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 are some risk factors for systemic sclerosis?

Answer 4

• Family history
• Silica exposure

These factors may increase the likelihood of developing the disease.

Question 5

True or false: Raynaud’s phenomenon often occurs at the same time as other features in lcSSc.

Answer 5

FALSE

In lcSSc, Raynaud’s phenomenon often predates other features by many years.

Question 6

What are some dermatological features of systemic sclerosis?

Answer 6

• Inflammation and puffiness of fingers and toes
• Hardening and thickening of the skin (sclerodactyly)
• Atrophy of fingertips with ulceration
• Hyperpigmentation and shiny skin
• Calcinosis

These features vary between lcSSc and dcSSc.

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 the gastrointestinal features of systemic sclerosis?

Answer 8

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

These features can lead to various digestive issues.

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 some cardiovascular features of systemic sclerosis?

Answer 10

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

These symptoms may occur as the disease progresses.

Question 11

What is scleroderma renal crisis?

Answer 11

Acute and rapidly progressive renal failure often associated with severe hypertension

It occurs due to severe intrarenal vasoconstriction leading to renal ischaemia.

Question 12

What are some bedside tests for systemic sclerosis?

Answer 12

• Blood pressure monitoring
• Urinalysis for proteinuria
• Nailfold capillaroscopy

These tests help assess hypertension and renal involvement.

Question 13

What are some blood tests used in systemic sclerosis diagnosis?

Answer 13

• Full blood count
• U&Es for renal function
• Liver function tests
• ESR and CRP

These tests help evaluate overall health and detect complications.

Question 14

What do anticentromere antibodies indicate?

Answer 14

Specific to lcSSc and associated with pulmonary hypertension

They are useful for diagnosis and predicting organ-specific complications.

Question 15

What is the gold standard investigation for diagnosing pulmonary arterial hypertension?

Answer 15

Right heart catheterisation

It provides definitive measurements of pulmonary artery pressures.

Question 16

What is the first-line management for scleroderma renal crisis?

Answer 16

ACE inhibitors

Other antihypertensives may also be required.

Question 17

What are some overlap syndromes associated with scleroderma?

Answer 17

• Myositis
• Rheumatoid arthritis
• Sjögren’s syndrome
• Systemic lupus erythematosus

These conditions may require additional management.

Question 18

What is the third commonest cause of death in systemic sclerosis?

Answer 18

Malignancy

Breast, lung, skin, and haematological cancers are the most common.

Question 19

What is the treatment for upper GI bleeding due to GAVE?

Answer 19

Endoscopic laser coagulation

This may be utilized to treat or prevent bleeding.

Question 20

What is a common complication of systemic sclerosis during pregnancy?

Answer 20

High risk situations include pulmonary arterial hypertension and severe organ involvement

Pregnancies should be planned for when the disease is stable.

Question 21

What is systemic sclerosis also known as?

Answer 21

scleroderma

It is a rare chronic autoimmune disease of unknown cause.

Question 22

Name the two main subtypes of systemic sclerosis.

Answer 22

• 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 23

What does CREST syndrome stand for?

Answer 23

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

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

Question 24

What are some risk factors for systemic sclerosis?

Answer 24

• Family history
• Silica exposure

These factors may increase the likelihood of developing the disease.

Question 25

True or false: **Raynaud's phenomenon** often occurs at the same time as other features in lcSSc.

Answer 25

FALSE ## Footnote In lcSSc, Raynaud's phenomenon often predates other features by many years.

Question 26

What are some **dermatological features** of systemic sclerosis?

Answer 26

* Inflammation and puffiness of fingers and toes * Hardening and thickening of the skin (sclerodactyly) * Atrophy of fingertips with ulceration * Hyperpigmentation and shiny skin * Calcinosis ## Footnote These features vary between lcSSc and dcSSc.

Question 27

What is the **'Prayer sign'** in systemic sclerosis?

Answer 27

Inability to bring the palmar surfaces of the hands together completely ## Footnote This is due to restricted hand mobility.

Question 28

What are the **gastrointestinal features** of systemic sclerosis?

Answer 28

* Oesophageal dysmotility * Delayed gastric emptying * Gastric antral vascular ectasia (GAVE) * Reduced small and large bowel motility ## Footnote These features can lead to various digestive issues.

