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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]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCREST syndrome
Follow this link to review classifications for CREST syndrome in Orphanet.

Professional guidelines

PubMed

Comparison of Screening Dilution and Automated Reading for Antinuclear Antibody Detection on HEP2 Cells in the Monitoring of Connective Tissue Diseases.
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
Telangiectasis in CREST syndrome and systemic sclerosis: correlation of clinical and pathological features with response to pulsed dye laser treatment.
Halachmi S, Gabari O, Cohen S, Koren R, Amitai DB, Lapidoth M
Lasers Med Sci 2014 Jan;29(1):137-40. Epub 2013 Mar 14 doi: 10.1007/s10103-013-1298-1. PMID: 23494102
Diagnosis and treatment of scleroderma.
Adnan ZA
Acta Med Indones 2008 Apr;40(2):109-12. PMID: 18560030

Recent clinical studies

Etiology

Risk factors for cancer in patients with primary biliary cholangitis and autoimmune hepatitis and primary biliary cholangitis overlap syndrome.
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
PACK syndrome: A case series and review.
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
Oral Health Experiences of the Limited Scleroderma Patient.
Beaty KL, Gurenlian JR, Rogo EJ
J Dent Hyg 2021 Aug;95(4):59-69. PMID: 34376545
Scleroderma and IgG4-related disease.
Reddi DM, Cardona DM, Burchette JL, Puri PK
Am J Dermatopathol 2013 Jun;35(4):458-62. doi: 10.1097/DAD.0b013e318276cbac. PMID: 23563250
Prostacyclin for pulmonary hypertension.
Paramothayan NS, Lasserson TJ, Wells AU, Walters EH
Cochrane Database Syst Rev 2002;(3):CD002994. doi: 10.1002/14651858.CD002994. PMID: 12137667

Diagnosis

PACK syndrome: A case series and review.
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
Friction Syndromes of the Knee.
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
PBC and related extrahepatic diseases.
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
Primary biliary cirrhosis.
Kumagi T, Heathcote EJ
Orphanet J Rare Dis 2008 Jan 23;3:1. doi: 10.1186/1750-1172-3-1. PMID: 18215315Free PMC Article
Systemic sclerosis.
Ng SC
Singapore Med J 1992 Apr;33(2):189-92. PMID: 1621127

Therapy

Risk factors for cancer in patients with primary biliary cholangitis and autoimmune hepatitis and primary biliary cholangitis overlap syndrome.
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
Evidence for the Use of Epoprostenol to Treat Raynaud's Phenomenon With or Without Digital Ulcers.
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
Primary biliary cirrhosis.
Kumagi T, Heathcote EJ
Orphanet J Rare Dis 2008 Jan 23;3:1. doi: 10.1186/1750-1172-3-1. PMID: 18215315Free PMC Article
Prostacyclin for pulmonary hypertension.
Paramothayan NS, Lasserson TJ, Wells AU, Walters EH
Cochrane Database Syst Rev 2003;(2):CD002994. doi: 10.1002/14651858.CD002994. PMID: 12804447
Systemic sclerosis.
Ng SC
Singapore Med J 1992 Apr;33(2):189-92. PMID: 1621127

Prognosis

Primary biliary cirrhosis.
Kumagi T, Heathcote EJ
Orphanet J Rare Dis 2008 Jan 23;3:1. doi: 10.1186/1750-1172-3-1. PMID: 18215315Free PMC Article
Prostacyclin for pulmonary hypertension.
Paramothayan NS, Lasserson TJ, Wells AU, Walters EH
Cochrane Database Syst Rev 2003;(2):CD002994. doi: 10.1002/14651858.CD002994. PMID: 12804447
Prostacyclin for pulmonary hypertension.
Paramothayan NS, Lasserson TJ, Wells AU, Walters EH
Cochrane Database Syst Rev 2002;(3):CD002994. doi: 10.1002/14651858.CD002994. PMID: 12137667
Systemic sclerosis.
Ng SC
Singapore Med J 1992 Apr;33(2):189-92. PMID: 1621127
Microvasculature in systemic sclerosis.
Carpentier PH, Maricq HR
Rheum Dis Clin North Am 1990 Feb;16(1):75-91. PMID: 2406812

Clinical prediction guides

PACK syndrome: A case series and review.
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
Oral Health Experiences of the Limited Scleroderma Patient.
Beaty KL, Gurenlian JR, Rogo EJ
J Dent Hyg 2021 Aug;95(4):59-69. PMID: 34376545
Prostacyclin for pulmonary hypertension.
Paramothayan NS, Lasserson TJ, Wells AU, Walters EH
Cochrane Database Syst Rev 2003;(2):CD002994. doi: 10.1002/14651858.CD002994. PMID: 12804447
Silicon and silicone: theoretical and clinical implications of breast implants.
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
Microvasculature in systemic sclerosis.
Carpentier PH, Maricq HR
Rheum Dis Clin North Am 1990 Feb;16(1):75-91. PMID: 2406812

Recent systematic reviews

Prostacyclin for pulmonary hypertension.
Paramothayan NS, Lasserson TJ, Wells AU, Walters EH
Cochrane Database Syst Rev 2003;(2):CD002994. doi: 10.1002/14651858.CD002994. PMID: 12804447
Prostacyclin for pulmonary hypertension.
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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