Scleroderma

Question 1

What is it

Answer 1

a chronic autoimune diffuse disease of connective tissues causing fibrosis of skin, joints, blood vessels, and internal organs

Question 2

what is scleroderma usually accompanied by

Answer 2

Reynauds Phenomenon

Question 3

how does skin look

Answer 3

taut, firm, edematous, firmly bound to subcutaneous tissue

Question 4

limited system sclerosis/scleroderma

Answer 4

symmetrical skin involvement of distal extremities and face
- late visceral and pulmonary hypertension involvement
- CREST syndrome: calcinosis, Raynaud’s, esophageal dysfunction, sclerodactyly, and telangiectasis

Question 5

diffuse systemic sclerosis/scleroderma

Answer 5

symmetrical widespread skin involvement of distal and proximal extremities, face, trunk
- rapid progression of skin changes
- important internal organs are involves

Question 6

management

Answer 6

no specific therapy
- supportive therapy including corticosteroids, vasodilators, analgesics, immunosuppressive agents

Question 7

PT management

Answer 7

slow development of contractures and deformities
- skin mangement
- exercise
- joint protection

Question 8

precautions

Answer 8

• sensitive to pressure, acute hypertension, stress regulation blood pressure checks and vitals monitoring
• pulmonary hypertension can lead to right sided heart failure