Top 5 Important Lessons Learned in Scleroderma

On Thursday at EULAR there was an oral session on systemic sclerosis (SSc, scleroderma). Information from the EUSTAR database was presented by Dr. Elhai, et al (#OP0030) using 5 factors to predict mortality in SSc. Nearly 11,200 patients have been enrolled and 10% have died (60% of deaths from SSc). 

The predictive factors of increased risk of dying were: male (2 points), upper gastrointestinal involvement (2), proteinuria (5), LVEF<50% (3), and DLCO<60 % of predicted (2). Each increase in score by 1 point increased 5-year mortality by a third. 

This is not fully intuitive but it was highly predictive. For instance, is UGI involvement increasing the risk of death from aspiration and/or progression of ILD? Males always increase mortality in diseases and the general population, and heart involvement and/or pulmonary / pulmonary vasculature (i.e. low DLCO can be from either problem) all have face validity. Renal disease in the general population increases mortality, but proteinuria of clinical relevance I would have guessed is extremely low in SSc as renal crisis is uncommon (3% of SSc overall; 15% of early dcSSc).

Take home messages:

1. A simple score is predictive of mortality in SSc.

EUSTAR data also were used to find that positive RNA polermase-3 in older, diffuse SSc patients are particularly at risk for malignancies around the time of onset of SSc (Lazzaroni, et al. #OP0031)

2. RNA polymerase-3 is associated with more cancer in patients you think may have cancer with SSc onset (older, worse and progressive skin score).

In patients with GI involvement in SSc (i.e. the majority), there is a change in bowel flora which causes further immune stimulation in a small study (Luchetti, et al. #OP0032)

3. No idea if antibiotics or probiotics would help for small bowel overgrowth to improve the gut microbiome in SSc.

Our data from the Canadian Scleroderma Research Group found the mean annual European SSc disease activity score over 3 years of following incident patients is more predictive of future disease activity and damage than scoring it once or looking at those only with serial activity scores above a certain cut point (Navakyska, et al. #OP0034).

4. You can determine early who will have organ damage using serial disease activity scores and perhaps this can increase use of appropriate treatment for those are at higher risk of progression.

5. Dr O. Distler studied a serotonin receptor inhibitor (terguride) in SSc and found it may improve skin fibrosis but larger studies are needed.

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