Systemic sclerosis

Systemic sclerosis is a condition of unknown aetiology characterised by hardened, sclerotic skin and other connective tissues. It is four times more common in females.

There are three patterns of disease:

Limited cutaneous systemic sclerosis

Raynaud's may be the first sign
scleroderma affects face and distal limbs predominately
associated with anti-centromere antibodies
a subtype of limited systemic sclerosis is CREST syndrome: Calcinosis, Raynaud's phenomenon, oEsophageal dysmotility, Sclerodactyly, Telangiectasia

Diffuse cutaneous systemic sclerosis

scleroderma affects trunk and proximal limbs predominately
associated with anti scl-70 antibodies
the most common cause of death is now respiratory involvement, which is seen in around 80%: interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH)
other complications include renal disease and hypertension
- patients with renal disease should be started on an ACE inhibitor - captopril is typically used due to its rapid onset and short half-life, allowing for dose titration
- ACE inhibitors target the underlying mechanism by reducing efferent arteriolar vasoconstriction and limiting renin-angiotensin system activation
poor prognosis

Scleroderma (without internal organ involvement)

tightening and fibrosis of skin
may be manifest as plaques (morphoea) or linear

Image used on license from DermNet NZ

Antibodies

ANA positive in 90%
RF positive in 30%
anti-scl-70 antibodies associated with diffuse cutaneous systemic sclerosis
- associated with a higher risk of severe interstitial lung disease
anti-centromere antibodies associated with limited cutaneous systemic sclerosis

+PassMedicine Notes

Systemic sclerosis is a condition of unknown aetiology characterised by hardened, sclerotic skin and other connective tissues. It is four times more common in females.

There are three patterns of disease:

Limited cutaneous systemic sclerosis

Raynaud's may be the first sign
scleroderma affects face and distal limbs predominately
associated with anti-centromere antibodies
a subtype of limited systemic sclerosis is CREST syndrome: Calcinosis, Raynaud's phenomenon, oEsophageal dysmotility, Sclerodactyly, Telangiectasia

Diffuse cutaneous systemic sclerosis

scleroderma affects trunk and proximal limbs predominately
associated with anti scl-70 antibodies
the most common cause of death is now respiratory involvement, which is seen in around 80%: interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH)
other complications include renal disease and hypertension

patients with renal disease should be started on an ACE inhibitor - captopril is typically used due to its rapid onset and short half-life, allowing for dose titration
ACE inhibitors target the underlying mechanism by reducing efferent arteriolar vasoconstriction and limiting renin-angiotensin system activation