crest syndrome


Summary: 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.

Top Publications

  1. Slobodin G, Rosner I, Rozenbaum M, Naschitz J, Zuckerman E, Yeshurun D. Systemic sclerosis sine scleroderma: is it always the same disease? Report of three patients and discussion. Rheumatol Int. 2002;22:170-2 pubmed
    ..By reporting three new cases and analyzing previously published data, we discuss possible subsets and variants of the disease form. ..
  2. Stahnke G, Meier E, Scanarini M, Northemann W. Eukaryotic expression of recombinant human centromere autoantigen and its use in a novel ELISA for diagnosis of CREST syndrome. J Autoimmun. 1994;7:107-18 pubmed
    ..the measurement of autoantibodies directed to the human CENP-B (ACA-B) representing a diagnostic marker for CREST syndrome, an autoimmune rheumatic disease...
  3. Lauritano D, Bussolati A, Baldoni M, Leonida A. Scleroderma and CREST syndrome: a case report in dentistry. Minerva Stomatol. 2011;60:443-65 pubmed
    b>CREST syndrome is part of the heterogeneous scleroderma group of autoimmune diseases that cause thickening, hardening and tightening of the connective tissue in different parts of the body, and it may lead to complex disorders...
  4. De Andres J, Garcia Delpech S, Pérez V, Diaz Llopis M, Udaondo P, Sánchez M, et al. Bilateral infusion pump implants as therapy for refractory corneal ulcers in a patient with CREST syndrome: an interdisciplinary approach. Arch Ophthalmol. 2008;126:964-7 pubmed publisher
    ..We report a case of severe eye pain due to xerophthalmia in a patient with CREST (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) syndrome who was treated with an implanted pump reservoir. ..
  5. Meyer O. [CREST syndrome]. Ann Med Interne (Paris). 2002;153:183-8 pubmed
    b>CREST syndrome has been described as a form of progressive systemic sclerosis in which there is relatively limited involvement of the skin, prominence of calcinosis, Raynaud's phenomenon, esophageal dysfunction and telangiectasia...
  6. Huerva V, Sanchez M. Juxtafoveolar telangiectasis associated with CREST syndrome. Ocul Immunol Inflamm. 2008;16:195-7 pubmed publisher
    To report a case of CREST syndrome associated with juxtafoveolar telangiectasias (JT). Case report. Observational case report. A 64-year-old woman affected with CREST syndrome developed bilateral visual loss...
  7. Bronze da Rocha E, Novoa A, Teixeira N, Vasconcelos C, Cerveira C, Castro e Melo J, et al. Evaluation of the reactivity of sera from patients with systemic lupus erythematosus against the human MCP1. J Clin Immunol. 2012;32:721-8 pubmed publisher
    ..The widely reported importance of phosphorylated epitopes as targets for autoantibodies in SLE could also be confirmed for native-MCP1. ..
  8. Herrera Esparza R, Aguilar J, Saucedo A, Gonzalez I, López Robles E, Avalos Diaz E. Scleroderma with type III glomerulonephritis and MPO-ANCA antibodies in the serum. J Eur Acad Dermatol Venereol. 2005;19:617-20 pubmed
    ..Glomerulonephritis is a rare association of CREST. We are reporting a patient with CREST who developed glomerulonephritis and had anticentromere and antineutrophil cytoplasmic autoantibodies (ANCA) in her serum. ..
  9. Launay D, Hachulla E, Hatron P, Goullard L, Onimus T, Robin S, et al. Aerosolized iloprost in CREST syndrome related pulmonary hypertension. J Rheumatol. 2001;28:2252-6 pubmed
    To assess the outcome of patients with CREST syndrome associated severe pulmonary hypertension treated by aerosolized iloprost in a noncomparative study...

More Information


  1. Madani G, Katz R, Haddock J, Denton C, Bell J. The role of radiology in the management of systemic sclerosis. Clin Radiol. 2008;63:959-67 pubmed publisher
    ..Neurological involvement is increasingly recognized and the major role of radiology is the exclusion of coexistent pathology. We present a thorough review of the role of radiology in the management of systemic sclerosis. ..
