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Collagenous sprue

MedGen UID:
137953
Concept ID:
C0341299
Disease or Syndrome
Synonyms: Collagenous Sprue; Collagenous Sprues; Sprue, Collagenous; Sprues, Collagenous
SNOMED CT: Non-gluten sensitive enteropathy syndrome (61738006); Non-gluten intolerance syndrome (61738006); Collagenous enteropathy syndrome (61738006); Collagenous sprue (61738006); Collagenous enteropathy (61738006)
 
Monarch Initiative: MONDO:0044092

Definition

A rare disorder affecting the digestive tract. Its cause is unclear but may be attributed, in part, to increased collagen synthesis without adequate fibrolysis. It is characterized histologically by atrophy of mucosal villi and crypts with extensive subepithelial collagen deposition. Clinical signs include nausea, vomiting, diarrhea and weight loss. Unlike celiac sprue (celiac disease), a gluten-free diet does not predict a certain regression of the disease. The clinical course follows a progression of malabsorption leading to nutritional deficiencies, small bowel ulceration/perforation, lymphoma and infection. Prognosis is usually dismal. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCollagenous sprue

Professional guidelines

PubMed

Chen ZE, Lee HE, Wu TT
Hum Pathol 2023 Feb;132:20-30. Epub 2022 Aug 4 doi: 10.1016/j.humpath.2022.07.017. PMID: 35932826
Brown I, Bettington M, Rosty C
Histopathology 2021 Jan;78(1):88-105. doi: 10.1111/his.14262. PMID: 33382496
Nielsen OH, Riis LB, Danese S, Bojesen RD, Soendergaard C
Ann Med 2014 Aug;46(5):311-7. Epub 2014 Apr 10 doi: 10.3109/07853890.2014.899102. PMID: 24716737

Recent clinical studies

Etiology

Choung RS, Sharma A, Chedid VG, Absah I, Chen ZE, Murray JA
Clin Gastroenterol Hepatol 2022 Sep;20(9):1977-1985.e1. Epub 2021 Dec 2 doi: 10.1016/j.cgh.2021.11.033. PMID: 34864160
Bayoumy AB, Simsek M, Seinen ML, Mulder CJJ, Ansari A, Peters GJ, De Boer NK
Expert Opin Drug Metab Toxicol 2020 Feb;16(2):111-123. Epub 2020 Feb 24 doi: 10.1080/17425255.2020.1719996. PMID: 32090622
Freeman HJ
World J Gastroenterol 2014 Aug 7;20(29):9990-7. doi: 10.3748/wjg.v20.i29.9990. PMID: 25110427Free PMC Article
Nielsen OH, Riis LB, Danese S, Bojesen RD, Soendergaard C
Ann Med 2014 Aug;46(5):311-7. Epub 2014 Apr 10 doi: 10.3109/07853890.2014.899102. PMID: 24716737
Freeman HJ
Can J Gastroenterol 2011 Apr;25(4):189-92. doi: 10.1155/2011/821976. PMID: 21523258Free PMC Article

Diagnosis

Smyrk TC
Surg Pathol Clin 2017 Dec;10(4):823-839. Epub 2017 Sep 23 doi: 10.1016/j.path.2017.07.004. PMID: 29103535
Freeman HJ
World J Gastroenterol 2014 Aug 7;20(29):9990-7. doi: 10.3748/wjg.v20.i29.9990. PMID: 25110427Free PMC Article
Nielsen OH, Riis LB, Danese S, Bojesen RD, Soendergaard C
Ann Med 2014 Aug;46(5):311-7. Epub 2014 Apr 10 doi: 10.3109/07853890.2014.899102. PMID: 24716737
Freeman HJ
Can J Gastroenterol 2011 Apr;25(4):189-92. doi: 10.1155/2011/821976. PMID: 21523258Free PMC Article
Br Med J 1971 Apr 10;2(5753):65-6. PMID: 5551269Free PMC Article

Therapy

Smyrk TC
Surg Pathol Clin 2017 Dec;10(4):823-839. Epub 2017 Sep 23 doi: 10.1016/j.path.2017.07.004. PMID: 29103535
Desruisseaux C, Bensoussan M, Désilets E, Tran HK, Arcand R, Poirier G, Wisniewski A, Manière T
Can J Gastroenterol Hepatol 2016;2016:4837270. Epub 2016 Apr 21 doi: 10.1155/2016/4837270. PMID: 27446843Free PMC Article
Nielsen OH, Riis LB, Danese S, Bojesen RD, Soendergaard C
Ann Med 2014 Aug;46(5):311-7. Epub 2014 Apr 10 doi: 10.3109/07853890.2014.899102. PMID: 24716737
Freeman HJ
Can J Gastroenterol 2011 Apr;25(4):189-92. doi: 10.1155/2011/821976. PMID: 21523258Free PMC Article
Freeman HJ
World J Gastroenterol 2010 Jan 21;16(3):296-8. doi: 10.3748/wjg.v16.i3.296. PMID: 20082473Free PMC Article

Prognosis

Freeman HJ
World J Gastroenterol 2014 Aug 7;20(29):9990-7. doi: 10.3748/wjg.v20.i29.9990. PMID: 25110427Free PMC Article
Nielsen OH, Riis LB, Danese S, Bojesen RD, Soendergaard C
Ann Med 2014 Aug;46(5):311-7. Epub 2014 Apr 10 doi: 10.3109/07853890.2014.899102. PMID: 24716737
Freeman HJ
Can J Gastroenterol 2011 Apr;25(4):189-92. doi: 10.1155/2011/821976. PMID: 21523258Free PMC Article
Freeman HJ
World J Gastroenterol 2008 Mar 21;14(11):1643-5. doi: 10.3748/wjg.14.1643. PMID: 18350593Free PMC Article
Colletti RB, Trainer TD
Gastroenterology 1989 Dec;97(6):1552-5. doi: 10.1016/0016-5085(89)90403-4. PMID: 2583419

Clinical prediction guides

Lan N, Shen B, Yuan L, Liu X
J Gastroenterol Hepatol 2017 Jan;32(1):120-127. doi: 10.1111/jgh.13592. PMID: 27620860
Desruisseaux C, Bensoussan M, Désilets E, Tran HK, Arcand R, Poirier G, Wisniewski A, Manière T
Can J Gastroenterol Hepatol 2016;2016:4837270. Epub 2016 Apr 21 doi: 10.1155/2016/4837270. PMID: 27446843Free PMC Article
Freeman HJ
World J Gastroenterol 2014 Aug 7;20(29):9990-7. doi: 10.3748/wjg.v20.i29.9990. PMID: 25110427Free PMC Article
Narla NP, Smyrk TC, Pardi DS, Tung J
J Pediatr Gastroenterol Nutr 2013 Nov;57(5):557-61. doi: 10.1097/MPG.0b013e3182a1df59. PMID: 23820404
O'Brien BH, McClymont K, Brown I
Am J Surg Pathol 2011 Aug;35(8):1151-7. doi: 10.1097/PAS.0b013e3182206ef5. PMID: 21716082

Recent systematic reviews

Nielsen OH, Riis LB, Danese S, Bojesen RD, Soendergaard C
Ann Med 2014 Aug;46(5):311-7. Epub 2014 Apr 10 doi: 10.3109/07853890.2014.899102. PMID: 24716737

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