Systemic Sclerosis

Question 1

Limited cutaneous systemic sclerosis

Answer 1

Limited cutaneous systemic sclerosis is the more limited version of systemic sclerosis. It used to be called CREST syndrome. This forms a helpful mnemonic for remembering the features of limited cutaneous systemic sclerosis:

C – Calcinosis
R – Raynaud’s phenomenon
E – oEsophageal dysmotility
S – Sclerodactyly
T – Telangiectasia

Question 2

Diffuse cutaneous systemic sclerosis

Answer 2

Diffuse cutaneous systemic sclerosis includes the features of CREST syndrome plus many internal organs causing:

• Cardiovascular problems: hypertension and coronary artery disease
• Lung problems: pulmonary hypertension and pulmonary fibrosis. Resp issues most common cause of death
• Kidney problems: glomerulonephritis and scleroderma renal crisis
• Scleroderma affects trunk and proximal limbs predominately

Question 3

Scleroderma Renal Crisis

Management

Answer 3

Hypertension + AKI

Management: captopril

Question 4

Autoantibodies

Answer 4

Antinuclear antibodies (ANA) are positive in most patients with systemic sclerosis. They are not specific to systemic sclerosis.

Anti-centromere antibodies are most associated with limited cutaneous systemic sclerosis.

Anti-Scl-70 antibodies are most associated with diffuse cutaneous systemic sclerosis. They are associated with more severe disease.

Question 5

What features do diagnostic criteria use?

Answer 5

clinical features

antibodies

nailfold capillaroscopy.

Question 6

Management

Answer 6

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

Non-medical management involves:

• Avoid smoking
• Gentle skin stretching to maintain the range of motion
• Regular emollients
• Avoiding cold triggers for Raynaud’s
• Physiotherapy to maintain healthy joints
• Occupational therapy for adaptations to daily living to cope with limitations

Medical management focuses on treating symptoms and complications:

• Nifedipine can be used to treat symptoms of Raynaud’s phenomenon
• Anti acid medications (e.g. PPIs) and pro-motility medications (e.g. metoclopramide) for gastrointestinal symptoms
• Analgesia for joint pain
• Antibiotics for skin infections
• Antihypertensives can be used to treat hypertension (usually ACE inhibitors)
• Treatment of pulmonary artery hypertension
• Supportive management of pulmonary fibrosis

Question 7

What triggers Raynaud’s phenommenon?

Answer 7

Beta Blockers