Systemic sclerosis

Systemic sclerosis PowerPoint PPT Presentation


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Epidemiology. Rare disease incidence in the US 19/million/year and prevalence of 19-75/100,000Rare in childrenPeak between age 35-65More women affectedFamily h/o other auto immune diseasesEthnic background influence survival and disease manifestation.. Clinical features. Raynaud'sTypical skin

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Systemic sclerosis

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1. Systemic sclerosis Also called scleroderma Multisystem disease of unknown etiology Pathogenesis include immune system activation , endothelial activation , and fibroblast activation this results in small blood vessels damage and tissue fibrosis.

2. Epidemiology Rare disease incidence in the US 19/million/year and prevalence of 19-75/100,000 Rare in children Peak between age 35-65 More women affected Family h/o other auto immune diseases Ethnic background influence survival and disease manifestation.

3. Clinical features Raynaud’s Typical skin changes Esophageal and small bowel dysfunction Interstitial lung disease Pulmonary hypertension Renal crises

4. What is Raynaud’s Phenomenon Reversible skin color changes:White to blue to red Due to vasospasm Induced by cold of emotion

5. Raynaud’s :Pallor phase

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