Systemic Sclerosis

Question 1

What is systemic sclerosis also known as?

Answer 1

scleroderma

It is a rare chronic autoimmune disease of unknown cause.

Question 2

Name the two main subtypes of systemic sclerosis.

Answer 2

• Limited cutaneous systemic sclerosis (lcSSc)
• Diffuse cutaneous systemic sclerosis (dcSSc)

lcSSc is characterized by skin fibrosis affecting the peripheries, while dcSSc presents with widespread skin involvement.

Question 3

What does CREST syndrome stand for?

Answer 3

• Calcinosis
• Raynaud’s phenomenon
• Esophageal dysmotility
• Sclerodactyly
• Telangiectasia

CREST syndrome is associated with limited cutaneous systemic sclerosis (lcSSc).

Question 4

What is the prevalence of systemic sclerosis in the UK?

Answer 4

1 in 10,000

Women are three times more commonly affected than men.

Question 5

At what ages do the majority of cases of systemic sclerosis present?

Answer 5

between 30 and 50

Family history and silica exposure are also risk factors.

Question 6

What are common dermatological features of systemic sclerosis?

Answer 6

• Raynaud’s phenomenon
• Sclerodactyly
• Calcinosis
• Hyperpigmentation
• Telangiectasia

Skin may also become itchy and dry with hair loss.

Question 7

What is the ‘Prayer sign’ in systemic sclerosis?

Answer 7

Inability to bring the palmar surfaces of the hands together completely

This is due to restricted hand mobility.

Question 8

What are some gastrointestinal features of systemic sclerosis?

Answer 8

• Oesophageal dysmotility
• Delayed gastric emptying
• Gastric antral vascular ectasia (GAVE)
• Reduced bowel motility

These can lead to symptoms like dyspepsia, reflux, and malabsorption.

Question 9

What is the main pulmonary manifestation of systemic sclerosis?

Answer 9

Pulmonary arterial hypertension

It is a leading cause of morbidity and mortality in systemic sclerosis.

Question 10

What are common cardiovascular features in diffuse cutaneous systemic sclerosis?

Answer 10

• Chest pain
• Palpitations
• Shortness of breath
• Dizziness
• Peripheral oedema

Ischaemic heart disease may occur secondary to microvascular coronary artery disease.

Question 11

What is a serious complication of systemic sclerosis related to the kidneys?

Answer 11

Scleroderma renal crisis

It can lead to acute and rapidly progressive renal failure.

Question 12

What are some investigations used for systemic sclerosis?

Answer 12

• Blood pressure monitoring
• Urinalysis
• Nailfold capillaroscopy
• ECG
• Blood tests for autoantibodies

Imaging studies like chest X-ray and echocardiography are also important.

Question 13

What is the first-line treatment for skin fibrosis in early diffuse cutaneous systemic sclerosis?

Answer 13

Mycophenolate mofetil (MMF)

Methotrexate is also considered.

Question 14

What lifestyle advice is important for managing Raynaud’s phenomenon?

Answer 14

• Wearing gloves
• Avoiding cold temperatures

Smoking cessation is also crucial.

Question 15

What surgical management options may be considered for digital ulcers?

Answer 15

• Debridement
• Digital sympathectomy
• Botox injection

These may be considered if medical management is insufficient.

Question 16

True or false: Fatigue is a common feature of systemic sclerosis.

Answer 16

TRUE

Other features include erectile dysfunction and ocular involvement.

Question 17

What is systemic sclerosis also known as?

Answer 17

scleroderma

It is a rare chronic autoimmune disease of unknown cause.

Question 18

Name the two main subtypes of systemic sclerosis.

Answer 18

• Limited cutaneous systemic sclerosis (lcSSc)
• Diffuse cutaneous systemic sclerosis (dcSSc)

lcSSc is characterized by skin fibrosis affecting the peripheries, while dcSSc presents with widespread skin involvement.

Question 19

What does CREST syndrome stand for?

