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scleroderma

scleroderma

introduction

  • scleroderma is a autoimmune connective tissue disorder characterised by “hardening” of the skin +/- internal organs
  • there is currently no curative treatment for the disease itself

limited scleroderma

  • features mainly limited to skin and oesophagus, formerly called CREST:
    • Calcinosis (the deposition of calcium nodules in the skin)
    • Raynaud's phenomenon
    • Esophageal dysfunction
    • Sclerodactyly (skin thickening on the fingers)
    • Telangiectasias (dilated capillaries on the face, hands and mucous membranes)

diffuse scleroderma

  • rapidly progressive severe form involving large areas of skin and end organs which may cause:
    • dry, persistent cough due to interstitial lung disease
    • progressive shortness of breath and/or chest pain due to pulmonary artery hypertension
    • cardiac conduction issues
    • hypertension
    • cardiac failure
    • SICCA (xerostomia and keratoconjunctivitis)
    • erectile dysfunction, dyspareunia
    • neurologic symptoms incl. trigeminal neuralgia, carpal tunnel, headaches, stroke, fatigue
  • 5yr survival is 70% while 10 year survival is 55%
scleroderma.txt · Last modified: 2015/12/29 15:10 (external edit)