  2. Tzeng D, Leslie K, Shelton D, Chan A. Unusual dyspnea in a woman with CREST syndrome. Chest. 2008;133:286-90 pubmed publisher
  3. McColl G, Buchanan R. Familial CREST syndrome. J Rheumatol. 1994;21:754-6 pubmed
    ..We conclude that familial occurrence of scleroderma may be associated with shared class II MHC antigens. ..
  4. Diaz L, Usatine R. Elbow nodules. J Fam Pract. 2010;59:35-9 pubmed
  5. Kutty K, Ledge G, Mahesh A, Ramesh S, Rajendran C. Childhood systemic sclerosis with calcified foci over scalp. J Assoc Physicians India. 2004;52:215 pubmed
  6. Echeverri A, Ospina F, Cañas C, Agualimpia A, Suso J, Tobon G, et al. Worsening of calcinosis cutis with teriparatide treatment in two osteoporotic patients. Br J Dermatol. 2016;175:1049-1051 pubmed publisher
    We present two cases of patients with systemic autoimmune diseases (one with dermatomyositis and one with CREST syndrome) who presented with a worsening of calcinosis cutis after treatment of osteoporosis with teriparatide...
  7. Ngo Mandag N, Van Gossum M, Rickaert F, Golstein M. [Autoimmune hepatitis and CREST syndrome]. Rev Med Brux. 2007;28:528-31 pubmed
    ..The patient had an history of CREST syndrome. The particularity of this case report is the rare association between these two autoimmune diseases.
  8. Stillwell E, Zhou J, Joshi H. Human ninein is a centrosomal autoantigen recognized by CREST patient sera and plays a regulatory role in microtubule nucleation. Cell Cycle. 2004;3:923-30 pubmed
    ..In these cells, nucleation of microtubules from the centrosome is also inhibited. These results thus suggest a regulatory role for ninein in microtubule nucleation. ..
  9. Darwish T. Medical image. Genital lupus. N Z Med J. 2008;121:86-7 pubmed
  10. de Oliveira J, Guedes A, Lanna C, Coelho L, Prados R, Feghali C, et al. Protease nexin-1 messenger RNA levels are not affected by serum or interferon beta in cultured systemic sclerosis fibroblasts. Arch Dermatol Res. 2002;293:584-9 pubmed
    ..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...
  11. Kasifoglu T, Korkmaz C, Yaşar S, Gulbas Z. Scleroderma and chronic myeloid leukemia: a sheer coincidence, a consequence of long lasting D-penicillamine therapy or a plausible relationship of both diseases?. Rheumatol Int. 2006;27:175-7 pubmed
    ..Ours is the second case with CML developing after the onset of CREST in the literature. We also briefly discuss the possible tendency to hematological malignancy in systemic sclerosis. ..
  12. Thomas Golbanov C, Wilke W, Fessler B, Hoffman G. Open label trial of tamoxifen in scleroderma. Clin Exp Rheumatol. 2003;21:99-102 pubmed
    ..Both patients subsequently relapsed, in one case 12 months, and in the other 24 months after treatment. Based on these results, we would not recommend tamoxifen for further large scale studies in scleroderma. ..
  13. Tham S, Grossman J. Limited microsurgical arteriolysis for complications of digital vasospasm. J Hand Surg Br. 1997;22:359-61 pubmed
    ..Following surgical microarteriolysis all digital ulcers healed completely. Severe digital ischaemic pain was significantly improved in all digits and completely resolved in 19 of 22 digits. ..
  14. Hamamoto K, Ohno T, Ogawa H. [Myelodysplastic syndrome with CREST syndrome successfully treated with metenolone--A case report]. Rinsho Ketsueki. 1996;37:362-5 pubmed
    A 54-year-old woman was diagnosed as having refractory anemia (RA) with CREST syndrome (incomplete type). She showed Raynaud's phenomenon, sclerodactyly and telangiectasia, but not calcinosis and esophageal dysmotility...
  15. Sabba C, Gasbarrini G. [Gastrointestinal angiodysplasias: Is Rendu-Osler-Weber disease or hereditary hemorrhagic telangiectasis the tip of an iceberg?]. Ann Ital Med Int. 2004;19:81-3 pubmed
  16. Ahmadi Simab K, Gross W. [Pulmonary arterial hypertension in collagenoses: clinical features, epidemiology, pathogenesis, diagnosis and treatment]. Z Rheumatol. 2006;65:297-300, 302-5 pubmed
    ..Since few months, also the phosphodiesterase inhibitor sildenafil (Revatio) is available. The combination of drugs with different mode of action will likely further improve the prognosis of PAH patients. ..
