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Systemic Sclerosis (Scleroderma): CREST Syndrome

Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer
Overview: What is CREST?
"Pure" CREST
"Plus" CREST
CREST Antibodies
CREST Overlaps
CREST Patient Stories
Also See

Overview: What is CREST?

CREST is a form of Systemic Sclerosis (scleroderma) which is characterized by Calcinosis (calcium deposits), usually in the fingers; Raynaud's;loss of muscle control of the Esophagus, which can cause difficulty swallowing; Sclerodactyly, a tapering deformity of the bones of the fingers; and Telangiectasia, small red spots on the skin of the fingers, face, or inside of the mouth. (Also see: What is Scleroderma?, Types of Scleroderma and Systemic Symptoms)






It takes only two of the five CREST symptoms for a diagnosis of CREST (either "pure" or "plus") to be made. For example, a patient with Calcinosis and Raynaud's would have CREST (which for precision may also be written as CRest, but it is CREST nonetheless.)
CREST Syndrome. "Patients with CREST syndrome are a subset of patients with scleroderma..." Medscape.

"Pure" CREST

"Pure"CREST — by itself: "Pure" CREST is diagnosed when patients have two or more symptoms of CREST but they do not meet the criteria for either Limited or Diffuse Scleroderma. That is, they must not have tight skin above their wrists, and if there is tight skin on their fingers, they must not have either pitting digital ulcers or lung fibrosis.

"Plus" CREST

"Plus" CREST — along with. When CREST symptoms appear along with another form of Scleroderma, it is referred to as, for example, "Limited Scleroderma plus CREST" or "Diffuse Scleroderma plus CREST." A person may also have any other autoimmune disease " plus CREST."


Although some doctors still believe CREST is a useful subcategory, the existing research studies have been unable to predict consistently how (or whether) the disease will progress to Diffuse Systemic Scleroderma in any specific individual.
Primary biliary cirrhosis accompanied by CREST syndrome. Second Department of Propedeutic Surgery, Medical School University of Athens, Greece. South Med J 2002 Sep;95(9):1058-9. PubMed, (Also see: Liver Involvement

History of CREST

CRST Syndrome In 1964, Winterbauer defined and named the CRST syndrome, a variant of scleroderma, when he was a Johns Hopkins medical student. Bull. Johns Hopkins Hosp. 114: 361-383, 1964.
CREST First Defined : In 1979, CRST was expanded to CREST by Shulman's group, also at Johns Hopkins, who added esophageal involvement to the cardinal manifestations. Arch Intern Med 1979 Nov;139(11):1240-4.

CREST Antibodies

Antibodies: Anticentromere Antibodies (ACA) are the most common antibodies found in CREST Syndrome. ISN.
Low prevalence of anti-centromere antibodies in scleroderma in Morocco (about 272 cases). the low prevalence of ACA observed in this survey, when compared to American, European and Japanese studies, is probably due to ethnic variation in frequency of ANA. Indeed, low rates or absence of ACA have been reported in south-African, Afro-American, Indian, and Thai studies. (PubMed) Ann Biol Clin (Paris). 2007 May-Jun;65(3):291-7.

CREST Overlaps

Granulomatous uveitis, CREST syndrome, and primary biliary cirrhosis. Our case report illustrates an association between ocular and hepatic diseases, which may be entirely coincidental but could, nevertheless, encourage further investigation for a common immunological pathway. Br J Ophthalmol 2000;84:546. (Also see: Liver Involvement and Uveitis)


CREST Patient and Caregiver Stories

CREST Patient and Caregiver Stories. Read personal stories from CREST patients and caregivers of CREST patients. ISN.

Also See

Calcinosis. ISN.
Gastrointestinal: Esophageal Involvement. ISN.
Raynaud's. ISN.
Scleroderma Photos. ISN.
Skin Involvement. ISN.
Telangiectasia. ISN.
Scleroderma Videos. ISN
Go to CREST Stories

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