Systemic sclerosis Flashcards
What is the peak onset age?
40-60
What are the 2 main subsets?
Limited cutaneous 3:1 Diffuse cutaneous
What environmental agents are associated?
Silica
Organic solvents
Vinyl chloride
What the three components of pathogenesis?
Inflammation
Vasculopathy
Interstitial fibrosis - skin, lungs, GIT, renal, myocardium
What is hte pattern of disease in diffuse cutaneous SSc?
Raynauds
Rapid onset skin changes
Tendon friction rubs
Early onset of internal organ involvement
What abs are associated with dSSc?
scl-70
Anti-RNA polymerase I and III
ANA with nucleolar pattern
What is the pattern of disease with limited cutaneous SSc
Raynauds over years
Gradual onset of skin changes
Calcinosis, telangiectasia
Oesophageal dysmotility
- interstitial lung, cardiac, and renal disease more rare
Which disease pattern was formally known as CREST?
Limited cutaneous SSc
What abs are associated with lSSc?
Anti-centromere ab
lower incidence of Scl-70
How prevalent is pul HTN?
10% in both
What is the reduced life expectancy?
23 in women
26 in men
What is the most common causes of mortality?
Pul fibrosis and HTN
What are the clinical features of skin thickening
Skin thickening
Contractures
What is the most common pattern of ILD?
NSIP more common than UIP
What are the high risk phenotypes for SSc-ILD?
Early dSSC and anti-Scl70
Early dSSC and elevated CRP
When FVC decline most rapidly?
In the first 5 years
How often should you do RFTs in early disease?
Every 3-5 months in first 3-5 years
What are criteria for clinically significant ILD that would prompt treatment?
Minimal to severe ILD on HRCT
FVC or DLCO
What is the first and second line therapy for ILD?
Mycophenolate
Next - Oral or IV cyclophosphamide
Which novel anti-fibrotic is evidence based?
Nintedanib - Tyrosine kinase inhibitor
What are other causes resp symptoms in SSc?
Neuromuscular dysfunction - myositis Thoracic restrction - scleroderma Aspiration - due to reflux Deconditioning Pul HTN
What is the mechanism of Pul HTN in SSc?
Primary pulmonary arteriole vaculopathy
But also L heart disease, ILD, chronic PE (anti-phospholipid abs mediated). and Pulmonary veno-occlusive disease
What is the R heart catheter cut off for mean pul art pressure?
> 20mmHg
What is limitation of TTE in screening?
40% don’t have sPAP as not TR jet
What is the annual screening for pul HTN
RFT
NT-proBNP
If either positive - right heart catheter
What Rx options are there for pul HTN?
ERA endothelin receptor antagonist
PDE-5 inhibitor
Riociguat
Continuous IV epoprostenol
What is scleroderma renal crisis?
Hyper-reninemic, rapidly progressive renal impairment characterised by abrupt onset of mod to severe HTN, normal urine sediment or only mild proteinuria, progressive renal failure
How do you treat SRC?
BP control with ACEi
What is the mortality of SRC?
25-35%
What is the mx of raynauds
Keep warm
Calcium channel blockers
PDE5 inhibitors
Topical or systemic nitrates
IV prostacyclin if severe
Botox
What is the mx of digital ulcers
Similar to raynauds
IV Prostacyclin
What are the GIT manifestations?
Oral rigidity Sicca symptoms Reflux Bloating, distension Bacterial overgrowth Diarrhoea/constipation Faecal incontinence
When is HSCT considered?
Patients with early progressive SSc at risk of organ failure
