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Hoffmann, R. A wiki for the life sciences where authorship matters. Nature Genetics (2008)
MeSH Review

CREST Syndrome

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Disease relevance of CREST Syndrome


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


Biological context of CREST Syndrome


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


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].


  1. The effect of diltiazem on calcinosis in a patient with the CREST syndrome. Farah, M.J., Palmieri, G.M., Sebes, J.I., Cremer, M.A., Massie, J.D., Pinals, R.S. Arthritis Rheum. (1990) [Pubmed]
  2. Myocardial function and perfusion in the CREST syndrome variant of progressive systemic sclerosis. Exercise radionuclide evaluation and comparison with diffuse scleroderma. Follansbee, W.P., Curtiss, E.I., Medsger, T.A., Owens, G.R., Steen, V.D., Rodnan, G.P. Am. J. Med. (1984) [Pubmed]
  3. 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. Alpert, M.A., Pressly, T.A., Mukerji, V., Lambert, C.R., Mukerji, B., Panayiotou, H., Sharp, G.C. Am. J. Cardiol. (1991) [Pubmed]
  4. The presence of dominant T-cell clones in peripheral blood of patients with collagen vascular disorders: a prospective study of 97 cases. Dereure, O., Gubler, B., Bessis, D., Guillot, B., Guilhou, J.J., Clot, J., Eliaou, J.F. Br. J. Dermatol. (2006) [Pubmed]
  5. Acute calcific periarthritis in scleroderma. Fam, A.G., Pritzker, K.P. J. Rheumatol. (1992) [Pubmed]
  6. Immunoglobulin M autoantibody to vimentin intermediate filaments. Senecal, J.L., Rothfield, N.F., Oliver, J.M. J. Clin. Invest. (1982) [Pubmed]
  7. Pulmonary hypertension in the CREST syndrome variant of systemic sclerosis. Stupi, A.M., Steen, V.D., Owens, G.R., Barnes, E.L., Rodnan, G.P., Medsger, T.A. Arthritis Rheum. (1986) [Pubmed]
  8. Speckled pattern antinuclear antibodies resembling anticentromere antibodies. Fritzler, M.J., Valencia, D.W., McCarty, G.A. Arthritis Rheum. (1984) [Pubmed]
  9. Nodular regenerative hyperplasia of the liver, CREST syndrome and primary biliary cirrhosis: an overlap syndrome? McMahon, R.F., Babbs, C., Warnes, T.W. Gut (1989) [Pubmed]
  10. Immunohistochemical expression of transforming growth factor beta3 in calcinosis in a patient with systemic sclerosis and CREST syndrome. Kawakami, T., Soma, Y., Mizoguchi, M., Saito, R. Br. J. Dermatol. (2000) [Pubmed]
  11. Studies of the microvascular endothelium in uninvolved skin of patients with systemic sclerosis: direct evidence for a generalized microangiopathy. Freemont, A.J., Hoyland, J., Fielding, P., Hodson, N., Jayson, M.I. Br. J. Dermatol. (1992) [Pubmed]
  12. Appearance of a gastric ulcer during diphosphonate therapy in a woman with CRST syndrome. Saunders, R.L. South. Med. J. (1977) [Pubmed]
  13. A case of CREST syndrome with rapidly progressive liver damage. Yabe, H., Noma, K., Tada, N., Mochizuki, S., Nagano, M. Intern. Med. (1992) [Pubmed]
  14. Centromeric structure identification in Entamoeba histolytica by anticentromeric/kinetochore antibodies obtained from patients with the CREST syndrome. López-Robles, M.C., Orozco, E., Beltrán-Castillo, J.A., Díaz, A., Hernández-Jáuregui, P., Gómez-Conde, E. Arch. Med. Res. (2000) [Pubmed]
  15. Stimulation of lymphocyte reactivity by a low molecular weight cutaneous antigen in patients with progressive systemic sclerosis (scleroderma). Kondo, H., Rabin, B.S., Rodnan, G.P. J. Rheumatol. (1979) [Pubmed]
  16. Antinuclear antibodies detected by indirect immunofluorescence on HEp2 cells and by immunoblotting in patients with systemic sclerosis. Briolay, J., Gioud, M., Monier, J.C., Rodde, J.L., Cohen, J., Roux, B., Kaliss, B. Autoimmunity (1989) [Pubmed]
  17. Protease nexin-1 messenger RNA levels are not affected by serum or interferon beta in cultured systemic sclerosis fibroblasts. de Oliveira, J.G., Guedes, A.C., Lanna, C.C., Coelho, L.F., Prados, R.Z., Feghali, C., Ferreira, P.C., Kroon, E.G. Arch. Dermatol. Res. (2002) [Pubmed]
  18. Clinical, serological and genetic study in patients with CREST syndrome. Akiyama, Y., Tanaka, M., Takeishi, M., Adachi, D., Mimori, A., Suzuki, T. Intern. Med. (2000) [Pubmed]
  19. Antibodies to fibrin bound tissue type plasminogen activator in systemic sclerosis. Fritzler, M.J., Hart, D.A., Wilson, D., García-De La Torre, I., Salazar-Páramo, M., Vázquez-Del Mercado, M., Senécal, J.L., Loyau, S., Anglés-Cano, E. J. Rheumatol. (1995) [Pubmed]
  20. The coexistence of rheumatoid arthritis and scleroderma: a case report and review of the literature. Baron, M., Srolovitz, H., Lander, P., Kapusta, M. J. Rheumatol. (1982) [Pubmed]
  21. Pulmonary function in progressive systemic sclerosis. Comparison of CREST syndrome variant with diffuse scleroderma. Owens, G.R., Fino, G.J., Herbert, D.L., Steen, V.D., Medsger, T.A., Pennock, B.E., Cottrell, J.J., Rodnan, G.P., Rogers, R.M. Chest (1983) [Pubmed]
  22. The diagnostic value of several immunological tests for anti-nuclear antibody in predicting the development of connective tissue disease in patients presenting with Raynaud's phenomenon. Wollersheim, H., Thien, T., Hoet, M.H., Van Venrooy, W.J. Eur. J. Clin. Invest. (1989) [Pubmed]
  23. Epidermal growth factor and gastrin in scleroderma/CREST syndrome. Jiménez-Balderas, F., Zonana-Nacach, A., Fonseca-Yerena, M.E., Ruíz-Chaparro, A., Pascoe-Lira, D. Arch. Med. Res. (1998) [Pubmed]
  24. Silicon and silicone: theoretical and clinical implications of breast implants. Yoshida, S.H., Chang, C.C., Teuber, S.S., Gershwin, M.E. Regulatory toxicology and pharmacology : RTP. (1993) [Pubmed]
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