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Scleroderma

 
 
 
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Overview

Scleroderma is also known as systemic sclerosis.
  • Ask About

  • 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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