Everything you always wanted to know about scleroderma but were afraid to ask

We’ve had some great learning this month during the ditties about scleroderma. A few folks asked questions I promised to answer in pearls. So in honor of the end/near end of rheum month, here we go =)
Scleroderma is rare and morbid
  • prevalence: ~1 per 10,000 people
  • diffuse systemic sclerosis is extremely morbid. Its has a 53% 10 year mortality, giving it only a somewhat better prognosis than early stage lung cancer.
  • While you may diagnose scleroderma only a couple times in your career (unless you are a rheumatologist) we see a lot of these patients because they are so sick.
Classifying systemic sclerosis and scleroderma
Big picture scleroderma thinking – check out the classifications in the diagram below. Era drew this beautifully on the board, I tried to recreate it in powerpoint =)
screen-shot-2017-02-24-at-11-55-19-am
More detail on organ-specific manifestations
  • The “limited” and “diffuse” monikers refer to the skin manifestations. Unfortunately, people with both kinds of scleroderma can have terrible organ manifestations.
  • Check out this table from an NEJM review on scleroderma about the prevalence of each organ manifestation stratified by scleroderma type.
screen-shot-2017-02-27-at-11-10-31-am
Antibodies?!?!
  • This is super complex! Most (89-97%) scleroderma patients are ANA positive.
  • anti-centromere antibodies are associated with limited scleroderma
  • anti-topoisomerase antibodies are associated with diffuse scleroderma
  • There are TONS of other scleroderma associated antibodies that portend involvement of various organs. see the table below for details.
screen-shot-2017-02-27-at-10-30-29-am
A word on scleroderma renal crisis
There are many renal manifestations of scleroderma. Renal crisis has three components
  • renal failure
  • sudden, severe hypertension, sometimes with associated MAHA or other manifestations of hypertensive emergency like PRES
  • bland urine sediment or isolated proteinuria (unless there is associated MAHA)
Risk factors
  • Much more common in diffuse systemic sclerosis and people w extensive skin involvement
  • Usually happens EARLY in disease course – mean 7.5 months after diagnosis.
  • recent glucocorticoid use
    • more reason to get these folks to a specialist EARLY for disease modifying therapy.
Treatment
  • Call rheum!
    • People with scleroderma can get all sorts of other types of AKI. So also do your usual AKI workup.
  • Ace inhibitors
    • have survival benefit and dialysis-free survival benefit in scleroderma renal crisis.
  • Other things
    • some evidence for plasma exchange
    • early evidence for bosentan and IV prostacyclines
Citations
Nihtyanova SI1Denton CP. Autoantibodies as predictive tools in systemic sclerosis. Nat Rev Rheumatol. 2010 Feb;6(2):112-6.
 
Gabrielli A1Avvedimento EVKrieg T.Scleroderma N Engl J Med. 2009 May 7;360(19):1989-2003.
 
And many up to date articles
Evernote: https://www.evernote.com/l/AMDiPgnHVFpAUrUU1uXRXzywESDvy672_Ts
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