Systemic Sclerosis (Scleroderma)

Question 1

Describe Scleroderma

Answer 1

“hardening of the skin”
Chronic, inflammatory, autoimmune connective tissue disorder Involving skin and other body systems
- Uncommon but high mortality

Question 2

What is Scleroderma often misdiagnosed as?

Answer 2

Often diagnosed as “probable SLE”

Question 3

Scleroderma Pathophysiology

Answer 3

Inflamed tissue becomes hardened/sclerotic

May involve kidneys, lungs, and vessels

Question 4

Leading cause of death?

Answer 4

KIDNEY FAILURE

Question 5

What are the two types of Scleroderma?

Answer 5

1. Limited cutaneous

2. Diffuse cutaneous

Question 6

What Syndrome is Limited Cutaneous Scleroderma associated with?

Answer 6

CREST Syndrome

Question 7

Describe CREST Syndrome

Answer 7

C = calcium deposits in skin
R = Raynaud's phenomenon
E = Esophageal dysfunction (reflux, decreased peristalsis)
S = Sclerodactyly: skin thickening + tightening 
T = Telangiectasis: capillary dilation, red marks on skin

Question 8

Assessment + Scleroderma:

What are the distinguishing characteristics?

Answer 8

1. Pitting edema of hands, fingers, and forearms
   - can progress
2. Skin thickening and Tightening
   - Shiny, taut skin (no wrankles)
   - loss of elasticity and ROM

Question 9

Assessment + Scleroderma:

Digestive Tract Involvement S/S

Answer 9

1. Dysphagia
2. Esophageal dysmotility, decreased peristalsis
3. Esophagitis
4. Malabsorption/Diarrhea

Question 10

Assessment + Scleroderma:

Cardiovascular Involvement S/S

Answer 10

1. Raynaud’s phenomenon

2. Digit necrosis (autoamputation)

Question 11

Assessment + Scleroderma:

Lung Involvement S/S

Answer 11

1. Pulmonary arterial HTN

Question 12

How is Scleroderma diagnosed? What can help to distinguish it? (2)

Answer 12

Diagnostic tests similar to SLE

Clinical presentation & Response to Meds help to distinguish it!

Question 13

Interventions + Scleroderma:

What are the two main care focuses?

Answer 13

1. COMFORT

2. Aspiration risk! (swallow study)

Question 14

Interventions + Scleroderma:

Comfort Interventions:

Answer 14

1. Bed Cradle***
2. Footboard
3. Room temperature (prevent Raynaud’s)
4. Gloves and socks, if tolerated (prevent Raynaud’s)
5. Joint pain interventions
6. Reduce stress