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BPT Rheumatology > Systemic sclerosis > Flashcards

Systemic sclerosis Flashcards

1
Q

What is the peak onset age?

A

40-60

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2
Q

What are the 2 main subsets?

A

Limited cutaneous 3:1 Diffuse cutaneous

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3
Q

What environmental agents are associated?

A

Silica
Organic solvents
Vinyl chloride

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4
Q

What the three components of pathogenesis?

A

Inflammation
Vasculopathy
Interstitial fibrosis - skin, lungs, GIT, renal, myocardium

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5
Q

What is hte pattern of disease in diffuse cutaneous SSc?

A

Raynauds
Rapid onset skin changes
Tendon friction rubs
Early onset of internal organ involvement

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6
Q

What abs are associated with dSSc?

A

scl-70
Anti-RNA polymerase I and III
ANA with nucleolar pattern

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7
Q

What is the pattern of disease with limited cutaneous SSc

A

Raynauds over years
Gradual onset of skin changes
Calcinosis, telangiectasia
Oesophageal dysmotility

  • interstitial lung, cardiac, and renal disease more rare
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8
Q

Which disease pattern was formally known as CREST?

A

Limited cutaneous SSc

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9
Q

What abs are associated with lSSc?

A

Anti-centromere ab

lower incidence of Scl-70

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10
Q

How prevalent is pul HTN?

A

10% in both

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11
Q

What is the reduced life expectancy?

A

23 in women

26 in men

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12
Q

What is the most common causes of mortality?

A

Pul fibrosis and HTN

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13
Q

What are the clinical features of skin thickening

A

Skin thickening

Contractures

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14
Q

What is the most common pattern of ILD?

A

NSIP more common than UIP

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15
Q

What are the high risk phenotypes for SSc-ILD?

A

Early dSSC and anti-Scl70

Early dSSC and elevated CRP

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16
Q

When FVC decline most rapidly?

A

In the first 5 years

17
Q

How often should you do RFTs in early disease?

A

Every 3-5 months in first 3-5 years

18
Q

What are criteria for clinically significant ILD that would prompt treatment?

A

Minimal to severe ILD on HRCT

FVC or DLCO

19
Q

What is the first and second line therapy for ILD?

A

Mycophenolate

Next - Oral or IV cyclophosphamide

20
Q

Which novel anti-fibrotic is evidence based?

A

Nintedanib - Tyrosine kinase inhibitor

21
Q

What are other causes resp symptoms in SSc?

A 
Neuromuscular dysfunction - myositis
Thoracic restrction - scleroderma
Aspiration - due to reflux
Deconditioning
Pul HTN
22
Q

What is the mechanism of Pul HTN in SSc?

A

Primary pulmonary arteriole vaculopathy

But also L heart disease, ILD, chronic PE (anti-phospholipid abs mediated). and Pulmonary veno-occlusive disease

23
Q

What is the R heart catheter cut off for mean pul art pressure?

A

> 20mmHg

24
Q

What is limitation of TTE in screening?

A

40% don’t have sPAP as not TR jet

25
Q

What is the annual screening for pul HTN

A

RFT
NT-proBNP

If either positive - right heart catheter

26
Q

What Rx options are there for pul HTN?

A

ERA endothelin receptor antagonist
PDE-5 inhibitor
Riociguat
Continuous IV epoprostenol

27
Q

What is scleroderma renal crisis?

A

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

28
Q

How do you treat SRC?

A

BP control with ACEi

29
Q

What is the mortality of SRC?

A

25-35%

30
Q

What is the mx of raynauds

A

Keep warm

Calcium channel blockers
PDE5 inhibitors
Topical or systemic nitrates

IV prostacyclin if severe
Botox

31
Q

What is the mx of digital ulcers

A

Similar to raynauds

IV Prostacyclin

32
Q

What are the GIT manifestations?

A 
Oral rigidity
Sicca symptoms 
Reflux 
Bloating, distension 
Bacterial overgrowth 
Diarrhoea/constipation
Faecal incontinence
33
Q

When is HSCT considered?

A

Patients with early progressive SSc at risk of organ failure

BPT Rheumatology (17 decks)

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