scleroderma

see also:

• connective tissue (CT) disorders
• ENA (extractable nuclear antigen)
• autoimmune disease

• 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

• 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)

• 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
  • gastro-oesophageal reflux
  • SICCA (xerostomia and keratoconjunctivitis)
  • erectile dysfunction, dyspareunia
  • chronic renal failure
  • neurologic symptoms incl. trigeminal neuralgia, carpal tunnel, headaches, stroke, fatigue
• 5yr survival is 70% while 10 year survival is 55%