Systemic Sclerosis

Question 1

Systemic sclerosis is interchangeable with which term?

Answer 1

Scleroderma

Question 2

Scleroderma translates directly to?

Answer 2

Hardening of the skin

Question 3

What is systemic sclerosis?

Answer 3

an autoimmune inflammatory and fibrotic connective tissue disease. The cause of the condition is unclear. It most notably affects the skin in all areas but it also affects the internal organs.

Question 4

What are the 2 main patterns of disease in systemic sclerosis?

Answer 4

Limited cutaneous systemic sclerosis

Diffuse cutaneous systemic sclerosis

Question 5

What are the features of Limited Cutaneous Systemic Sclerosis?

Answer 5

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 6

What are the features of Diffuse Cutaneous Systemic Sclerosis?

Answer 6

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

Cardiovascular problems, particularly hypertension and coronary artery disease.

Lung problems, particularly pulmonary hypertension and pulmonary fibrosis.

Kidney problems, particularly glomerulonephritis and a condition called scleroderma renal crisis.

Question 7

What is scleroderma?

Answer 7

Hardening of the skin

This gives a the appearance of shiny, tight skin without the normal folds in the skin. These changes are most notable on the hands and face.

Question 8

What is Sclerodactyly?

Answer 8

describes the skin changes in the hands

As the skin tightens around joints it restricts the range of motion in the joint and reduces the function of the joints. As the skin hardens and tightens further the fat pads on the fingers are lost. The skin can break and ulcerate.

Question 9

What is Telangiectasia?

Answer 9

dilated small blood vessels in the skin

They are tiny veins that have dilated

They have a fine, thready appearance

Question 10

What is Calcinosis?

Answer 10

Where calcium deposits build up under the skin

This is most commonly found on the fingertips

Question 11

What is Raynaud’s phenomenon?

Answer 11

where the fingertips go completely white and then blue in response to even mild cold

It is caused by vasoconstriction of the vessels supplying the fingers, this commonly occurs without any associated disease, however it is a classical feature of systemic sclerosis

Question 12

Oesophageal dysmotility?

Answer 12

caused by connective tissue dysfunction in the oesophagus

This is commonly associated with swallowing difficulties, acid reflex and oesophagitis

Question 13

Systemic and pulmonary hypertension is caused by?

Answer 13

connective tissue dysfunction in the systemic and pulmonary arterial systems

Systemic hypertension can be worsened by renal impairment

Question 14

Pulmonary fibrosis?

Answer 14

can occur in severe systemic sclerosis

This presents with gradual onset dry cough and shortness of breath

Question 15

what is Scleroderma renal crisis ?

Answer 15

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

Question 16

Autoantibodies involved with Systemic Sclerosis?

Answer 16

many & helpful in predicting the extent of disease and which organs will be affected

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 17

What is Nailfold Capillaroscopy used for?

Answer 17

This is a technique where the area where the skin meets the fingernails at the base of the fingernail (the nailfold) is magnified and examined.

This allows us to examine the health of the peripheral capillaries. Abnormal capillaries, avascular areas and micro-haemorrhages indicate systemic sclerosis.

It is useful to support a diagnosis of systemic sclerosis and to investigate patients with Raynaud’s phenomenon to exclude systemic sclerosis.

Patients with primary Raynaud’s without systemic sclerosis will have normal nailfold capillaries.

Question 18

Diagnosis of Systemic Sclerosis?

Answer 18

Diagnosis is based on classification criteria from the American College of Rheumatology (ACR) and European League Against Rheumatism (EULAR) published in 2013.

This involves meeting a number of criteria for clinical features, antibodies and nailfold capillaroscopy.

Question 19

Non-medical management of Systemic Sclerosis involves?

Answer 19

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

Question 20

Medical management of Systemic Sclerosis?

Answer 20

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