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CREST Syndrome (Syndrome, CREST)

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A mild form of LIMITED SCLERODERMA, a multi-system disorder. Its features include symptoms of CALCINOSIS; RAYNAUD DISEASE; ESOPHAGEAL MOTILITY DISORDERS; sclerodactyly, and TELANGIECTASIS. When the defect in esophageal function is not prominent, it is known as CRST syndrome.

Also Known As:

Syndrome, CREST; Calcinosis, Raynaud's phenomenon, Esophageal dismobility, Sclerodactyly, Telangiectasia Syndrome; Calcinosis-Raynaud Phenomenon-Sclerodactyly-Telangiectasia; CREST Syndromes; CRST Syndromes; Calcinosis Raynaud Phenomenon Sclerodactyly Telangiectasia; Phenomenon-Sclerodactyly-Telangiectasia, Calcinosis-Raynaud; Syndrome, CRST; CRST Syndrome

Networked: 169 relevant articles (2 outcomes, 13 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Calcinosis
2.	Telangiectasis (Telangiectasia)
3.	Biliary Liver Cirrhosis (Primary Biliary Cirrhosis)
4.	Systemic Scleroderma (Systemic Sclerosis)
5.	Systemic Lupus Erythematosus (Libman-Sacks Disease)

Experts

1.	Paramothayan, N S: 2 articles (01/2003 - 01/2002)
2.	Lasserson, T J: 2 articles (01/2003 - 01/2002)
3.	Walters, E H: 2 articles (01/2003 - 01/2002)
4.	Wells, A U: 2 articles (01/2003 - 01/2002)
5.	Ikeda, S: 2 articles (07/2001 - 05/2001)
6.	Herrero, Laura: 1 article (01/2015)
7.	Bargalló, Xavier: 1 article (01/2015)
8.	Santamaría, Gorane: 1 article (01/2015)
9.	Fernández, Pedro Luis: 1 article (01/2015)
10.	Velasco, Martín: 1 article (01/2015)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to CREST Syndrome:

