Chapter 6- Systemic Sclerosis (Scleroderma)

Question 1

Characteristics of Systemic Sclerosis (3)

Answer 1

Chronic inflammation
Widespread damage to small blood vessels
Progressive interstitial and perivascular fibrosis in the skin and multiple organs

Question 2

What organs can be involved in systemic sclerosis? (5)

Answer 2

GI tract, kidneys, heart, muscles, lungs

Question 3

How does majority of death occur in Systemic Sclerosis?

Answer 3

Renal failure, cardiac failure, pulmonary insufficiency, or malabsorption

Question 4

Diffuse Scleroderma

Answer 4

Widespread skin involvement at onset with rapid progression

Question 5

Limited Scleroderma

Answer 5

Skin involvement is confined to fingers, forearms, and face

Late visceral involvement

Question 6

Cause of systemic sclerosis

Answer 6

Autoimmunity, vascular damage, fibrosis

Question 7

Epidemiology of systemic sclerosis

Answer 7

female to male ratio 3:1

peak incidence in age 50-60

Question 8

Clinical features of systemic sclerosis

Answer 8

cutaneous changes - skin thickening
Raynaud phenomenon
dysphagia 
respiratory difficulty
myocardial fibrosis

Question 9

Dysphagia in systemic sclerosis

Answer 9

from esophageal fibrosis causing hypomotility

Question 10

Myocardial fibrosis in systemic sclerosis

Answer 10

cause arrhythmias or cardiac failure

Question 11

Raynaud phenomenon

Answer 11

Episodic vasoconstriction of the arteries and arterioles of extremities

Question 12

Clinical features of limited scleroderma

Answer 12

CREST syndrome

Question 13

CREST Syndrome

Answer 13

Calcinosis
Raynaud phenomenon
Esophageal dysmotility
Sclerodactyly
Telangiectasia