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24 Cards in this Set
- Front
- Back
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Define Scleroderma
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Disease/condition manifested by inappropriate scar tissue formation (fibrosis) throughout the entire body.
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Scleroderma is also known as what? x2
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PSS - Progressive Systemic Sclerosis
SSC - Systemic Sclerosis |
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Epidemiology of scleroderma:
- population - gender |
19 to 75 per 100,000
1 male per 7 to 12 females |
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Scleroderma classification x3
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Localized Scleroderma
Limited Scleroderma Diffuse Systemic Sclerosis |
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Which form of Scleroderma predominantly affects the trunk?
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Diffuse systemic sclerosis
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What are the symptoms of Limited Scleroderma? x5
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1. Calcinosis
2. Raynaud's phenomenon 3. Esophogeal dysmotility 4. Sclerodactyly 5. Telangiectasia |
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List three Scleroderma-like diseases.
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Eosinophilic fasciitis
Eosinophilic myalgia syndrome Scleredema seen in Diabetes Mellitus |
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Describe Raynaud's phenomenon and include the causes behind the manifestations.
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Distinctive changes in color due to stress (cold or emotion) induced vasospasm:
Red - Vasodilation & oxygenated RBC White (Blanching) - Vasospasm Blue (Cyanosis) - Deoxygenated RBC |
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What are the three stages of skin progression in Scleroderma, and include characters of each.
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Earliest stage - Edematous skin
Fibrotic stage - Hard and thick skin Atrophic stage - Tight skin |
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Scleroderma manifestations:
- mouth x2 |
Decrease oral aperture
Purse string lips |
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Scleroderma manifestations:
- esophagus x2 |
Esophagus dysmotility of lower third
Decreased LES (lower esophagus sphincter) |
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Esophagus dysmotility causes what?
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Decreased peristalsis
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Decreased LES (lower esophagus sphincter) causes what? x2
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1. Reflux esophagitis
2. Barrett's (stricture formation) |
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Scleroderma manifestations:
- Small intestine x5 |
Reduced peristalsis leads to:
Bacterial overgrowth Diarrhea Stasis Malnutrition Increased dilation |
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Scleroderma manifestations:
- Large intestine |
Thinning of muscular walls will lead to WIDE-MOUTH DIVERTICULUM
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Scleroderma manifestations:
- Liver |
Primary Biliary Cirrhosis
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Scleroderma manifestations:
- renal - bad prognosis if what? x5 |
Scleroderma Renal Crisis
Creatinine > 3 mg/dl Older age Male Anemia (new onset) RNA (anti-polymerase III antibody) |
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Scleroderma manifestations in the pulmonary would show what two diseases?
What is the main symptom for both diseases? |
Interstitial Lung Disease
(aka - fibrosing alveolitis & pulmonary fibrosis) Pulmonary Vascular Disease (leads to Pulmonary HTN) Dsypnea |
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Scleroderma manifestations in the cardiac would show what two diseases?
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Pericardial effusion
Band necrosis |
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Scleroderma manifestations in the musculoskeletal would show what? x5
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Myositis
Distal Phalangeal Tuft absorption Arthritis Tendon friction rubs Arthralgia |
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Lab antibody finding for Limited Scleroderma.
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Anti-centromere antibody
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Lab antibody findings for Diffuse Systemic Sclerosis.
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Anti-topoisomerase SCL-70 antibody
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Treatment for Scleroderma depends on what?
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Manifestations and organ involved
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How would you treat the prevention of Scleroderma renal crisis
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ACE inhibitors
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