3: Systemic Sclerosis + Scleroderma Flashcards
What is scleroderma
Autoimmune skin disease characterised by progressive induration and hardening
Which gender are more likely to suffer from scleroderma
Females (3:1)
Which ethnicity are more likely to suffer from scleroderma
African American
Which age group are more likely to suffer from scleroderma
30-50y
What gene predisposes to limited scleroderma
STAT4
What gene predisposes to diffuse scleroderma
IRF5
What are 3 triggers for scleroderma
Sillicone
Radiation
Benzenes
What regions will be affected in limited scleroderma
Hands and Face
What is a subtype of limited scleroderma
CREST syndrome
What is CREST syndrome
Calcinosis Raynaud's Esophageal dysmotility Sclerodactylyl Telangectasia
What parts does diffuse scleroderma affect
All over. Commonly first affects the trunk and lower limbs. Also affects the internal organs.
What part is first affected in diffuse cutaneous scleroderma
Trunk and lower limbs
What is a sign of scleroderma within the mouth
- Microstomia (purse string appearance of the lips)
- Widening of the periodontal ligament space
How will the skin present in scleroderma
- Tightness, hardening
- Sclerodactylyl
- ‘Shiny’ - due to loss of wrinkles and skin folds
- Loss of mobility - due to contractures
What is an early sign of limited cutaneous scleroderma
Raynaud’s phenomenon
What antibody is associated with limited scleroderma
anti-centromere antibody
what antibody is associated with diffuse scleroderma
anti scl-70
what antibody is in 95% of patients with diffuse and limited scleroderma
anti-nuclear antibody
why may a CT be requested in scleroderma
Check for pulmonary involvement
what may be seen on CT in scleroderma and what does this indicate
Ground glass appearance - indicates lung fibrosis
why is an ECG requested in scleroderma
Look for cardiac involvement
How is scleroderma managed
Myclophenolate
Methotrexate
- Cyclophosphamide if refractory
When is an ACEi given in scleroderma
if renal involvement
When is nifedipine given in scleroderma
If raynaud’s phenomenon
what is the most common cause of death in diffuse cutaneous systemic sclerosis
pulmonary involvement - 80% is due to interstitial lung disease
what is the 5-year mortality of systemic sclerosis
35-75%
what are the 3 factors that are >80% accurate in predicting 5-year mortality of sytstemic sclerosis
- Proteinuria
- Raised ESR
- Low diffusing capacity for carbon monoxide
