Transcript crest - UNC School of Medicine

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
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
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
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
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)
Systemic Sclerosis and Scleroderma Disorder

Treatment

Pulmonary



Renal


APAP, NSAIDS, PT
Myositis


NSAIDS or steroids for pericarditis
Arthritis


PPI/H2 for GERD, Abx for malabsorption, hypomotility metoclopramide or erythromycin
Cardiac


Monitor BP, ACE-I (not ARB) for HTN crisis
GI


Fibrosis: cyclophosphamide
Pulm HTN: vasodilators
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
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=jUjPkZ
wMzCQT8M:&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