MY ACR2014 Favorites: Systemic Sclerosis Advances and a Challenge


(ACR2014): This member of the Rheumatology Network Editorial Board liked two promising presentations about systemic sclerosis, and another that raised some provocative questions about which doctors we like to listen to.

Castelino FV, George LM, Bain G et al.Autotaxin Is Highly Expressed in Systemic Sclerosis (SSc) Skin, Mediates Dermal Fibrosis Via IL-6, and Is a Target for SSc Therapy. Abstract #1796. 2014;66:S1306. Abstract Supplement, 2014 ACR/ARHP Annual Meeting

Khanna D, Denton CP, Jagerschmit A et al.SAR100842, an Antagonist of Lysophaphatidic Acid Receptor 1, As a Potential Treatment for Patients with Systemic Sclerosis: Results from a Phase 2a Study. Abstract #876. 2014;66:S1306. Abstract Supplement, 2014 ACR/ARHP Annual Meeting

Autotaxin is responsible for production of lysophosphatidic acid (LPA), which is implicated in scleroderma pathogenesis.

In the first report above, using a murine model of dermal fibrosis, an inhibitor of autotaxin attenuated dermal fibrosis and IL-6 expression. Autotaxin expression was increased in skin from systemic sclerosis patients, compared to healthy controls. The authors concluded that targeting autotaxin may be a therapeutic strategy for scleroderma fibrosis.

Combining this information with the second report above, describing efficacy of an oral antagonist of the LPA receptor in a Phase 2a study of systemic sclerosis patients, I offered substituting tocilizumab for adalimumab to my patient with an overlap syndrome [RF/CCP+ RA, primary Sjogren syndrome (+ANA/SSA/SSB) and recent progression of morphea].

She shares my hope that this will prove beneficial.

Another report about systemic sclerosis caught my eye:

Bernstein EJ, Peterson ER, Bathon JM and Lederer DJ.  One-Year Survival of Adults with Systemic Sclerosis Following Lung Transplantation: A Nationwide Cohort Study. Abstract #1797. 2014;66:S1306. Abstract Supplement, 2014 ACR/ARHP Annual Meeting

Bernstein et al studied the one-year survival rate of adults with systemic sclerosis and end-stage lung disease due to interstitial lung disease (ILD) and/or pulmonary hypertension (PAH) following lung transplantation. These patients had a 48% higher risk of death at 1 year than adults with ILD not due to systemic sclerosis, but no increased risk compared to adults with (PAH) at 1 year. If patients with systemic sclerosis made it past 1 year, there was a slight decrease in mortality. This shows promise for risk stratification of those systemic sclerosis patients who might benefit from lung transplantation.


... and a celebrity challenge for teachers (and doctors?) of medicine:

While we're choosing "favorites," a presentation at a non-clinical session this year raised some interesting questions about the real criteria we use to judge our sources of clinical information.

Each year ACR invites the latest winner of the Charles H. Griffith III Educational Research Award, named by the Clerkship Directors in Internal Medicine, to present the Medical Education Year in Review at the annual meeting. The 2013 award winner, my colleague Andrew Hoellein, MD, presented one paper that got a lot of attention because of its implications .

Rannelli et al (How do medical students form impressions of the effectiveness of classroom teachers? Med Ed. 2014;48:831-837) identified  six categories  of teaching attributes encompassing 30 adjectives. Two of the principal components that medical students appraise when rating the effectiveness of a classroom teacher "won" this contest for enhancing memorability in the classroom: charisma and intellect. 

Only one of these two, charisma (not intellect!), was associated with teaching effectiveness ratings.

Those with little personal magnetism but an appealing visage can take heart: An attractive photo lessened the negative effect of a low charisma rating on teaching effectiveness.

To what degree are teaching awards and promotions based on these subjective factors in rating teaching effectiveness? The authors raised the question.

And should we also studythese factors in relation to Best Doctor Awards?

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