Buerger's disease, or thromboangiitis obliterans, is a severe disabling systemic disease of vessels. The paper describes a case of thromboangiitis obliterans in a patient with three extremities amputated during vascular therapy. The course intravenous administration of the stable prostacyclin analogue iloprost (Ilomedin ®) allows the only extremity to be preserved.
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...
Aim. On the basis of clinical manifestations of Raynaud’s phenomenon (RP) to determine the RP expression level and to evaluate the long-term efficacy of treatment of RP in patients with rheumatic diseases (RD) with iloprost and alprostadil. Methods. Treatment for indications with prostanoids (intravenous iloprost, alprostadil or their combinations) was administered to 40 patients with secondary RP in RD. During 3-year follow up, clinical instrumental parameters (frequency of attacks of Raynaud's, digital ulcers (DU) formation, pain intensity on visual analogue scale (VAS) were evaluated. The control group included 30 patients with RP in RD who did not receive prostanoid therapy. The method of factor analysis was used to determine the index of generalized expression of RP, on the basis of which the expression levels of the RP were determined. Results. The “expression of RP” scale revealed in the course of factor analysis as an indicator of generalized manifestation of RP, is the average value of two subscales, the first one of which consisted of four indices - “digital ulcer”, “digital pitting scars”, “phalange amputation” and “Raynaud's attack frequency”, and the second one included “intensity of pain”, “duration of illness”, “whitening of fingers”. Verification of the correlation revealed during the study of subscales, showed its reliability (r = 0.294, p = 0.053). The final expression of RP was 1.51 ± 0.86. The low level of RP expression had values up to 0.65, high - over 2.37. When included in the study, a high level of RP expression was defined in 16 (22.9 %) patients, medium - in 43 (61.4 %), low - in 11 (15.7 %). The use of iloprost in the treatment of RP was effective for healing of DU and a statistically significant decrease of the generalized expression of RP from 2.25 [1; 3] to 1.75 [1; 2] (p = 0.012) was observed. On alprostadil treatment, statistically significantly decreased frequency of attacks from multiple daily (more than once a day) to once a month and reduced numbness during RP attack were observed as well as a decrease of the level of generalized index of RP expression from 1.26 ± 0.71 to 0.97 ± 0.57 (р = 0.038). The combination of prostanoids had a contradictory clinical effect: the pain and frequency of RP attacks decreased, but the formation of DU and new cases of amputations of phalanges were registered, the treatment had no effect on the value of RP expression. Conclusion. Based on the clinical manifestations of RP, a generalized index of RP severity was identified and the levels of RP severity were determined. Treatment with iloprost and alprostadil has a significant effect on reducing the clinical manifestations of RP with a corresponding decrease in its severity.
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