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24 Cards in this Set

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Define Scleroderma
Disease/condition manifested by inappropriate scar tissue formation (fibrosis) throughout the entire body.
Scleroderma is also known as what? x2
PSS - Progressive Systemic Sclerosis

SSC - Systemic Sclerosis
Epidemiology of scleroderma:

- population
- gender
19 to 75 per 100,000

1 male per 7 to 12 females
Scleroderma classification x3
Localized Scleroderma

Limited Scleroderma

Diffuse Systemic Sclerosis
Which form of Scleroderma predominantly affects the trunk?
Diffuse systemic sclerosis
What are the symptoms of Limited Scleroderma? x5
1. Calcinosis
2. Raynaud's phenomenon
3. Esophogeal dysmotility
4. Sclerodactyly
5. Telangiectasia
List three Scleroderma-like diseases.
Eosinophilic fasciitis

Eosinophilic myalgia syndrome

Scleredema seen in Diabetes Mellitus
Describe Raynaud's phenomenon and include the causes behind the manifestations.
Distinctive changes in color due to stress (cold or emotion) induced vasospasm:

Red - Vasodilation & oxygenated RBC

White (Blanching) - Vasospasm

Blue (Cyanosis) - Deoxygenated RBC
What are the three stages of skin progression in Scleroderma, and include characters of each.
Earliest stage - Edematous skin

Fibrotic stage - Hard and thick skin

Atrophic stage - Tight skin
Scleroderma manifestations:

- mouth x2
Decrease oral aperture

Purse string lips
Scleroderma manifestations:

- esophagus x2
Esophagus dysmotility of lower third

Decreased LES (lower esophagus sphincter)
Esophagus dysmotility causes what?
Decreased peristalsis
Decreased LES (lower esophagus sphincter) causes what? x2
1. Reflux esophagitis

2. Barrett's (stricture formation)
Scleroderma manifestations:

- Small intestine x5
Reduced peristalsis leads to:

Bacterial overgrowth
Diarrhea
Stasis
Malnutrition
Increased dilation
Scleroderma manifestations:

- Large intestine
Thinning of muscular walls will lead to WIDE-MOUTH DIVERTICULUM
Scleroderma manifestations:

- Liver
Primary Biliary Cirrhosis
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)
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
Scleroderma manifestations in the cardiac would show what two diseases?
Pericardial effusion

Band necrosis
Scleroderma manifestations in the musculoskeletal would show what? x5
Myositis

Distal Phalangeal Tuft absorption

Arthritis

Tendon friction rubs

Arthralgia
Lab antibody finding for Limited Scleroderma.
Anti-centromere antibody
Lab antibody findings for Diffuse Systemic Sclerosis.
Anti-topoisomerase SCL-70 antibody
Treatment for Scleroderma depends on what?
Manifestations and organ involved
How would you treat the prevention of Scleroderma renal crisis
ACE inhibitors