Systemic Sclerosis

Question 1

What is systemic sclerosis?

Answer 1

Autoimmune inflammatory and fibrotic connective tissue disease, unclear cause, affects skin (scleroderma) and internal organs.

Question 2

What are the two main patterns of disease?

Answer 2

1. Limited cutaneous systemic sclerosis (CREST syndrome)

2. Diffuse cutaneous systemic sclerosis

Question 3

What are the main features of limited cutaneous systemic sclerosis (CREST)?

Answer 3

C - Calcinosis
R - Raynaud's phenomenon
E - oEsophageal dysmotility
S - Sclerodactyly
T - Telangiectasia

Question 4

What is the difference between limited and diffuse cutaneous systemic sclerosis?

Answer 4

Diffuse has features of CREST like limited, but also affects internal organs.

Question 5

What are the common cardiovascular problems in diffuse cutaneous systemic sclerosis?

Answer 5

Hypertension and coronary artery disease

Question 6

What are the common lung problems in diffuse cutaneous systemic sclerosis?

Answer 6

Pulmonary hypertension and pulmonary fibrosis

Question 7

What are the common kidney problems in diffuse cutaneous systemic sclerosis?

Answer 7

Glomerulonephritis and scleroderma renal crisis

Question 8

What is this describing (part of CREST)?
Restricted range of movement of hands due to tight skin, reduced joint function and loss of fat on fingers, skin can break and ulcerate.

Answer 8

Sclerodactyly

Question 9

What is this describing (part of CREST)?

Dilated small blood vessels in the skin, fine and thready appearance.

Answer 9

Telangiectasia

Question 10

What is this describing (part of CREST)?

Calcium deposit build up in the skin, most commonly found in fingertips.

Answer 10

Calcinosis

Question 11

What is this describing (part of CREST)?
Fingertips go white then blue in response to even mild cold, caused by vasoconstriction of vessels supplying fingers. Can occur without systemic disease.

Answer 11

Raynaud’s phenomenon

Question 12

What is this describing (part of CREST)?

Swallowing difficulties, acid reflux, and oesophagitis caused by connective tissue dysfunction.

Answer 12

Oesophageal dysmotility

Question 13

Which antibodies are involved in systemic sclerosis?

Answer 13

1. Anti-nuclear - +ve in most with SS, not specific.
2. Anti-centromere - limited cutaneous systemic sclerosis
3. Anti-Scl-70 - diffuse cutaneous systemic sclerosis (severe disease)

Question 14

What is nailfold capillaroscopy and what can it help to diagnose/rule out?

Answer 14

1. Magnify and examine capillaries in nailbed.

2. Performed in Raynaud’s phenomenon, -ve finding rules out systemic sclerosis.

Question 15

How is a diagnosis of systemic sclerosis made?

Answer 15

Based on classification criteria - clinical features, antibodies, nailfold capillaroscopy.

Question 16

When are steroids and immunosuppressants started in a patient with systemic sclerosis?

Answer 16

With diffuse disease and in the presence of complications such as pulmonary fibrosis.

Question 17

What is the non-pharmaceutical management for systemic sclerosis?

Answer 17

Avoid smoking, gentle skin stretching, emollients, avoid cold triggers (Raynaud’s), PT and OT input.

Question 18

What is the pharmaceutical management for these aspects of systemic sclerosis?

1. Raynaud’s phenomenon
2. GI symptoms

Answer 18

1. Nifedipine

2. Anti-acid and pro-motility medications