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Systemic sclerosis (scleroderma)

Sclerodermia systemica · Systémová sklerodermie

What it is

Fibrosis of skin and internal organs, driven by immune activation and vascular injury.

Symptoms & signs

  • Tight thickened skin
  • Raynaud's
  • reflux
  • breathlessness from lung fibrosis
  • kidney crisis
  • telangiectasia

🚩 Get help if…

  • call 112 / 911 now Sudden severe headache, fits or confused thinking with high blood pressure
  • call 112 / 911 now Fingertip that turns black or very painful and does not recover
  • see a doctor soon Breathlessness on effort or a dry cough that is getting worse
  • see a doctor soon Passing little urine or ankles swelling

Where it strikes

Medical treatment

  • Mycophenolate or nintedanib for lung fibrosis
  • ACE inhibitors for renal crisis
  • calcium blockers for Raynaud's
  • PPIs for reflux
  • autologous stem-cell transplant in severe disease
  • Tocilizumab — FDA-approved 2021 to slow lung decline in scleroderma lung disease

Natural & lifestyle

  • Keep warm and stop smoking (both critical for Raynaud's)
  • skin emollients
  • hand therapy
  • small frequent meals

Emerging & experimental

  • CAR-T therapy case series

Alternative, off-label & anecdotal

Approaches used outside the guidelines, with what their supporters report and what testing has found so far.

  • Vitamin E gel on finger ulcers — a small study of faster healing early / small studies
  • Anti-inflammatory and elimination diets — anecdotal anecdotal / traditional

ICD codes