Glossary of Progressive Systemic Sclerosis (aka, Scleroderma)
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- What is the other name for Systemic Progressive Sclerosis?
- 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
- GI tract
- Musculoskeletal System
- 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?
- 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?
- 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
- 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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