What is Systemic sclerosis?

a condition of unknown aetiology characterised by hardened, sclerotic skin and other connective tissues

What patient groups is Systemic sclerosis most common?

females (4:1)

What antibodies are found in Systemic sclerosis?

ANA positive in 90% RF positive in 30%

What is Scleroderma?

tightening and fibrosis of skin

What is often the first sign of Limited cutaneous systemic sclerosis?

Raynaud's

What parts of the body is effected by scleroderma in Limited cutaneous systemic sclerosis?

affects face and distal limbs predominately

What antibodies are associated with Limited cutaneous systemic sclerosis?

anti-centromere antibodies

What is CREST syndrome?

• a subtype of limited systemic sclerosis
• Calcinosis, Raynaud's phenomenon, oEsophageal dysmotility, Sclerodactyly, Telangiectasia

What parts of the body is effected by scleroderma in Diffuse cutaneous systemic sclerosis?

trunk and proximal limbs predominately

What antibodies are associated with Diffuse cutaneous systemic sclerosis?

anti scl-70 antibodies

What respiratory complications are seen in Diffuse cutaneous systemic sclerosis?

interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH)

What non-respiratory complications can develop in Diffuse cutaneous systemic sclerosis?

renal disease and hypertension

What is the prognosis in Diffuse cutaneous systemic sclerosis?

poor