LAUSR

Raynaud’s phenomenon (RP) secondary to rheumatic diseases (RD) can progress to irreversible tissue damage with digital ulceration, scarring and, rarely, gangrene requiring amputation1. Current medical treatments for RP are far from ideal: they are often either ineffective and/or poorly tolerated, thus a significant proportion of patients discontinue drug therapy2.To determine RP expression levels and to evaluate the long-term efficacy of iloprost and alprostadil in RP patients with RD.Indicated therapy with intravenous iloprost (n=10), alprostadil (n=17) or their combinations (n=13) was carried out for three years in patients with secondary RP in RD. Frequency of Raynaud’s attacks, digital ulcers (DU) formation and pain intensity on visual analogue scale (VAS) were evaluated. A control group included 30 patients with RP in RD who did not receive prostanoid therapy. By factor analysis method a generalized index of RP expression was identified, on the basis of which levels of RP expression were determined.“RP expression” scale, revealed as an indicator of RP generalized manifestation, was an average value of two subscales: (1) consisted of 4 indices “DU”, “digital pitting scars”, “phalange amputation” and “frequency of Raynaud’s attack”, (2) included 3 indicators: “intensity of pain”, “duration of illness”, “whitening of fingers”. Correlation of subscales showed their reliability (r=0.294, p=0.053). RP final expression (severity) was 1.51±0.86. A low level of RP expression had values below 0.65, a high level – over 2.37. At baseline, the high level of RP severity was defined in 16 (22.9%) patients, medium – in 43 (61.4%), low – in 11 (15.7%).RP treatment with iloprost was effective in the healing of DU in 100% of patients and led to decrease of RP expression generalized index from 2.25 [1; 3] to 1.75 [1; 2] (p=0.012). Alprostadil therapy reduced pain intensity on VAS (p<0.05) and numbness during Raynaud’s attacks (p<0.01) and decreased RP expression from 1 [1; 2] to 1 [0.5; 1.5] (p=0.038). Patients on prostanoids combination had new DU and amputations; pain intensity reduced by 47% (p<0.05), RP expression generalized indicator did not change.Based on RP clinical manifestations in RD patients, a generalized index of RP expression was identified and levels of RP severity were determined. Treatment with iloprost or alprostadil has significant effects on reducing the clinical manifestations of RP with a corresponding decrease in its severity. Iloprost is indicated in patients with medium and high levels of RP expression index, alprostadil – with medium and low index and non-effectiveness of calcium channel blockers.[1]Hughes M, Herrick AL. Digital ulcers in systemic sclerosis.Rheumatology (Oxford) 2017;56 (1):14–25.[2]Kowal-Bielecka O, Fransen J, Avouac J et al. Update of EULAR recommendation for the treatment of systemic sclerosis.Ann Rheum Dis2017;76(8):1327–39.Professor LP. Anan’evaIlshat Gaisin Speakers bureau: Boehringer Ingelheim, KRKA, Berlin-Chemie Menarini, Sanofi, Zukhra Bagautdinova: None declared, Marianna Glavatskikh: None declared, Nikolay Maximov Speakers bureau: Pfizer, KRKA, Rosa Valeeva: None declared, Oxana Desinova: None declared, Rushana Shayakhmetova: None declared