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CREST syndrome

The CREST variant of scleroderma. Calcinosis Raynaud's phenomenon Esophageal dysmotility Sclerodactyly Telangiectasias 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: 1) anticentromere antibodies (70-90%) 2) anti SCL-70 (10%) 3) pulmonary function testing shows decreased DLCO Special laboratory: - echocardiogram to assess pulmonary artery hypertension (initial test) - right heart catherization necessary to establish diagnosis Complications: 1) pulmonary hypertension 2) biliary cirrhosis 3) gangrene of the digits Management: 1) problem oriented (see scleroderma) 2) prognosis a) better for CREST than scleroderma (systemic sclerosis) b) 70% 10 year survival for CREST

Related

calcinosis esophageal dysmotility Raynaud's phenomenon sclerodactyly telengiectasia

General

systemic sclerosis syndrome

References

  1. Manual of Medical Therapeutics, 28th ed, Ewald & McKenzie (eds), Little, Brown & Co, Boston, 1995, pg 525
  2. Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 876
  3. Medical Knowledge Self Assessment Program (MKSAP) 11, 17, 18, 19. American College of Physicians, Philadelphia 1998, 2015, 2018, 2022.