  17. van Paassen P, Damoiseaux J, Tervaert J. Laboratory assessment in musculoskeletal disorders. Best Pract Res Clin Rheumatol. 2003;17:475-94 pubmed
    ..Our main goal was to address the role of the determination of autoantibodies in the diagnosis and follow-up of musculoskeletal disorders. ..
  18. Daoussis D, Antonopoulos I, Liossis S, Yiannopoulos G, Andonopoulos A. Treatment of systemic sclerosis-associated calcinosis: a case report of rituximab-induced regression of CREST-related calcinosis and review of the literature. Semin Arthritis Rheum. 2012;41:822-9 pubmed publisher
    ..Rituximab appears as a promising treatment especially in view of recent evidence that this therapy may be also effective in the underlying disease. ..
  19. Akman A, Akova Y, Yucel E, Aydin P. Granulomatous anterior uveitis in a patient with CREST syndrome. Ocul Immunol Inflamm. 2000;8:201-3 pubmed
    The CREST syndrome is a variant form of progressive systemic sclerosis. Apart from the occurrence of keratoconjunctivitis sicca, other types of ocular involvement associated with this variant are quite rare...
  20. Berends J, Dompeling E, van der Star J, Hoorntje J. Pulmonary hypertension with limited cutaneous scleroderma (CREST syndrome). Neth J Med. 2000;57:229-32 pubmed
    ..a typical manifestation of pulmonary hypertension associated with limited cutaneous scleroderma, also known as CREST syndrome. The patient was treated with a calcium antagonist, oral anticoagulation and, because of evidence for ..
  21. Van Hooser A, Mancini M, Allis C, Sullivan K, Brinkley B. The mammalian centromere: structural domains and the attenuation of chromatin modeling. FASEB J. 1999;13 Suppl 2:S216-20 pubmed
    ..This specialized centromere subdomain would be predicted to have a very tight and static nucleosome structure as a result of the absence of H3 phosphorylation and acetylation. ..
  22. Li Q, Ke Y, Kapp J, Fertig N, Medsger T, Joshi H. A novel cell-cycle-dependent 350-kDa nuclear protein: C-terminal domain sufficient for nuclear localization. Biochem Biophys Res Commun. 1995;212:220-8 pubmed
    ..Molecular cloning and transfection studies reveal that the 350-kDa AH protein contains a coiled-coil and a globular domain at the C-terminus that is sufficient for nuclear localization. ..
  23. Pakunpanya K, Verasertniyom O, Vanichapuntu M, Pisitkun P, Totemchokchyakarn K, Nantiruj K, et al. Incidence and clinical correlation of anticentromere antibody in Thai patients. Clin Rheumatol. 2006;25:325-8 pubmed
    ..The presence of ACA detected in patients with vague musculoskeletal symptoms does not suggest a diagnosis of CREST syndrome. Even high-titer ACA can be found in nonautoimmune diseases.
  24. Amathieu R, Tual L, Rouaghe S, Stirnemann J, Fain O, Dhonneur G. [Splenic rupture associated with CMV infection: case report and review]. Ann Fr Anesth Reanim. 2007;26:674-6 pubmed
    ..We report the case of a 60-yr-woman with spontaneous splenic rupture during CMV primary infection. Non-operative treatment failed and splenectomy was done. ..
  25. Gómez V, Sesé M, Santamaria A, Martinez J, Castellanos E, Soler M, et al. Regulation of aurora B kinase by the lipid raft protein flotillin-1. J Biol Chem. 2010;285:20683-90 pubmed publisher
    ..These observations are the first evidence for a function of Flotillin-1 outside of lipid rafts and suggest its critical role in the maintenance of a pool of active Aurora B. ..
  26. Wiesent F, Weinerth J. [Digital ulcers in systemic sclerosis--an interdisciplinary challenge]. Med Klin (Munich). 2010;105:578-81 pubmed publisher
    ..The described case illustrates the essential interdisciplinary collaboration for optimal management of these patients. ..
  27. Assassi S, Wu M, Tan F, Chang J, Graham T, Furst D, et al. Skin gene expression correlates of severity of interstitial lung disease in systemic sclerosis. Arthritis Rheum. 2013;65:2917-27 pubmed publisher
    ..A limited number of skin transcripts including genes involved in extravasation and adhesion of inflammatory cells correlate with severity of ILD. ..
