System Sclerosis

Question 1

What is the most common population affected in systemic sclerosis?

Answer 1

Women between 45-65 (middle aged females are the key demographic)

Question 2

Where does the skin thickening have to occur for it to be characterized as diffuse? For limited?

Answer 2

Diffuse skin thickening is when it occurs proximal to the elbows or knees at any time in the disease. Limited is when it is limited to distal of elbows or knees (can involve face)

Question 3

What is more rapid in its progression, diffuse or limited scleroderma?

Answer 3

Diffuse is developed much faster after first disease symptoms, with 90% show internal organ involvement in first two years. Limited patients can show restricted or non-progressive skin thickening over many years

Question 4

What antibody is prototypical of limited scleroderma? Which is prototypical for diffuse?

Answer 4

Anti-centromere antibodies for limited. Anti-Scl-70 for diffuse. (antibodies to DNA topoisomerase I)

Question 5

What is the CREST syndrome acronym from pathoma about scleroderma, limited type?

Answer 5

C: Calcinosis/anti-Centromere antibodies
R: Raynaud's
E: esophageal dysmotility
S: Sclerodactyly
T: telangiectasia (dilation of capillaries)

Question 6

What are the most commonly affected internal organs?

Answer 6

Vessels (Raynaud’s)
GI tract (esophageal dysmotility and reflux)
Lungs (interstitial fibrosis and pulmonary hypertension)
Kidneys (scleroderma renal crisis)

Question 7

What is the most common cardiac pathology with SSc?

Answer 7

Fibrosis of the heart causing congestive heart failure (diastolic dysfunction) and arrhythmias

Question 8

What are the 3 main components of the pathogenesis of SSc?

Answer 8

1. Vascular/endothelial cell injury and obliteration
2. Fibrosis with overproduction of structurally normal collagen
3. Immune system activation

Question 9

What is the most plasuible initiating event in SSc?

Answer 9

Autoimmune mesenchymal damage. Secondary to that is endothelial dysfunction which leads to inflammation and secretion of growth factors (as well as vasoconstriction)

Question 10

What is the cell type that causes the most damage in SSc?

Answer 10

Fibroblast activation! Laying down of collagen causing fibrosis leads to skin tightening and organ damage.