016 Feb;51(2):203-16. Epub 2015 Dec 30 doi: 10.1002/ppul.23368. PMID: 26716396
Marchand E, Cordier JF
Orphanet J Rare Dis 2006 Apr 6;1:11. doi: 10.1186/1750-1172-1-11. PMID: 16722612Free PMC Article
Marchand E, Cordier JF
Semin Respir Crit Care Med 2006 Apr;27(2):134-41. doi: 10.1055/s-2006-939516. PMID: 16612764
Lombard CM, Tazelaar HD, Krasne DL
Chest 1987 May;91(5):734-6. doi: 10.1378/chest.91.5.734. PMID: 3568777

Therapy

Murillo AD, Castrillon AI, Serrano CD, Fernandez-Trujillo L
BMC Pulm Med 2024 Feb 8;24(1):74. doi: 10.1186/s12890-024-02868-3. PMID: 38331769Free PMC Article
Brenard E, Pilette C, Dahlqvist C, Colinet B, Schleich F, Roufosse F, Froidure A
Lung 2020 Apr;198(2):355-360. Epub 2020 Feb 12 doi: 10.1007/s00408-020-00336-3. PMID: 32052155
Giovannini-Chami L, Blanc S, Hadchouel A, Baruchel A, Boukari R, Dubus JC, Fayon M, Le Bourgeois M, Nathan N, Albertini M, Clément A, de Blic J
Pediatr Pulmonol 2016 Feb;51(2):203-16. Epub 2015 Dec 30 doi: 10.1002/ppul.23368. PMID: 26716396
Marchand E, Cordier JF
Semin Respir Crit Care Med 2006 Apr;27(2):134-41. doi: 10.1055/s-2006-939516. PMID: 16612764
Lombard CM, Tazelaar HD, Krasne DL
Chest 1987 May;91(5):734-6. doi: 10.1378/chest.91.5.734. PMID: 3568777

Prognosis

Giovannini-Chami L, Blanc S, Hadchouel A, Baruchel A, Boukari R, Dubus JC, Fayon M, Le Bourgeois M, Nathan N, Albertini M, Clément A, de Blic J
Pediatr Pulmonol 2016 Feb;51(2):203-16. Epub 2015 Dec 30 doi: 10.1002/ppul.23368. PMID: 26716396
Blanc S, Albertini M, Leroy S, Giovannini-Chami L
BMJ Case Rep 2013 Feb 15;2013 doi: 10.1136/bcr-2012-008238. PMID: 23417937Free PMC Article
Marchand E, Cordier JF
Orphanet J Rare Dis 2006 Apr 6;1:11. doi: 10.1186/1750-1172-1-11. PMID: 16722612Free PMC Article
Marchand E, Cordier JF
Semin Respir Crit Care Med 2006 Apr;27(2):134-41. doi: 10.1055/s-2006-939516. PMID: 16612764
Marchand E, Reynaud-Gaubert M, Lauque D, Durieu J, Tonnel AB, Cordier JF
Medicine (Baltimore) 1998 Sep;77(5):299-312. doi: 10.1097/00005792-199809000-00001. PMID: 9772920

Clinical prediction guides

Murillo AD, Castrillon AI, Serrano CD, Fernandez-Trujillo L
BMC Pulm Med 2024 Feb 8;24(1):74. doi: 10.1186/s12890-024-02868-3. PMID: 38331769Free PMC Article
Zaibi H, Fessi R, Ben Jemia E, Ouertani H, Ben Amar J, Aouina H
Tunis Med 2023 Mar 5;101(3):386-390. PMID: 38263918Free PMC Article
Brenard E, Pilette C, Dahlqvist C, Colinet B, Schleich F, Roufosse F, Froidure A
Lung 2020 Apr;198(2):355-360. Epub 2020 Feb 12 doi: 10.1007/s00408-020-00336-3. PMID: 32052155
Shinohara T, Tsuji S, Okano Y, Machida H, Hatakeyama N, Ogushi F
Intern Med 2018 Dec 15;57(24):3507-3514. Epub 2018 Aug 10 doi: 10.2169/internalmedicine.0841-18. PMID: 30101907Free PMC Article
Marchand E, Reynaud-Gaubert M, Lauque D, Durieu J, Tonnel AB, Cordier JF
Medicine (Baltimore) 1998 Sep;77(5):299-312. doi: 10.1097/00005792-199809000-00001. PMID: 9772920

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