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MSK History
 

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
Last updated on July 18th, 2020
 
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