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CREST syndrome

MedGen UID:
60083
Concept ID:
C0206138
Disease or Syndrome
Synonyms: Calcinosis, Raynaud's phenomenon, Esophageal dismobility, Sclerodactyly, Telangiectasia syndrome; Calcinosis-Raynaud phenomenon-sclerodactyly-telangiectasia
SNOMED CT: CREST - Calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, telangiectasia (31848007); CREST - Calcinosis, Raynaud's phenomenon, sclerodactyly, esophageal involvement, telangiectasia syndrome (31848007); Calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia (CREST) syndrome (31848007); Calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia (CREST) syndrome (31848007); Calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia syndrome (31848007); CREST syndrome (31848007); Calcinosis cutis, Raynaud's, esophageal dysfunction, sclerodactyly AND telangiectasia (31848007)
 
Monarch Initiative: MONDO:0019563
OMIM®: 181750
Orphanet: ORPHA90290

Definition

A variant of systemic sclerosis characterized by calcinosis, Raynaud phenomenon, esophageal motility disorders, sclerodactyly, and telangiectasia. [from NCI]

Professional guidelines

PubMed

Depincé-Berger AE, Moreau A, Bossy V, Genin C, Rinaudo M, Paul S
J Clin Lab Anal 2016 Sep;30(5):471-8. Epub 2016 May 26 doi: 10.1002/jcla.21881. PMID: 27225012Free PMC Article
Adnan ZA
Acta Med Indones 2008 Apr;40(2):109-12. PMID: 18560030
Chaffee NR
J Prosthodont 1998 Sep;7(3):155-60. doi: 10.1111/j.1532-849x.1998.tb00198.x. PMID: 9807098

Recent clinical studies

Etiology

Braga MH, Cançado GGL, Bittencourt PL, Couto CA, Guedes LV, Lima AMC, Ferraz MLG, Villela-Nogueira CA, Nardelli MJ, Faria LC, Gomes NMF, Oliveira EMG, Rotman V, Oliveira MB, da Cunha SMCF, Cunha-Silva M, Mendes LSC, Ivantes CAP, Codes L, de Almeida E Borges VF, Pace FHL, Pessoa MG, Signorelli IV, Coral GP, Filho JG, Chagas AL, Terrabuio DRB, Cançado ELR; Members of the Brazilian Cholestasis Study Group Consortium
Ann Hepatol 2023 Jul-Aug;28(4):101105. Epub 2023 Apr 23 doi: 10.1016/j.aohep.2023.101105. PMID: 37088418
Collins B, Dillon D, Silver RM
Am J Med Sci 2023 Apr;365(4):321-328. Epub 2022 Dec 16 doi: 10.1016/j.amjms.2022.12.005. PMID: 36535538
Merlino G, Germano S, Carlucci S
Aesthetic Plast Surg 2013 Dec;37(6):1214-9. Epub 2013 Oct 19 doi: 10.1007/s00266-013-0224-z. PMID: 24142114
Reddi DM, Cardona DM, Burchette JL, Puri PK
Am J Dermatopathol 2013 Jun;35(4):458-62. doi: 10.1097/DAD.0b013e318276cbac. PMID: 23563250
Lauritano D, Bussolati A, Baldoni M, Leonida A
Minerva Stomatol 2011 Sep;60(9):443-65. PMID: 21956352

Diagnosis

Collins B, Dillon D, Silver RM
Am J Med Sci 2023 Apr;365(4):321-328. Epub 2022 Dec 16 doi: 10.1016/j.amjms.2022.12.005. PMID: 36535538
Pharis H, Kong A, Robbins M, Waranch C, Wissman R
J Knee Surg 2022 Apr;35(5):491-497. Epub 2022 Feb 21 doi: 10.1055/s-0042-1743222. PMID: 35189665
Floreani A, Cazzagon N
Best Pract Res Clin Gastroenterol 2018 Jun-Aug;34-35:49-54. Epub 2018 May 23 doi: 10.1016/j.bpg.2018.05.013. PMID: 30343710
Kumagi T, Heathcote EJ
Orphanet J Rare Dis 2008 Jan 23;3:1. doi: 10.1186/1750-1172-3-1. PMID: 18215315Free PMC Article
Ng SC
Singapore Med J 1992 Apr;33(2):189-92. PMID: 1621127

