Systemic sclerosis

Question 1

What are the 3 types of sclerosis of the skin that exist?

Answer 1

Isolated Raynaud’s
Localised scleroderma
Systemic sclerosis

Question 2

What is Raynaud’s?

Answer 2

Innapropriate vasospasm in response to cold

Question 3

In which conditions is Raynaud’s a symptom?

Answer 3

Rheumatoid arthritis
Systemic sclerosis
MCTD
Systemic Lupus Erythematosus

Question 4

How can you differentiate between idiosynchratic Raynaud’s and Systemic sclerosis?

Answer 4

Auto-antibody testing

Microscopy imaging of the capillaries in the nail beds

Question 5

Why is SSc such a complicated disease?

Answer 5

Multi-systemic disease

Question 6

What are the different forms of SSc?

Answer 6

Limited
Diffused
Overlap

Question 7

What trait determines survivability of a SSc patient?

Answer 7

Involvement of internal organs

Question 8

What are complications of SSc present in all patients?

Answer 8

Raynaud’s

GI involvement

Question 9

What are complications of SSc present in diffuse stages of the condition?

Answer 9

Lung fibrosis
Pulmonary hypertension
Renal and heart involvement

Question 10

How does SSc affect the GI tract?

Answer 10

Causes fibrosis of the gut wall

Question 11

What is a symptom of the GI tract that indicates Raynaud’s is a symptom of SSc?

Answer 11

Acid reflux

Question 12

How does SSc affect esophageal mobility?

Answer 12

No normal peristaltic waves causes the acid of the stomach to form acid reflux

Damage of the esophagus and acid aspiration

Question 13

How can we measure esophageal mobility?

Answer 13

Manometry

Question 14

What are the consequences of esophageal immobility?

Answer 14

Acid aspiration

Acid reflux

Question 15

What are the two vascular pathologies triggered in SSc?

Answer 15

Pulmonary hypertension

Scleroderma renal crisis

Question 16

How can we treat scleroderma renal crisis?

Answer 16

ACE inhibitors

50% present with chronic kidney disease and need dialysis

Question 17

How can we treat pulmonary hypertension?

Answer 17

Endothelin antagonists

Question 18

What are current therapies for SSc?

Answer 18

Esophageal reflux disease - reduce volume of stomach acid
Intestinal bacterial overgrowth - antibiotics
Lung fibrosis - immunosuppression

Question 19

What are complications of SSc which have no treatment?

Answer 19

Digital ulcers 
GI disease - malnutrition 
Severe pain 
Sexual dysfunction 
Physiological and emotional impact