  28. Carneiro A, Barbosa I, Chaves F. [CREST syndrome and pulmonary hypertension: a dark prognosis]. Acta Med Port. 2004;17:409-14 pubmed
    The CREST syndrome initially described as a limited, more indolent form of diffuse scleroderma, is characterized by calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasias...
  29. Nelson M, Heffernan M. Synopsis of antinuclear antibodies in dermatology. Dermatol Nurs. 2002;14:165-9, 203 pubmed
    ..As skin manifestations are common with connective tissue diseases, knowledge of the diagnostic and prognostic value of ANA testing is essential to the nurse in dermatology. ..
  30. Imafuku Y. [Immunologic tests: Anticentromere antibody]. Nihon Rinsho. 2005;63 Suppl 7:460-3 pubmed
  31. Teng A, Robbin M, Furey C, Easley S, Abdul Karim F, Bohlman H. Tumoral calcinosis in the cervical spine in a patient with CREST syndrome. A case report. J Bone Joint Surg Am. 2006;88:193-7 pubmed
  32. Nakamura T, Higashi S, Tomoda K, Tsukano M, Sugi K. Primary biliary cirrhosis (PBC)-CREST overlap syndrome with coexistence of Sjögren's syndrome and thyroid dysfunction. Clin Rheumatol. 2007;26:596-600 pubmed
    ..Clinically, they were diagnosed as both asymptomatic PBC and incomplete CREST syndrome. Their human leukocyte antigen typing showed both DR4 and DR8 positive...
  33. Pramyothin P, Kim D, Young L, Wichansawakun S, Apovian C. Anemia and leukopenia in a long-term parenteral nutrition patient during a shortage of parenteral trace element products in the United States. JPEN J Parenter Enteral Nutr. 2013;37:425-9 pubmed publisher
  34. Malandrini A, Selvi E, Villanova M, Berti G, Sabadini L, Salvadori C, et al. Autonomic nervous system and smooth muscle cell involvement in systemic sclerosis: ultrastructural study of 3 cases. J Rheumatol. 2000;27:1203-6 pubmed
    ..Our findings indicate early involvement of the autonomic nervous system and to a lesser extent of smooth muscle cells. We confirmed the presence of early vascular lesions and involvement of mast cells in the pathological process. ..
  35. Russman A, Lederman R, Calabrese L, Embi P, Forghani B, Gilden D. Multifocal varicella-zoster virus vasculopathy without rash. Arch Neurol. 2003;60:1607-9 pubmed
    A 51-year-old woman with CREST syndrome (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) developed stepwise progressive focal neurological deficits without zoster rash...
  36. López Meseguer M, Roman A, Monforte V, Bravo C, Sole J, Morell F. [Double-lung transplantation in 15 patients with pulmonary hypertension]. Arch Bronconeumol. 2009;45:366-70 pubmed publisher
    ..Double-lung transplantation is a therapeutic option that, in certain cases, has similar outcomes to those achieved with the most aggressive medical treatment for pulmonary hypertension. ..
  37. Johnson P, Neperud J, Arnold J, Thomas J. Livedo reticularis and bowel ischemia after carbon dioxide arteriography in a patient with CREST syndrome. J Vasc Interv Radiol. 2011;22:395-9 pubmed publisher
    ..which diffuse livedo reticularis, bowel ischemia, and renal insufficiency developed following CO(2) angiography in a patient with CREST syndrome (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia).
  38. Lee S, Yun S, Lee J, Lee S, Won Y. A case of porphyria cutanea tarda in association with idiopathic myelofibrosis and CREST syndrome. Br J Dermatol. 2001;144:182-5 pubmed
    ..Sclerodermoid changes were also found on both hands, the face and neck. The patient had suffered from CREST syndrome, manifesting with Raynaud's phenomenon and sclerodactyly, for more than 15 years...
