Scleroderma

Question 1

What is the peak age of scleroderma incidence?

Answer 1

30 - 50

Question 2

What is the pathophysiology of scleroderma?

Answer 2

1- unknown trigger
2- CD4+ T-cells activated
3- cytokine & growth factor
4- leukocyte recruitment & increased fibroblasts
5- increased deposition of collagen & extracellular matrix proteins into tissue
6- small vasculopathy & multiorgan interstitial fibrosis (systemic sclerosis)

Question 3

What are the differences between diffuse & limited scleroderma?

Answer 3

DIFFUSE LIMITED
- widespread skin involvement - limited to distal extremities (face, hands, neck)
- rapid onset after Raynauds - delayed onset after Raynauds
- significant visceral involvement - visceral involvement late
- antitopoisomerase I (antiscleroderma) - anticentromere
- renal failure & interstitial lung disease - CREST syndrome -> Calcinosis, Raynaud, Esophageal dysmotility,
Sclerodactyly of the fingers, Telangiectases

Question 4

What are the clinical features of scleroderma?

Answer 4

1- Raynaud Phenomenon -> vasospasm & thickening of vessel walls in digits

• can lead to digital ischemia with ulceration & infarction
• cold temperature & stress precipitate it

2- cutaneous fibrosis -> thickening & hardening of the skin (smooth, shiny, puffy)

• sclerodactyly -> fibrotic thickening & tightening of skin on fingers & hands
• digital pitting -> hyperkeratotic scarring on fingertips
• loss of facial expressions

3- GI involvement

• esophageal dysmotility -> dysphagia & reflux
• small bowel dysmotility -> bloating, gas, constipation, & cramping

4- Pulmonary disease

• pulmonary HTN (limited
• interstitial lung disease (diffuse
• increased risk of lung cancer

5- Cardiac disease -> fibrosis, myocarditis, pericarditis

6- renal disease -> thickening of arteriolar walls -> decreased renal blood flow -> reduced kidney function
- scleroderma renal crises

7- CREST syndrome

Question 5

What is the clinical presentation of sclerodermal renal crises?

Answer 5

occures in diffuce SSc

• oliguric renal failure
• malignant hypertension
• encephalopathy
• microangiopathic hemolytic anemia

Question 6

How is scleroderma diagnosed?

Answer 6

• ANA
• limited -> anticentromere antibodies (ACA)
• diffuse -> anti-Scl-70 (anti-topoisomerase I) & anti-RNA polymerase III

Question 7

How is scleroderma managed?

Answer 7

symptomatic treatment only

• Raynaud -> body warming & CCBs
• renal crises -> ACEIs
• interstitial lung fibrosis -> corticosteroids
• heart affection -> corticosteroids
• GI affection -> frequent small meals