Therapy

Braga MH, Cançado GGL, Bittencourt PL, Couto CA, Guedes LV, Lima AMC, Ferraz MLG, Villela-Nogueira CA, Nardelli MJ, Faria LC, Gomes NMF, Oliveira EMG, Rotman V, Oliveira MB, da Cunha SMCF, Cunha-Silva M, Mendes LSC, Ivantes CAP, Codes L, de Almeida E Borges VF, Pace FHL, Pessoa MG, Signorelli IV, Coral GP, Filho JG, Chagas AL, Terrabuio DRB, Cançado ELR; Members of the Brazilian Cholestasis Study Group Consortium
Ann Hepatol 2023 Jul-Aug;28(4):101105. Epub 2023 Apr 23 doi: 10.1016/j.aohep.2023.101105. PMID: 37088418
Cruz JE, Ward A, Anthony S, Chang S, Bae HB, Hermes-DeSantis ER
Ann Pharmacother 2016 Dec;50(12):1060-1067. Epub 2016 Jul 26 doi: 10.1177/1060028016660324. PMID: 27465880
Lauritano D, Bussolati A, Baldoni M, Leonida A
Minerva Stomatol 2011 Sep;60(9):443-65. PMID: 21956352
Kumagi T, Heathcote EJ
Orphanet J Rare Dis 2008 Jan 23;3:1. doi: 10.1186/1750-1172-3-1. PMID: 18215315Free PMC Article
Ng SC
Singapore Med J 1992 Apr;33(2):189-92. PMID: 1621127

Prognosis

Kumagi T, Heathcote EJ
Orphanet J Rare Dis 2008 Jan 23;3:1. doi: 10.1186/1750-1172-3-1. PMID: 18215315Free PMC Article
Leuschner U
Clin Liver Dis 2003 Nov;7(4):741-58. doi: 10.1016/s1089-3261(03)00101-6. PMID: 14594129
Steen VD
Semin Cutan Med Surg 1998 Mar;17(1):48-54. doi: 10.1016/s1085-5629(98)80062-x. PMID: 9512107
Ng SC
Singapore Med J 1992 Apr;33(2):189-92. PMID: 1621127
Carpentier PH, Maricq HR
Rheum Dis Clin North Am 1990 Feb;16(1):75-91. PMID: 2406812

Clinical prediction guides

Collins B, Dillon D, Silver RM
Am J Med Sci 2023 Apr;365(4):321-328. Epub 2022 Dec 16 doi: 10.1016/j.amjms.2022.12.005. PMID: 36535538
Freitas R, Martins P, Dourado E, Santiago T, Guimarães F, Fernandes BM, Garcia S, Samões B, Pinto AS, Gonçalves N, Lourenço MH, Costa E, Rocha M, Couto M, Duarte AC, Araújo F, Cordeiro I, Godinho F, Resende C, Salvador MJ, Cordeiro A, Santos MJ
ARP Rheumatol 2022 Jan-Mar;1(1):21-29. PMID: 35633574
Lauritano D, Bussolati A, Baldoni M, Leonida A
Minerva Stomatol 2011 Sep;60(9):443-65. PMID: 21956352
Yoshida SH, Chang CC, Teuber SS, Gershwin ME
Regul Toxicol Pharmacol 1993 Feb;17(1):3-18. doi: 10.1006/rtph.1993.1002. PMID: 8441826
Carpentier PH, Maricq HR
Rheum Dis Clin North Am 1990 Feb;16(1):75-91. PMID: 2406812

Recent systematic reviews

Paramothayan NS, Lasserson TJ, Wells AU, Walters EH
Cochrane Database Syst Rev 2003;(2):CD002994. doi: 10.1002/14651858.CD002994. PMID: 12804447
Paramothayan NS, Lasserson TJ, Wells AU, Walters EH
Cochrane Database Syst Rev 2002;(3):CD002994. doi: 10.1002/14651858.CD002994. PMID: 12137667

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