AB0703 Prostanoids for Treatment of Secondary Raynaud's Phenomenon: Results From a Prospective Observational Single-Centre Cohort Study

Abstract

BackgroundRaynaud's phenomenon (RP) secondary to rheumatic diseases (RD) is associated with significant morbidity (e.g., ulcerations and gangrene).1 Three groups of specific drugs have been marketed for RP (prostanoids, ERAs and PDE-5 inhibitors).2ObjectivesTo evaluate the comparative efficacy and safety of two prostanoids (intravenous iloprost and alprostadil) in treatment of secondary RP.MethodsIn 2013 and 2014, all 25 inpatients at the regional clinic with secondary RP were treated with prostanoids [23 females; mean (SD) age 47.5 (10.2) yrs]. 20 (80%) pts had systemic sclerosis (SSc),3 by 1 (4%) – RA, SLE, dermatomyositis, cryoglobulinaemic vasculitis and overlap-syndrome. Duration of RD was 19.1±9.5 yrs, time since onset of RP 13.2±6.4 yrs. Limited cutaneous SSc (13 pts) manifestated with RP in 54%, diffuse SSc (7 pts) - in 57%. 80% of pts had activity of both RD and RP.6 pts was treated by intravenous iloprost 25 μg for 3–5 days every 3 months. 10 pts received intravenous alprostadil 20 μg for 10 days every 6 months. III group had alprostadil-switched-to-iloprost (due to alprostadil ineffectiveness; 7 pts) or iloprost-switched-to-alprostadil (due to iloprost side effects; 2 pts) therapy. There were no significant differences between three groups at baseline in Raynaud's Condition Score (RCS), SSc activity and Health Assessment Questionnaire Disability Index (HAQ-DI). All pts were treated with conventional synthetic DMARDs.ResultsAt baseline, the incidence of finger pallor and numbing was 80%, digital pain – 68%, fingertip clefts – 44%, digital tip ulcers – 40%, fingertip pitting scars – 16%. 56% of pts had constant RP symptoms and signs. Joint and muscle involvement had 76% of pts, pulmonary involvement – 68%, heart diseases – 56%.All treatments allowed reducing or stopping digital numbness and pain. About six-sevenths of pts with RP could be classified as good responders to iloprost and two-thirds – to alprostadil. Iloprost reduced the frequency of RP attacks by ∼1.2/day compared with alprostadil (p=0.024) and daily duration of RP attacks by ∼45.3 min in comparison (p=0.015). Treatment with iloprost showed significant benefit on RCS with ∼2.8 difference with alprostadil (p=0.032). After a 24-month follow-up all iloprost pts had healed up digital clefts and ulcers. A trend towards a decrease in HAQ-DI was seen in alprostadil groups, and significant HAQ-DI reduction – in iloprost. Treatment with iloprost was associated with a trend towards improvements in pulmonary hypertension and functions. Both prostanoids were well tolerated, except for incidents of tachycardia and hypotension in 2 iloprost pts.ConclusionsAlprostadil has significant but moderate efficacy in secondary RP. Iloprost is superior to alprostadil and might be recommended not only for severe RP but for preventing digital ulcers.ReferencesWigley FM. Clinical practice. Raynaud's phenomenon. N Engl J Med 2002;347:1001–1008.Kowal-Bielecka O, Landewé R, Avouac J, et al. EULAR recommendations for the treatment of systemic sclerosis: a report from the EU...