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