Answer 19

• Calcinosis
• Raynaud’s phenomenon
• Esophageal dysmotility
• Sclerodactyly
• Telangiectasia

CREST syndrome is associated with limited cutaneous systemic sclerosis (lcSSc).

Question 20

What is the prevalence of systemic sclerosis in the UK?

Answer 20

1 in 10,000

Women are three times more commonly affected than men.

Question 21

At what ages do the majority of cases of systemic sclerosis present?

Answer 21

between 30 and 50

Family history and silica exposure are also risk factors.

Question 22

What are common dermatological features of systemic sclerosis?

Answer 22

• Raynaud’s phenomenon
• Sclerodactyly
• Calcinosis
• Hyperpigmentation
• Telangiectasia

Skin may also become itchy and dry with hair loss.

Question 23

What is the ‘Prayer sign’ in systemic sclerosis?

Answer 23

Inability to bring the palmar surfaces of the hands together completely

This is due to restricted hand mobility.

Question 24

What are some gastrointestinal features of systemic sclerosis?

Answer 24

• Oesophageal dysmotility
• Delayed gastric emptying
• Gastric antral vascular ectasia (GAVE)
• Reduced bowel motility

These can lead to symptoms like dyspepsia, reflux, and malabsorption.

Question 25

What is the main **pulmonary manifestation** of systemic sclerosis?

Answer 25

Pulmonary arterial hypertension ## Footnote It is a leading cause of morbidity and mortality in systemic sclerosis.

Question 26

What are common **cardiovascular features** in diffuse cutaneous systemic sclerosis?

Answer 26

* Chest pain * Palpitations * Shortness of breath * Dizziness * Peripheral oedema ## Footnote Ischaemic heart disease may occur secondary to microvascular coronary artery disease.

Question 27

What is a serious complication of systemic sclerosis related to the kidneys?

Answer 27

Scleroderma renal crisis ## Footnote It can lead to acute and rapidly progressive renal failure.

Question 28

What are some **investigations** used for systemic sclerosis?

Answer 28

* Blood pressure monitoring * Urinalysis * Nailfold capillaroscopy * ECG * Blood tests for autoantibodies ## Footnote Imaging studies like chest X-ray and echocardiography are also important.

Question 29

What is the **first-line treatment** for skin fibrosis in early diffuse cutaneous systemic sclerosis?

Answer 29

Mycophenolate mofetil (MMF) ## Footnote Methotrexate is also considered.

Question 30

What lifestyle advice is important for managing **Raynaud's phenomenon**?

Answer 30

* Wearing gloves * Avoiding cold temperatures ## Footnote Smoking cessation is also crucial.

Question 31

What surgical management options may be considered for **digital ulcers**?

Answer 31

* Debridement * Digital sympathectomy * Botox injection ## Footnote These may be considered if medical management is insufficient.

Question 32

True or false: **Fatigue** is a common feature of systemic sclerosis.

Answer 32

TRUE ## Footnote Other features include erectile dysfunction and ocular involvement.

Question 33

Which antibody is seen in only cutaneous systemic sclerosis?

Answer 33

Anti-centromere antibodies

Question 34

How does limited cutaneous SCC present?

Answer 34

This form tends to present over a longer time period and is not associated with internal organ involvement (such as the lungs in this case) and tends to affect the distal limbs predominantly, rather than the proximal limbs

Question 35

Which antibody is only seen in diffuse SCC?

Answer 35

Anti-SCC-70 antiboides -> skin tightening over proximal limbs and evidence of interstitial lung disease (shortness of breath, dry cough, and crackles on auscultation

Question 36

What are the central antibodies involved in systemic sclerosis?

Answer 36

Central for anti-centromere

Question 37

Which body parts are affected in diffuse systemic sclerosis?

Answer 37

scleroderma affects trunk and proximal limbs predominately

Question 38

Which body parts are affected in limited cutaneous systemic sclerosis?

Answer 38

face and distal limbs predominately

Question 39

What causes difficulty with swallowing: limited or diffuse sclerosis?

Answer 39

Limited cutaneous sclerosis: oesophageal dysmotility linked to CREST syndrome