Disease relevance of CREST Syndrome

• The effect of diltiazem on calcinosis in a patient with the CREST syndrome [1].
• The prevalence of thallium perfusion abnormalities was similar in the groups with CREST syndrome and diffuse scleroderma, (64 percent versus 77 percent), but the defects were significantly smaller in the CREST syndrome (p less than 0.01) [2].
• Acute and long-term effects of nifedipine on pulmonary and systemic hemodynamics in patients with pulmonary hypertension associated with diffuse systemic sclerosis, the CREST syndrome and mixed connective tissue disease [3].
• More precisely, such a dominant clone was present in 43% and 37% of cutaneous and systemic lupus erythematosus, respectively, in 75% of multiple morphea, 75% of regional scleroderma and 60% of CREST syndrome patients [4].
• Three patients with limited cutaneous scleroderma (CREST syndrome) and acute calcific periarthritis are presented. "Chalky" bursal effusion in one patient demonstrated masses of calcium hydroxyapatite crystals [5].

High impact information on CREST Syndrome

• Serum from a patient with the CREST Syndrome and systemic lupus erythematosus contained an IgM antibody that reacted at dilutions up to 1:800 with a fibrous cytoplasmic network in several epithelioid and fibroblastic cell lines [6].
• In comparison with 287 CREST syndrome patients without PHT, these 20 patients had markedly reduced diffusing capacity for carbon monoxide (DLCO) (mean 39% of predicted normal) [7].
• Two distinct nuclear antigens, designated NSpI and NSpII, have been characterized and differentiated from the centromeric antigen that reacts with sera from patients with the CREST syndrome [8].
• We present a case of NRHL with CREST syndrome and serological and biochemical features of PBC, a newly recognised overlap syndrome [9].
• Immunohistochemical expression of transforming growth factor beta3 in calcinosis in a patient with systemic sclerosis and CREST syndrome [10].

Chemical compound and disease context of CREST Syndrome

• 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].
• Appearance of a gastric ulcer during diphosphonate therapy in a woman with CRST syndrome [12].
• A patient with CREST syndrome (calcinosis cutis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) who had severe jaundice (total bilirubin 29.1 mg/dl) and rapidly progressive liver damage is reported [13].

Biological context of CREST Syndrome

• Centromeric structure identification in Entamoeba histolytica by anticentromeric/kinetochore antibodies obtained from patients with the CREST syndrome [14].

Anatomical context of CREST Syndrome

• Lymphocytes from patients with the CREST syndrome, rheumatoid arthritis, and from normal controls did not respond to the antigen [15].
• With nuclear extracts of HeLa cells, the sera from 75.8% of CREST syndrome subjects stained 18 and 22 kD proteins [16].
• The aim of the present study was to analyze the effect of serum and human recombinant beta interferon (rIFNbeta) treatment on PN-1 mRNA levels in cultured dermal fibroblasts obtained from the skin of healthy donors and from lesional skin of systemic sclerosis (SSc) patients with the limited (CREST syndrome) or the diffuse form of SSc [17].
• CONCLUSION: It was suggested that there was a genetic difference between CREST syndrome alone and CREST-PBC overlap syndrome and there were differences (the positivity of AMA and the severity of bile duct lesion) between PBC and CREST-PBC overlap syndrome [18].

Gene context of CREST Syndrome

• 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 [19].
• We describe a patient with all the features of the CREST syndrome variant of scleroderma who developed a destructive arthropathy typical of rheumatoid arthritis (RA) [20].
• Pulmonary function in progressive systemic sclerosis. Comparison of CREST syndrome variant with diffuse scleroderma [21].
• ANA-testing by immunoblotting was of special help in predicting the development of scleroderma, the CREST syndrome and mixed connective tissue disease [22].
• Epidermal growth factor and gastrin in scleroderma/CREST syndrome [23].

Analytical, diagnostic and therapeutic context of CREST Syndrome

• In the past 10 years, there have been multiple published reports associating silicone breast implants with scleroderma, morphea, SLE, rheumatoid arthritis, CREST syndrome and "human adjuvant disease." The alleged offending material, silicone, is a synthetic polymer containing a silicon-oxygen backbone [24].
• METHODS: EGF and GA levels were measured by radioimmunoassay in saliva, serum and urine in 10 patients (51 years, median; range, 35-66 years); 9 females and 1 male with Scl, 3 females with CREST syndrome, and 18 age- and sex-matched controls, 17 females and 1 male free of any systemic inflammatory disease [23].

References