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Glossary of Progressive Systemic Sclerosis (aka, Scleroderma)

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What is the other name for Systemic Progressive Sclerosis?
Scleroderma
What tissue is effected by scleroderma and in what way is the tissue effected?
Connective tissue inflammation with excessive fibrosis throughout the body.
What organs are effected by scleroderma?
- skin
- lungs
- GI tract
- Musculoskeletal System
- Kidneys
What gender is most affected? How many more times (X) is this gender affected compared to the other.
Women are effected 3X more than men.
Is scleroderma an autoimmune disease? yes or no?
Yes
A patient with scleroderma presents with what problems?
- tissue inflammation (small vessel vasospasm and fibrosis)
- Fibrous tissue decreases size of vessel lumen
- loss of vascularity
- induration (hardening of connective tissue)
There are two kinds of scleroderma. What are they?
1. Diffuse scleroderma
2. CREST Phenomenon
Of diffuse scleroderma and CREST phenomenon, which is the most severe?
Diffuse scleroderma
What % of patients have lung involvement with scleroderma?
70%
What organ, affected by scleroderma, is the most common cause of death?
The lungs
In scleroderma, the alveoli become fibrotic, what does this cause?
Impaired gas exchange
How does scleroderma affect the GI tract?
- atrophy of muscularis, replace by fibrotic tissue
- Lower esophagus becomes rigid and causes dysphagia
- malabsorption in the small intestine and colon
How does scleroderma affect the musculoskeletal system?
- inflammation and fibrosis (arthritis) in fingers and knees
50% of patients with scleroderma have failure of what organ?
Kidney - from hypertension, and intimal thickening of vessel walls
As a doc. your clinical findings would be what?
- color changes in skin
- vasoconstriction and spasm of digital arteries
- dysphagia
- rheumatoid-like pain in finger and knee joints
What is C.R.E.S.T. when referring to progressive systemic sclerosis?
C = calcinosis
R = Raynaud's phenomenon
E = esophageal dysfunction
S = scleroderma
T = telangiectasia (dilatation of blood vessels)
On an x-ray, what would you see in a patient with scleroderma?
Resorption of soft tissue in finger tips. Resorption of bone in the terminal distal phalanges
If you did a blood lab, what would you find in a patient with progressive systemic sclerosis?
- 50% of people have elevated levels of IgG
- 20-30% have + Rheumatoid Factor
- 40-50% have + anti-nuclear antibodies (ANA)

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