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Rheumatology > 3: Systemic Sclerosis + Scleroderma > Flashcards

3: Systemic Sclerosis + Scleroderma Flashcards

1
Q

What is scleroderma

A

Autoimmune skin disease characterised by progressive induration and hardening

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2
Q

Which gender are more likely to suffer from scleroderma

A

Females (3:1)

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3
Q

Which ethnicity are more likely to suffer from scleroderma

A

African American

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4
Q

Which age group are more likely to suffer from scleroderma

A

30-50y

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5
Q

What gene predisposes to limited scleroderma

A

STAT4

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6
Q

What gene predisposes to diffuse scleroderma

A

IRF5

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7
Q

What are 3 triggers for scleroderma

A

Sillicone
Radiation
Benzenes

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8
Q

What regions will be affected in limited scleroderma

A

Hands and Face

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9
Q

What is a subtype of limited scleroderma

A

CREST syndrome

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10
Q

What is CREST syndrome

A 
Calcinosis 
Raynaud's 
Esophageal dysmotility 
Sclerodactylyl 
Telangectasia
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11
Q

What parts does diffuse scleroderma affect

A

All over. Commonly first affects the trunk and lower limbs. Also affects the internal organs.

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12
Q

What part is first affected in diffuse cutaneous scleroderma

A

Trunk and lower limbs

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13
Q

What is a sign of scleroderma within the mouth

A
  • Microstomia (purse string appearance of the lips)

- Widening of the periodontal ligament space

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14
Q

How will the skin present in scleroderma

A
  • Tightness, hardening
  • Sclerodactylyl
  • ‘Shiny’ - due to loss of wrinkles and skin folds
  • Loss of mobility - due to contractures
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15
Q

What is an early sign of limited cutaneous scleroderma

A

Raynaud’s phenomenon

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16
Q

What antibody is associated with limited scleroderma

A

anti-centromere antibody

17
Q

what antibody is associated with diffuse scleroderma

A

anti scl-70

18
Q

what antibody is in 95% of patients with diffuse and limited scleroderma

A

anti-nuclear antibody

19
Q

why may a CT be requested in scleroderma

A

Check for pulmonary involvement

20
Q

what may be seen on CT in scleroderma and what does this indicate

A

Ground glass appearance - indicates lung fibrosis

21
Q

why is an ECG requested in scleroderma

A

Look for cardiac involvement

22
Q

How is scleroderma managed

A

Myclophenolate
Methotrexate

  • Cyclophosphamide if refractory
23
Q

When is an ACEi given in scleroderma

A

if renal involvement

24
Q

When is nifedipine given in scleroderma

A

If raynaud’s phenomenon

25
Q

what is the most common cause of death in diffuse cutaneous systemic sclerosis

A

pulmonary involvement - 80% is due to interstitial lung disease

26
Q

what is the 5-year mortality of systemic sclerosis

A

35-75%

27
Q

what are the 3 factors that are >80% accurate in predicting 5-year mortality of sytstemic sclerosis

A
  1. Proteinuria
  2. Raised ESR
  3. Low diffusing capacity for carbon monoxide

Rheumatology (10 decks)

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