CREST syndrome
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Introduction
The CREST variant of scleroderma.
Pathology
- 70% have lung involvement with pulmonary hypertension
Clinical manifestations
- skin thickening limited to face, neck & hands
- sclerodactyly
- telangiectasias
- calcinosis
- Raynaud's phenomenon
- esophageal dysmotility
- pulmonary hypertension (70%)
- fixed split second heart sound
Laboratory
- anticentromere antibodies (70-90%)
- anti SCL-70 (10%)
- pulmonary function testing shows decreased DLCO
Diagnostic procedures
- echocardiogram to assess pulmonary artery hypertension (initial test)
- right heart catherization necessary to establish diagnosis
Complications
Management
- problem oriented (see scleroderma)
- prognosis
- better for CREST than scleroderma (systemic sclerosis)
- 70% 10 year survival for CREST
More general terms
Additional terms
References
- ↑ Manual of Medical Therapeutics, 28th ed, Ewald & McKenzie (eds), Little, Brown & Co, Boston, 1995, pg 525
- ↑ Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 876
- ↑ Medical Knowledge Self Assessment Program (MKSAP) 11, 17, 18, 19. American College of Physicians, Philadelphia 1998, 2015, 2018, 2022.