Overview
Scleroderma is also known as systemic sclerosis.
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- Diagnosis - when scleroderma was diagnosed, presenting symptoms, limited or diffuse
- Manifestations - cardiovascular, GI, respiratory, renal, MSK, neurological
- Functional impairment
- Management
Types of Scleroderma
- Limited scleroderma (CREST syndrome) - slowly progressive; less associated with cardiac disease and renal crisis
- Diffuse scleroderma - rapidly progressive; associated with lung disease, renal crisis and cardiac disease
Manifestations
Manifestations of CREST Syndrome
- Calcinosis cutis: hard masses beneath the skin, due to calcium deposition
- Raynaud's phenomenon: areas of white, blue or red change in the hands
- (O)Esophageal dysmotility
- Sclerodactyly: thickening of the fingers / toes
- Telangiectasia: visible dilated blood vessels
Manifestations of Scleroderma
- Cardiovascular - Raynaud's phenomenon, telangiectasias, pulmonary hypertension, pericarditis, arrhythmias, erectile dysfunction
- Gastrointestinal - oesophageal dysmotility, reflux, GI bleeding, bacterial overgrowth
- Respiratory - interstitial fibrosis, aspiration pneumonia
- Renal - scleroderma renal crisis
- Musculoskeletal - sclerodactyly, calcinosis cutis, tendon fibrosis
- Neurological - entrapment neuropathies (e.g. carpal tunnel), autonomic neuropathy
Management
There is no specific treatment available for scleroderma, and therefore treatment is aimed at addressing organ involvement.
Management Options
- Raynaud's - cold avoidance, smoking cessation, calcium channel blockers, topical nitrates, PDE5 inhibitors, prostacyclin
- Skin thickening - physiotherapy, immunosuppression
- Reflux - proton pump inhibitors
- Interstitial lung disease - immunosuppression
- Pulmonary arterial hypertension - PDE5 inhibitors, endothelin antagonists, prostacyclin analogues
- Renal crisis - ACE inhibitors, dialysis
Advanced Options
- Autologous stem cell transplant
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