Question 29

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

Answer 29

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

Question 30

What are some **cardiovascular features** of systemic sclerosis?

Answer 30

* Chest pain * Palpitations * Shortness of breath * Dizziness * Peripheral oedema ## Footnote These symptoms may occur as the disease progresses.

Question 31

What is **scleroderma renal crisis**?

Answer 31

Acute and rapidly progressive renal failure often associated with severe hypertension ## Footnote It occurs due to severe intrarenal vasoconstriction leading to renal ischaemia.

Question 32

What are some **bedside tests** for systemic sclerosis?

Answer 32

* Blood pressure monitoring * Urinalysis for proteinuria * Nailfold capillaroscopy ## Footnote These tests help assess hypertension and renal involvement.

Question 33

What are some **blood tests** used in systemic sclerosis diagnosis?

Answer 33

* Full blood count * U&Es for renal function * Liver function tests * ESR and CRP ## Footnote These tests help evaluate overall health and detect complications.

Question 34

What do **anticentromere antibodies** indicate?

Answer 34

Specific to lcSSc and associated with pulmonary hypertension ## Footnote They are useful for diagnosis and predicting organ-specific complications.

Question 35

What is the **gold standard investigation** for diagnosing pulmonary arterial hypertension?

Answer 35

Right heart catheterisation ## Footnote It provides definitive measurements of pulmonary artery pressures.

Question 36

What is the **first-line management** for scleroderma renal crisis?

Answer 36

ACE inhibitors ## Footnote Other antihypertensives may also be required.

Question 37

What are some **overlap syndromes** associated with scleroderma?

Answer 37

* Myositis * Rheumatoid arthritis * Sjögren's syndrome * Systemic lupus erythematosus ## Footnote These conditions may require additional management.

Question 38

What is the **third commonest cause of death** in systemic sclerosis?

Answer 38

Malignancy ## Footnote Breast, lung, skin, and haematological cancers are the most common.

Question 39

What is the **treatment** for upper GI bleeding due to GAVE?

Answer 39

Endoscopic laser coagulation ## Footnote This may be utilized to treat or prevent bleeding.

Question 40

What is a common **complication** of systemic sclerosis during pregnancy?

Answer 40

High risk situations include pulmonary arterial hypertension and severe organ involvement ## Footnote Pregnancies should be planned for when the disease is stable.

Question 41

Qhixh antibody is indicated in systemic csclerosis?

Answer 41

Anti-centromere antibody

Question 42

Which antibody is found in diffuse systemic sclerosis?

Answer 42

Anti-RNA polymerase

Question 43

How to differentiate limited and diffuse systemic sclerosis?

Answer 43

Limited systemic sclerosis primarily affects the face, hands, and forearms, sparing the upper arms and trunk Diffuse sclerosis extends beyond the elbows

Question 44

What antibody is present in diffuse systemic sclerosis?

Answer 44

Anti-RNA polymerase antibody

Question 45

Which antibody is seen in diffuse cutaneous systemic sclerosis?

Answer 45

Anti topoisomerase I antibodies

Question 46

How does diffuse cutaneous systemic sclerosis present?

Answer 46

present with sclerodactyly, Reynaud's phenomenon, pulmonary fibrosis, and skin thickening

Question 47

What does history of scleroderma with severe hypertension raise complication risk for?

Answer 47

Scleroderma renal crisis, hypertensive retinopathy, hypertensive encephalopathy

Question 48

How to differentiate central and systemic sclerosis?

Answer 48

Involvement of the chest and upper arms

Question 49

What indicates systemic features of systemic sclerosis?

Answer 49

Efectile dysfunction Respiratory involvement Anti SCL-70 antibodies

Question 50

What is the most common cause of death in Diffuse cutaneous systemic sclerosis?

Answer 50

Respiratory involvement

Question 51

Which organ can be implicated in diffuse cutaneous sclerosis,

Answer 51

Renal disease ->should be started on an ACE inhibitor - captopril is typically used due to its rapid onset and short half-life, allowing for dose titration

Question 52

What is a Lung complication of systemic sclerosis?

Answer 52

Pulmonary hypertension presents as right heart failure with exertional dyspnoea, fatigue and weakness are the primary symptoms, accompanied by signs of right heart failure