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A Clinician's Perspective on New Treatment in Scleroderma
June 10 2016 11:28 AM ET — via RheumReports
Raynaud’s
Can a new oral prostacyclin receptor agonist (selexipag) help SSc Raynaud's?
Why did I ask this question? Treating Raynaud's phenomenon (RP) in SSc becomes problematic once CCBs and maybe losartan have failed (often due to lack of access of drugs). The next step is then PDE-5 inhibitors (with many but not all positive trials) or use of intravenous prostacyclin (iloprost) which has availability problems in North America (in both Canada and the US it is not approved for RP and has no DIN number). In Canada we can obtain it for our patients after government paper work is filed for each individual needing treatment. It can be given as IV daily for 5 days on an outpatient basis. So an effective oral prostacyclin would be preferred.
Answer: No.
A negative RCT of selexipag, an oral, selective, prostacyclin receptor agonist that has been used for pulmonary arterial hypertension, was presented by Denton et al (#FRI0265). The placebo controlled trial of SSc patients with active RP was negative since the selexipag and placebo groups had a similar reduction in the number of RP attacks.
Interstitial Lung Disease
What is the effect of cyclophosphamide on treatment of ILD according to the extent of ILD involvement? (i.e. Are there differential effects of cyclophosphamide when I treat extensive ILD compared to less extensive ILD?)
The answer is NO. The extent of ILD does not influence the effect of CYC.
Van Den Hombergh et al (Wieneke, #FRI0254) presented data from The Netherlands but only on 79 patients treated with pulse CYC for SSc-ILD, so the answer may be true but the study may be underpowered to detect differences.
Should I treat SSc-ILD with combination treatment (e.g. MMF with rituximab)?
Answer: I don't know.
Rituximab has sometimes been combined with immune suppressive drugs in the treatment of SSc-ILD (e.g. with CYC, AZA or MMF). A small, uncontrolled, Italian study of 18 patients with SSc-ILD seemed to show some benefit and good safety (Fraticelli, #FRI0255).
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About the Author
Dr. Janet Pope
Dr. Janet Pope is Professor of Medicine at Western University and Division Head of Rheumatology. Dr. Pope's research interests include epidemiologic studies in scleroderma, classification criteria in systemic sclerosis, systemic lupus erythematosus and rheumatoid arthritis.
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