Systemic sclerosis Flashcards
Types of systemic sclerosis
Frequency
diffuse cutaneous
limited cutaneous (3x more common)
SSc
- sex frequency
- peak incidence
- leading cause of death
- rank in mortality of rheumatic diseases
F:M 3:1
peak incidence 40-60 y.o
cardiopulmonary mortality
highest mortality among rheumatic diseases
Genetic associations of SSc
HLA-DR1, STAT4, interferon
Hallmarks of SSc
Autoimmunity
Vasculopathy
Interstitial fibrosis
Antibodies associated with
SSc
Diffuse cutaneous SSc
- Scl-70 (anti-topoisomerase 1)
- Anti-RNA polymaerase 1 / 3 antibodies
- ANA with nucleolar pattern
Limited cutaneous SSc
- Anticentromere
Scl-70 less common
Difference between disease pattern in diffuse and limited disease
diffuse disease is characterised by early internal organ involvement including renal crisis, involvement of skin on trunk
limited disease often has protracted history of raynauds, skin disease confined to upper limbs and face
SSc-ILD
- ILD pattern
- how to grade severity
- treatment
Non-specific interstitial pneumonitis (NSIP) more common than Usual interstitial pneumonitis (UIP) pattern
- grade severity on basis of FVC, DLCO, and HRCT
- treatment: treat GORD, vaccinations, quit smoking
- Pharmacotherapy: mycophenolate 3g/day, cyclophosphamide
- Other agents: ?nintedanib, pirfenidone, rituximab, tocilizumab, lung transplant, autologous stem cell transplant?
NINTEDANIB: reduced rate of fall in FVC, unclear if clinically
Features of NSIP
ground glass appearance with fine fibrosis
Features of UIP
peripheral reticulo-nodular opacities with honey-combing and traction bronchiectasis
SSc-PAH
- antibodies associated with higher risk
Anti-U3 RNP or nucleolar Ab
PAH screening
Screening:
1. DLCO <70% predicted with FVC/DLCO ratio>1.8
2. NT-proBNP > 210pg/mL
If either present should refer patient for right heart catheter
If negative repeat in 6-12 months
What is the sensitivity and specificity of ECHO for pulmonary hypertension?
What percentage of people cannot have PAP estimated?
Sens 88%, Spec 83%
Up to 39% of patients cannot have PAP estimated
Are usual PAH therapies effective in SSc PAH?
usually less effective
Scleroderma renal crisis
- features
- precipitants
- treatment
rapid onset, hyper-reninaemic, associated with hypertension
precipitated by glucocorticoids
treatment with ACEi
Raynauds
treatments
non-pharm - gloves, keep warm
pharm - CCB (nifedipine / amlo), topical nitrates
