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CREST

CREST. J. Ryan Altman, MD AM REPORT 9 December 2009. Picture 1. Picture 2. Picture 3. Picture 4. Picture 5. What’s my diagnosis?. Other findings: picture 6. Systemic Sclerosis and Scleroderma Disorder. Definitions Scleroderma: presence of tight, thickened skin

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CREST

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  1. CREST J. Ryan Altman, MD AM REPORT 9 December 2009

  2. Picture 1

  3. Picture 2

  4. Picture 3

  5. Picture 4

  6. Picture 5

  7. What’s my diagnosis?

  8. Other findings: picture 6

  9. Systemic Sclerosis and Scleroderma Disorder • Definitions • Scleroderma: presence of tight, thickened skin • Anatomic distribution of sclerodermatous skin defines subtypes and disease associations • Localized scleroderma: morphea, linear, en coup de saber • Systemic sclerosis: multiorgan involvement • SSc with diffuse cutaneous involvement • SSc with limited cutaneous involvement (CREST) • SSc sine scleroderma (visceral disease without skin involvement) • Peak onset: age 30-50, Female>Male

  10. Systemic Sclerosis and Scleroderma Disorder • Classification criteria (1 maj. or 2 min.) • Major: skin findings extend prox to MCT or MTP joints • Minor: sclerodactyly (limited to fingers), digital pitting scars (from loss of substance on finger pad), bibasilar pulmonary fibrosis (mechanism: stimulatory autoantibodies against PDGF receptor activating collagen gene expression) • Proposed additions: presence of Reynaud's phenomenon, dropout or dilatation on nailfold capillaroscopy, and serologies

  11. Systemic Sclerosis and Scleroderma Disorder • Diagnostic studies • + anti-Scl-70 (anti-topoisomerase 1): 40% diffuse, 15% limited • + anti-centromere pattern (kinetochore): 60-80% limited, <5% diffuse • + ANA (>90%) • + RF (30%) • HLA Association: DR1 (DQ5), DQB1/DR4 (D13 subtypes) • If renal involvement: incr BUN and Cr, proteinuria • If pulm involvement: interstitial pattern on CXR/chest CT, restriction and/or decr DLCO on PFTs; Pulm HTN revealed by ECHO • Skin bx not routine, but helpful to assess other possible causes for skin thickening

  12. Systemic Sclerosis and Scleroderma Disorder • Limited involvement findings • Skin: thickening on distal extremities and face only • Nails: capillary dropout +/- dilitation • Pulm: Pulm HTN>Fibrosis • Renal: none (diffuse: renovascular HTN) • Cardiac: none (diffuse: restrictive cardiomyopathy) • Other: CREST (Calcinosis cutis, Raynaud’s, Esophageal dysmotility, Sclerodactyly, Telangiectasias) • Antibodies: Anticentromere (diffuse: Anti-Scl 70) • Prognosis: survival >70% at 10yr (diffuse: 40-60% at 10yr)

  13. Systemic Sclerosis and Scleroderma Disorder • Treatment • Pulmonary • Fibrosis: cyclophosphamide • Pulm HTN: vasodilators • Renal • Monitor BP, ACE-I (not ARB) for HTN crisis • GI • PPI/H2 for GERD, Abx for malabsorption, hypomotility metoclopramide or erythromycin • Cardiac • NSAIDS or steroids for pericarditis • Arthritis • APAP, NSAIDS, PT • Myositis • MTX, AZA, steroids • Skin • PUVA for morphea, emollients or oral steroids (caution can precipitate HTN renal crisis) for pruritis, immunosuppressants only minimal to modest benefit for fibrosis

  14. Bibliography • Dry gangrene: http://images.google.com/imgres?imgurl=http://www.wsiat.on.ca/images/mlo/diabetic_fig5.jpg&imgrefurl=http://www.wsiat.on.ca/english/wsiatDocs/mlo/diabetic_screen.htm&usg=__WP71rmUnUCfoMaE095mQ2utCjkA=&h=385&w=288&sz=63&hl=en&start=7&tbnid=jUjPkZwMzCQT8M:&tbnh=123&tbnw=92&prev=/images%3Fq%3Ddry%2Bgangrene%26gbv%3D2%26hl%3Den%26safe%3Doff • Raynaud Phenomenon: www.csmc.edu/images/354016_Raynaudsphenomenon.jpg • Sclerodactyly: bestpractice.bmj.com/.../295-10-tn_default.jpg • Scleroderma: www.scleroderma.org/.../diffuse-hands_jpg.jpg • Capillary telangiectasias: http://www.aafp.org/afp/2004/0315/p1417.html • Calcinosis cutis: www.dermis.net/bilder/CD051/100px/img0063.jpg • Connective Tissue Disorders: Pocket Medicine 3rd ed., Marc Sabatine • Uptodateonline.com: Classification of scleroderma disorders

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