CREST syndrome

CREST syndrome
CREST syndrome
Classification and external resources
ICD-10 M34.1
ICD-9 710.1
OMIM 181750
DiseasesDB 29764
MeSH D017675

The limited cutaneous form of systemic scleroderma (lcSSc) is often referred to as CREST syndrome.[1] "CREST" is an acronym for the five main features:

It is a form of systemic scleroderma associated with antibodies against centromeres and usually spares the kidneys. If the lungs are involved it is usually in the form of pulmonary arterial hypertension.

CREST is a systemic inflammatory rheumatic disease and usually results in more pathologies than the five symptoms above.

Patients with lcSSc commonly and slowly produce a pulmonary artery hypertension which can result in heart failure. Blood vessel thrombosis and arteriosclerosis has also led to the necessity of amputation of fingers. Open leg sores can result from burst blood vessels and thin skin, leading to chronic infections. Other symptoms of CREST patients can be exhaustion, weakness, difficulties with breathing, dizziness and badly healing wounds.

The origin of CREST lies in the immune system. There is production of anti-nuclear and anti-centromere antibodies, although it is not known if these antibodies are involved in the cause of the symptoms of the disease. There is no known infectious cause.

There is no cure for this disease as there is no cure for scleroderma in general. It slowly progresses and should be examined and treated as soon as it has been diagnosed with immunosuppressives and other medications. There are treatments that can ameliorate the symptoms of esophageal reflux, pulmonary hypertension and Raynaud phenomenon.

See also

References

  1. ^ Winterbauer RH (1964). "Multiple telangiectasia, Raynaud'S phenomenon, sclerodactyly, and subcutanious calcinosis: a syndrome mimicking hereditary hemorrhagic telangiectasia". Bulletin of the Johns Hopkins Hospital 114: 361–83. PMID 14171636. 

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Look at other dictionaries:

  • CREST syndrome — a disease characterized by the association of calcinosis, Raynaud s disease, (o)esophageal malfunction, scleroclerodactyly (tapering fingers), and ataxia telangiectasia. It represents a variant of systemic sclerosis and may be associated with… …   Medical dictionary

  • CREST syndrome — a disease characterized by the association of calcinosis, Raynaud s phenomenon, (o)esophageal malfunction, sclerodactyly (tapering fingers), and telangiectasia. It represents a variant of systemic sclerosis and may be associated with severe… …   The new mediacal dictionary

  • Syndrome de crest — Pour les articles homonymes, voir Crest. Syndrome de CREST CIM 10 : M34.1 Le CREST Syndrome est une forme particulière de sclérodermie. Il associe à des degrés divers: Des Calcifications sous cutanées. = C Un syndrome de Raynaud. = R …   Wikipédia en Français

  • Crest — may refer to: Contents 1 In science 2 In popular culture 3 In commerce 4 Places 5 Acronyms …   Wikipedia

  • Syndrome de CREST — Pour les articles homonymes, voir Crest. Classification internationale des maladies CIM 10 : M34.1 Le CREST Syndrome est une forme particulière de sclérodermie. Il associe à des degrés divers: Des Calcifications sous cutanées. = C …   Wikipédia en Français

  • Syndrome de raynaud — On parle ici de la forme secondaire de la maladie de Raynaud (syndrome de Raynaud ou phénomène de Raynaud). Ce syndrome est beaucoup plus rare et généralement plus grave que la maladie. Le phénomène de Raynaud est un trouble de la circulation… …   Wikipédia en Français

  • Syndrome de fryns — Autre nom Hernie diaphragmatique faciès anormal Référence MIM …   Wikipédia en Français

  • Crest (Homonymie) — Crest Cette page d’homonymie répertorie les différents sujets et articles partageant un même nom. Crest est une ville du Sud de la France (Drôme) Le CREST est un centre de recherche français en économie et statistiques Le syndrome de CREST est… …   Wikipédia en Français

  • Crest (homonymie) — Crest Cette page d’homonymie répertorie les différents sujets et articles partageant un même nom. Crest est une ville du Sud de la France (Drôme) Le CREST est un centre de recherche français en économie et statistiques Le syndrome de CREST est… …   Wikipédia en Français

  • Syndrome de Fryns — Référence MIM 229850 Transmission Récessive Chromosome 8p23.1 15q26.2 Mutation Microdélétion ? Incidence 7 s …   Wikipédia en Français

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