Systemic sclerosis

Question 1

What is systemic sclerosis

Answer 1

Autoimmune inflammatory and fibrotic connective tissue disease

Cause is unclear

Question 2

What are the two main disease patterns in systemic sclerosis?

Answer 2

Limited cutaneous systemic sclerosis

Diffuse cutaneous systemic sclerosis

Question 3

How does Limited cutaneous systemic sclerosis present?

Answer 3

Used to be called CREST syndrome

C – Calcinosis - calcium deposits under the skin
R – Raynaud’s phenomenon
E – oEsophageal dysmotility
S – Sclerodactyly
T – Telangiectasia

Question 4

How does diffuse cutaneous systemic sclerosis present?

Answer 4

The features of CREST syndrome plus effects on internal organs:

• Cardiovascular problems, particularly hypertension and coronary artery disease.
• Lung problems, particularly pulmonary hypertension and pulmonary fibrosis (fibrosis can occur in severe systemic sclerosis)
• Kidney problems, particularly glomerulonephritis and a condition called scleroderma renal crisis.

Question 5

What is scleroderma?

Answer 5

Refers to hardening of the skin.

This gives a the appearance of shiny, tight skin without the normal folds in the skin.

Most notable on the hands and face.

Question 6

What is sclerodactyly?

Answer 6

Skin tightening restricts range of motion and reduces function of the joint

The skin can break and ulcerate

Question 7

What are telangectasia?

Answer 7

Dilated small blood vessels in the skin.

They are tiny veins that have dilated. They have a fine, thready appearance.

Question 8

What causes systemic and pulmonary hypertension in systemic sclerosis?

Answer 8

Connective tissue dysfunction in the systemic and pulmonary arterial systems.

Systemic hypertension can be worsened by renal impairment.

Question 9

What is scleroderma renal crisis?

Answer 9

An acute condition where there is a combination of severe hypertension and renal failure.

Question 10

What are the antibodies seen in systemic sclerosis?

Answer 10

ANA positive in 90%
RF positive in 30%

anti-scl-70 antibodies associated with diffuse cutaneous systemic sclerosis

anti-centromere antibodies associated with limited cutaneous systemic sclerosis

Question 11

What are possible investigations in suspected systemic sclerosis?

Answer 11

Rheumatoid screen including FBC, CRP, ESR, U&Es
Autoantibody screen

Nailfold capillaroscopy - useful to support a diagnosis of systemic sclerosis and to investigate patients with Raynaud’s phenomenon to exclude systemic sclerosis

Question 12

Management of systemic sclerosis

Answer 12

Refer to specialist - rheumatology

Specialist MDT

Steroids and immunosuppressants usually started with diffuse disease and complications such as pulmonary fibrosis.

Conservative:

• Avoid smoking
• Gentle skin stretching
• Regular emollients
• Avoiding cold triggers for Raynaud’s

Medical:

• Nifedipine can treat Sx of Raynaud’s
• PPIs and pro-motility medications (e.g. metoclopramide) for GI Sx
• Treating any complications e.g. antibiotics for skin infections, antihypertensives for HTN