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[CREST syndrome]
1Service de Rhumatologie, Hôpital Bichat, 46, rue Henri-Huchard, 75877 Paris Cedex 18. olivier.meyer@bch.ap-hop-paris.fr
Annales De Medecine Interne
|September 10, 2002
Summary
CREST syndrome, a variant of systemic sclerosis, typically has a good prognosis. However, rare complications like pulmonary hypertension can be severe and life-threatening.
Area of Science:
- Rheumatology
- Dermatology
- Internal Medicine
Background:
- CREST syndrome is a subtype of progressive systemic sclerosis.
- It is characterized by calcinosis, Raynaud's phenomenon, esophageal dysfunction, and telangiectasia.
- The condition was first described in 1910 and the acronym coined in 1964.
Purpose of the Study:
- To define the characteristics and prognosis of CREST syndrome.
- To identify associated complications and their impact on patient outcomes.
Main Methods:
- Review of clinical case reports and literature.
- Analysis of characteristic autoantibodies (antinuclear antibodies targeting centromere proteins).
Main Results:
- CREST syndrome typically presents with limited skin involvement.
- Antinuclear antibodies against centromere proteins are found in over 50% of cases.
- The long-term prognosis is generally favorable, with disease duration often exceeding 10 years.
Conclusions:
- CREST syndrome has a good long-term prognosis.
- Rare but severe complications include digital gangrene and pulmonary hypertension.
- Pulmonary hypertension, though infrequent, carries a high mortality rate.