1.	Norethindrone (Norethisterone)FDA LinkGeneric 01/01/1994 - "[Vascular gastric anomalies, CREST syndrome and primary biliary cirrhosis: efficacy of ethinyl estradiol-norethisterone combination]."
2.	Ethinyl Estradiol (Estinyl)FDA LinkGeneric 01/01/1994 - "[Vascular gastric anomalies, CREST syndrome and primary biliary cirrhosis: efficacy of ethinyl estradiol-norethisterone combination]."
3.	AntibodiesIBA 09/01/1995 - "Our study demonstrates that 20% of unselected patients with SSc have anti-tPA antibodies and that there is a higher representation of patients with CREST syndrome in this subgroup. " 05/01/2009 - "Anti-centromere and anti-Th/To antibodies are often associated to the limited SSc and to the CREST syndrome. " 09/01/2003 - "Micronuclei derived from acentric fragments or from whole chromosomes were evaluated by immunofluorescent staining using anti-kinetochore antibodies from the serum of scleroderma (CREST syndrome) patients. " 11/01/2000 - "However, the association with anticentromere antibodies suggests a possible role in the subgroup of patients with CREST syndrome or scleroderma." 07/01/2000 - "Centromeric structure identification in Entamoeba histolytica by anticentromeric/kinetochore antibodies obtained from patients with the CREST syndrome."
4.	Antinuclear AntibodiesIBA 12/01/2013 - "So far this antinuclear antibody specificity has been mainly reported in patients with the CREST syndrome. " 10/01/1980 - "The serums of 27 patients with the CREST syndrome have been examined for the presence of antinuclear antibodies. " 07/13/1985 - "Using HEp2 cells to study antinuclear antibodies has resulted in the discovery of the anti-centromere antibody which is thought to separate the CREST syndrome from progressive systemic sclerosis (scleroderma). " 08/01/1989 - "Sixty eight per cent of the patients with diffuse scleroderma and 62% of those with the CREST syndrome had a positive antinuclear antibody test, which was associated with oesophageal hypomotility (p less than 0.001), fat malabsorption (p less than 0.01) and pulmonary dysfunction (p less than 0.05). " 06/01/1980 - "Antinuclear antibodies were demonstrated in the sera of 43 of 45 (96%) patients with progressive systemic sclerosis (22 of 24 with diffuse scleroderma and 21 of 21 with the CREST syndrome variant). "
5.	CalciumIBA 02/01/1992 - "We performed an electron-microscopic study on calcium deposits in two patients with calcifying scleroderma (CRST syndrome). " 04/01/1983 - "An ultrastructural and crystallographic study was done on calcium deposits obtained from a patient with the CREST syndrome. " 02/01/1992 - "The extrafibrillary calcium deposits on these mucopolysaccharide structures may represent an early event in the complex pathogenesis of calcification in the CRST syndrome." 06/01/2001 - "The calcium antagonist diltiazem reduces subcutaneous calcifications in CREST syndrome and in isolated cases of children with dermatomyositis. "
6.	AutoantibodiesIBA 07/01/1998 - "The development of the CREST syndrome in our patient was preceded by the appearance of anti-centromere autoantibodies. " 05/01/1989 - "The induction by diethylsulphate of micronuclei derived from acentric fragments or from whole chromosomes was studied in Chinese hamster V79 cells using autoantibodies from the serum of a scleroderma patient (CREST-syndrome) to detect centromere--kinetochore structures. " 05/01/2000 - "Kinetics of anti-fibrillin-1 autoantibodies in MCTD and CREST syndrome." 02/01/1994 - "An ELISA established with the eukaryotically expressed and purified recombinant human full-length CENP-B demonstrated its excellent specificity, sensitivity and reproducibility for the measurement of autoantibodies directed to the human CENP-B (ACA-B) representing a diagnostic marker for CREST syndrome, an autoimmune rheumatic disease. " 08/01/2010 - "Elucidating the true incidence of cerebral aneurysms associated with CREST syndrome would help to clarify the relationship between SSc-related autoantibodies and aneurysm formation."
7.	Diffuse systemic sclerosisIBA 10/01/1989 - "Of the five, four girls were classified as having diffuse systemic sclerosis and the remaining boy, as suffering from the CREST syndrome. " 10/01/1989 - "Five patients with childhood scleroderma, were studied from a total group of 50 cases with the disease, 39 of them with diffuse systemic sclerosis and 11 with the CREST syndrome. " 10/01/2014 - "Although it has been reported in diffuse systemic sclerosis, it has not been reported in calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly and telangiectasia (CREST) syndrome. " 06/01/1992 - "Various parameters for assessing endothelial cell (EC) metabolism, including immunohistochemistry and adenosine uptake, have been compared in the clinically uninvolved skin of patients with diffuse systemic sclerosis (DSS), CREST, incomplete CREST syndrome (ICREST), primary Raynaud's disease (1 degree RD) and normal controls. " 11/01/1992 - "To assess the pulmonary and systemic hemodynamic effects of oral captopril in patients with connective tissue disease and pulmonary hypertension, we performed right heart catheterization in eight patients with diffuse systemic sclerosis, the CREST syndrome, or mixed connective tissue diseases prior to and immediately following administration of captopril (dose range 12.5 to 50.0 mg, short-term study). "
8.	ChromatinIBA 03/01/1989 - "Antikinetochore antibodies from patients with the CREST syndrome of scleroderma were used as probes to study homogeneously staining regions (HSRs) in multidrug resistant (MDR) mouse tumor cells, and chromatin bodies (CBs) in MDR mouse-hamster hybrid cells. " 07/01/2007 - "We determined the significance of anti-nuclear antibodies (ANA), anti- double-stranded DNA (anti-dsDNA), anti-chromatin, and anti-C1q antibodies, as well as complement factors C3 and C4, in relation to disease activity in SLE patients with (n = 47; long-term follow-up data for 33 patients) and without (n = 31) biopsy-confirmed lupus nephritis, microscopic polyangiitis (n = 37), Wegener's granulomatosis (n = 66), primary Sjögren's syndrome (n = 17), limited scleroderma (CREST syndrome) (n = 6), and progressive systemic scleroderma (PSS) (n = 11). "
9.	Captopril (Capoten)FDA LinkGeneric 10/01/1984 - "The CREST syndrome--successful reduction of pulmonary hypertension by captopril." 11/01/1992 - "To assess the pulmonary and systemic hemodynamic effects of oral captopril in patients with connective tissue disease and pulmonary hypertension, we performed right heart catheterization in eight patients with diffuse systemic sclerosis, the CREST syndrome, or mixed connective tissue diseases prior to and immediately following administration of captopril (dose range 12.5 to 50.0 mg, short-term study). "
10.	GhrelinIBA 01/01/2010 - "In this study, we assessed the serum levels of leptin, adiponectin, and ghrelin, in association with the measurements of body composition, in 15 female patients with various autoimmune diseases (systemic lupus erythematosus, primary Sjögren's syndrome, sarcoidosis, mixed connective tissue disease, vasculitis, CREST syndrome, and polymyositis) and in 15 healthy female controls. "

Therapies and Procedures

1.	Gas Lasers 09/01/2003 - "Successful palliation and significant remission of cutaneous calcinosis in CREST syndrome with carbon dioxide laser." 09/01/2003 - "Carbon dioxide laser vaporization may offer effective remission of symptoms in cutaneous calcinosis of CREST syndrome (including cutaneous calcinosis, Raynaud's phenomenon, sclerodactyly, and telangiectasia)."
2.	Argon Plasma Coagulation 06/01/1997 - "[Argon plasma coagulation in endoscopic therapy of CREST syndrome associated upper gastrointestinal hemorrhage]."
3.	Catheters 02/01/1999 - "We describe a PD-associated peritonitis in a 51-year-old woman that was caused by Propionibacteria sp. This patient's risk factors for developing Propionibacteria sp peritonitis include a history of CREST syndrome, malignancy of the breast, and recent catheter surgery. "
4.	Dye Lasers 01/01/2014 - "Telangiectasis in CREST syndrome and systemic sclerosis: correlation of clinical and pathological features with response to pulsed dye laser treatment."
5.	Low-Level Laser Therapy (LLLT) 01/01/2014 - "Pulsed LLLT represents a treatment alternative for osteoarticular signs and symptoms in limited scleroderma (CREST syndrome)."