  39. Lachter J, Suissa A, Schiff E, Rosner I. Anemia in CREST syndrome. Isr Med Assoc J. 2003;5:449 pubmed
  40. Shimizu Y, Kuwabara H, Ono A, Higuchi S, Hisada T, Dobashi K, et al. Intracellular Th1/Th2 balance of pulmonary CD4(+) T cells in patients with active interstitial pneumonia evaluated by serum KL-6. Immunopharmacol Immunotoxicol. 2006;28:295-304 pubmed
    ..n = 6), Sjogren's syndrome (n = 2), dermatomyositis (n = 1), progressive systemic sclerosis (n = 2), and CREST syndrome (n = 1) as the underlying diseases...
  41. Steensma E, Wu F. Images in clinical medicine. Colonic saccular diverticula. N Engl J Med. 2008;359:e13 pubmed publisher
  42. Jung C, Ferrari M, Rödiger C, Bahrmann P, Goebel B, Lauten A, et al. [Bedside visualization of altered microflow in autoimmune hemolysis by sidestream dark-field technology]. Med Klin (Munich). 2008;103:447-9 pubmed publisher
  43. Kouraklis G, Glinavou A, Karatzas G. Primary biliary cirrhosis accompanied by CREST syndrome. South Med J. 2002;95:1058-9 pubmed
    b>CREST syndrome, a relatively benign, slowly progressive variant of systemic scleroderma consists of calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia...
  44. Lopes M, Ludwig E, do Amaral B, Francisconi C, von Muhlen C. Motor activity of the gallbladder in systemic sclerosis. Am J Gastroenterol. 1999;94:3487-91 pubmed
    ..Five patients presented with CREST syndrome, 13 with the diffuse form of scleroderma. Four were men, 14 women (average age = 46.6+/-15.4 yr)...
  45. Jecan C, Bedereag Ş, Sinescu R, Grigorean V, Cozma C, Bordianu A, et al. A case of a generalized symptomatic calcinosis in systemic sclerosis. Rom J Morphol Embryol. 2016;57:865-869 pubmed
    ..We describe the case of a 52-year-old woman with systemic sclerosis and CREST syndrome who was admitted in our Department with multiple painful and disabling tumoral masses, situated in nearly all ..
  46. Nakae R, Idei M, Kumano K, Okita S, Yamane K. Intracranial aneurysms in patients with CREST syndrome. Neurol Med Chir (Tokyo). 2009;49:402-6 pubmed
    b>CREST syndrome is a variant of scleroderma characterized by calcinosis, Raynaud's phenomenon, esophageal hypomotility, sclerodactyly, and telangiectasia, and is a collagen vascular disease characterized by inflammation and fibrosis of ..
  47. Johnson S, Iazzetta J, Dewar C. Severe Raynaud's phenomenon with yohimbine therapy for erectile dysfunction. J Rheumatol. 2003;30:2503-5 pubmed
    ..We describe a patient with CREST syndrome (calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia) who ..
  48. Preston I, Klinger J, Houtchens J, Nelson D, Mehta S, Hill N. Pulmonary edema caused by inhaled nitric oxide therapy in two patients with pulmonary hypertension associated with the CREST syndrome. Chest. 2002;121:656-9 pubmed
    ..We describe two of nine patients with PAH and CREST syndrome who had pulmonary edema develop during acute iNO testing...
  49. Marín Gabriel J, Solis Herruzo J. Primary biliary cirrhosis and CREST syndrome. Rev Esp Enferm Dig. 2004;96:219-20 pubmed
  50. Lavie F, Rozenberg S, Coutaux A, Koeger A, Bourgeois P, Fautrel B. Bronchiectasis in a patient with CREST syndrome. Joint Bone Spine. 2002;69:515-8 pubmed
    ..We report the case of a 70-year-old woman with CREST syndrome and vasculitis who developed multifocal symptomatic bronchiectasis...
  51. Mason M. Looking for trouble -- patient preference, misdiagnosis and overtesting: a teachable moment. JAMA Intern Med. 2014;174:1548-9 pubmed publisher
  52. Tuteja A, Gurney M. Primary esophageal melanoma in a patient with limited scleroderma. J Clin Rheumatol. 2005;11:122-4 pubmed
  53. Corpechot C, Barbu V, Chazouilleres O, Poupon R. Fetal microchimerism in primary biliary cirrhosis. J Hepatol. 2000;33:696-700 pubmed
    ..049). Fetal microchimerism does not seem to play a major role in most cases of PBC. However, the association with anticentromere antibodies suggests a possible role in the subgroup of patients with CREST syndrome or scleroderma.