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18 Cards in this Set
- Front
- Back
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What is scleroderma?
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An autoimmune connective tissue disorder of unknown etiology characterized by:
1. Fibrosis 2. Vascular Dysfunction 3. Immune Dysregulation |
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What is seen with regard to patients skin in scleroderma?
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Tough, Tight, Leathery skin
Raynauds Phenomenon - discoloration of fingers and toes Calcinosis - calcium deposits in skin |
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What are the 3 classifications of Systemic Scleroderma?
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1. Diffuse
2. Limited 3. Overlap |
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What are the characteristics of Diffuse Cutaneous Scleroderma?
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- symmetric, widespread skin fibrosis
- RAPID progression - EARLY visceral organ involvement |
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What are the characterisitcs of Limited Cutaneous scleroderma?
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- Symmetric skin fibrosis LIMITED to distal extremities and face
- Raynauds seen 10 yrs b4 skin affected - SLOW progression |
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What is the acronym for Limited Cutaneous scleroderma?
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CREST
C = calcinosis R = Raynauds E = Esophageal Dismotility S = Sclerodactyly T = telangectasias - abnormal dilation of blood vessels |
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What is the female:male ratio in schleroderma?
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4:1
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Is there a single gene that confers predictable risk for scleroderma?
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NO
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What is the end result of the pathogenesis of scleroderma?
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FIBROSIS
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Are autoantibodies directly pathogenic in scleroderma?
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NO
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What is the most important cytokine in scleroderma?
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TGF-B
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What role does TGF-B play in scleroderma?
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Sensitizes fibroblasts to stay persistently active
- SSc fibroblasts express increased levels of TGF-B receptors - Decreases production of collagen degrading enzymes |
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What proteins act in the signaling pathways of TGF-B
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SMAD proteins
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What is the second most important cytokine in scleroderma?
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CTGF (Connective Tissue Growth Factor)
- stimulated by TGF-B - sensitizes fibroblasts to stay persistently active |
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What is most likely the initial causative event in scleroderma?
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Vascular dysfunction
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What phenotypic changes are seen in fibroblasts that cause connective tissue contracture?
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Because of TGF-B, CTGF, change from fibroblasts to myofibroblasts
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What gene is upregulated in scleroderma?
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COL1A1 that causes excess production of (normal) collagen
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What 2 other organs are commonly affected in scleroderma?
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1. Kidney - vascular damage, can cause renal crisis
2. Lung - leading cause of death, fibrosis of lungs |
