All::Rheumatology::Diseases::Systemic sclerosis
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What is Systemic sclerosis?
a condition of unknown aetiology characterised by hardened, sclerotic skin and other connective tissues
Systemic sclerosisWhat patient groups is Systemic sclerosis most common?
females (4:1)
Systemic sclerosisWhat antibodies are found in Systemic sclerosis?
ANA positive in 90% RF positive in 30%
Systemic sclerosisWhat is Scleroderma?
tightening and fibrosis of skin
Systemic sclerosisLimited cutaneous systemic sclerosis
What is often the first sign of Limited cutaneous systemic sclerosis?
Raynaud's
Systemic sclerosisWhat parts of the body is effected by scleroderma in Limited cutaneous systemic sclerosis?
affects face and distal limbs predominately
Systemic sclerosisWhat antibodies are associated with Limited cutaneous systemic sclerosis?
anti-centromere antibodies
Systemic sclerosisWhat is CREST syndrome?
- a subtype of limited systemic sclerosis
- Calcinosis, Raynaud's phenomenon, oEsophageal dysmotility, Sclerodactyly, Telangiectasia
Diffuse cutaneous systemic sclerosis
What parts of the body is effected by scleroderma in Diffuse cutaneous systemic sclerosis?
trunk and proximal limbs predominately
Systemic sclerosisWhat antibodies are associated with Diffuse cutaneous systemic sclerosis?
anti scl-70 antibodies
Systemic sclerosisWhat respiratory complications are seen in Diffuse cutaneous systemic sclerosis?
interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH)
Systemic sclerosisWhat non-respiratory complications can develop in Diffuse cutaneous systemic sclerosis?
renal disease and hypertension
Systemic sclerosisWhat is the prognosis in Diffuse cutaneous systemic sclerosis?
poor
Systemic sclerosis