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Evidence summaries

Iloprost and Cisaprost for Raynaud's Phenomenon in Progressive Systemic Sclerosis

Intravenous iloprost is effective for Raynaud's phenomenon in progressive systemic sclerosis. Level of evidence: "A"

Seven randomised trials and 322 patients were included in a Cochrane review [Abstract] 1. Intravenous iloprost was effective in the treatment of Raynaud's phenomenon secondary to scleroderma at decreasing the frequency of attacks and preventing or healing digital ulcers. The number of digital ulcers healed was highly significant in the i.v. iloprost group (Peto OR 23.17, 95% CI 2.20 to 243.52; 1 study, n=11). Side effects were also more common in in the iloprost patients compared to the placebo subjects (Peto OR 9.44, 95% CI 5.05 to 17.67; 2 studies, n=166). Physician global assessment (with respect to number of subjects improved) was statistically significant with an odds ratio of 2.61 (95% CI 1.27 to 5.38; 1 study, n=122). The effect seems to be prolonged after the intravenous infusion is given. Oral iloprost may have less efficacy, and cisaprost has minimal or no efficacy.

EULAR 2 recommends that i.v. iloprost should be considered as treatment option for severe severe systemic sclerosis-related Raynaud phenomenon.

    References

    • Pope J, Fenlon D, Thompson A, et al. Iloprost and cisaprost for Raynaud's phenomenon in progressive systemic sclerosis. Cochrane Database Syst Rev 2000;1998(2):CD000953 [PubMed]
    • Kowal-Bielecka O, Fransen J, Avouac J, et al. Update of EULAR recommendations for the treatment of systemic sclerosis. Ann Rheum Dis 2017;76(8):1327-1339 [PubMed]

Primary/Secondary Keywords