scholarly journals PReS-FINAL-2171: Consensus: what agent to use when first-line vasodilators fail in Raynaud's phenomenon or digital ulcers secondary to rheumatic diseases in children?

2013 ◽  
Vol 11 (S2) ◽  
Author(s):  
M Katsicas ◽  
◽  
M Gonzalez ◽  
R Russo
2018 ◽  
Vol 99 (6) ◽  
pp. 911-918
Author(s):  
Z R Bagautdinova ◽  
I R Gaisin ◽  
M M Glavatskikh ◽  
T A Bragina

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.


2018 ◽  
Vol 16 (6) ◽  
pp. 178-184
Author(s):  
Z. R. Bagautdinova ◽  
◽  
I. R. Gaysin ◽  
L. V. Ivanova ◽  
A. A. Trukhina ◽  
...  

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1256.3-1257
Author(s):  
S. Lambova

Background:Videocapillaroscopy is the gold standard for evaluation of nailfold capillaries and the major tool used for differentiation of primary and secondary Raynaud’s phenomenon (RP) in rheumatology practice. However, nowadays, there are also accessible alternatives such as USB capillaroscopes, which offer the opportunity to apply capillaroscopic examination at a significantly lower price.Objectives:The aim of the current study was to study the utility of USB capillaroscope (Dinolite) via assessment of capillaroscopic images obtained by patients with primary and secondary RP in rheumatic diseases.Methods:The study represents analysis of capillaroscopic images of 32 patients with RP – primary and secondary in the context of SSc or other rheumatic diseases i.e., undifferentiated connective tissue disease (UCTD) and systemic lupus erythematosus (SLE). All the patients had signed an informed consent for participation in a study of their capillaroscopic, laboratory and clinical associations. The study represents retrospective analysis of the capillaroscopic images obtained from 8 fingers (II-V bilaterally) using USB capillaroscope (Dinolite) at magnification 200x. Capillary diameters were measured (arterial, venous and apical loop) as well as the number of capillaries per millimeter. The capillaroscopic images were categorized into the following groups i.e., I. Absence of microangiopathy: i) normal pattern, ii) nonspecific changes (dilated capillaries with arterial diameter > 0.015mm, venous > 0.020mm; haemorhhages and/or other nonspecific changes), II. Presence of microangiopathy i.e., “scleroderma”/”scleroderma-like” pattern. Presence of giant capillaries with capillary diameter >0.050mm was considered as a sufficient criterion for classifying the image as “scleroderma”/”scleroderma-like” pattern. For “scleroderma” type images in SSc patients staging of Cutolo et al (2000) was used i.e., “early”, ”active”, ”late” phase (1).Results:Images suitable for analysis with good visibility that permits analysis of the major capillaroscopic parameters were available in all patients. Among 32 included patients, 9 patients were with SSc, 12 cases with primary RP, and 10 patients with secondary RP in other CTD (7 patients with UCTD and 3 patients with SLE). „Scleroderma“ pattern was detected in 6 patients with SSc and in all these cases the capillaroscopic images were classifiable into one of the three distinct phases i.e., “early”, ”active” and ”late” phase. Presence of microvascular changes (“scleroderma-like” pattern) was detected also in 5 among the 10 patients with other CTD i.e., UCTD and SLE. In primary RP patients capillaroscopy revealed either normal pattern or nonspecific findings but without features of microangiopathy.Conclusion:Good capillaroscopic images, which could be analyzed and interpreted, are usually obtained using USB capillaroscope. This permits evaluation of the major capillaroscopic parameters. The available software although less sophisticated vs those of videocapillaroscopes, gives the opportunity for measurement of capillary diameters, mean capillary density, etc. The images received from USB capillaroscope are easily classified into “scleroderma”, “scleroderma-like”, non-specific changes and normal pattern. The most important conclusion from capillaroscopy is about presence or absence of microangiopathy. This was easily detected via USB capillaroscope that could be suggested as an ideal alternative for videocapillaroscopes in every day rheumatology practice especially at low budget cases. Measurements of capillary diameters and capillary density provide quantitative data that make these devises also appropriate for scientific research.References:[1]Cutolo M, Sulli A, Pizzorni C AS. Nailfold videocapillaroscopy assessment of microvascular damage in systemic sclerosis. J Rheumatol. 2000;27(1):155–60.Disclosure of Interests:None declared.


2019 ◽  
Vol 5 (2) ◽  
pp. 159-164 ◽  
Author(s):  
Jan-Gerd Rademacher ◽  
Chris Wincup ◽  
Björn Tampe ◽  
Peter Korsten

Background: Systemic sclerosis is a rare autoimmune disease characterized by skin and organ fibrosis, and vasculopathy. Raynaud’s phenomenon is almost universally present in systemic sclerosis and can be the most debilitating symptom. Raynaud’s phenomenon may lead to the development of digital ulcers, potentially complicated by infection, tissue necrosis, and auto-amputation. Recommended treatments have variable efficacy. Methods: We report the case of a 30-year-old woman with diffuse systemic sclerosis suffering from severe Raynaud’s phenomenon and digital ulcers with digital tissue necrosis who was treated with combination therapy of an endothelin receptor antagonist and phosphodiesterase 5 inhibitor. In addition, we reviewed the literature on the topic. Results: Previous therapy with calcium-channel blockers, intravenous iloprost, and bosentan had all failed to control symptoms. We added sildenafil in combination with bosentan and observed a rapid and sustained treatment effect. Raynaud’s phenomenon severity, number of attacks, and attack duration decreased within 2 weeks of initiating treatment. Furthermore, this resulted in the healing of established digital ulcers. Conclusion: Our case report suggests that combination therapy may be a feasible treatment for the most severely affected and refractory patients. In our literature review, we found one retrospective study and three additional cases with similarly encouraging results.


VASA ◽  
2001 ◽  
Vol 30 (4) ◽  
pp. 281-284 ◽  
Author(s):  
Mahdi Al-Awami ◽  
M. Schillinger ◽  
M. E. Gschwandtner ◽  
T. Maca ◽  
M. Haumer ◽  
...  

Background: Patients who had been treated with low level laser (LLL) for their digital ulcers reported an impressive improvement of their symptoms of episodic digital ischaemia. Therefore this pilot study was performed to evaluate the efficacy of LLL as a new non-drug non-invasive treatment for patients with primary and secondary Raynaud’s phenomenon (RP). Patients and methods: Forty patients (29 female, 11 male, mean age 51 years) with active primary (28%) and secondary (72%) Raynaud’s phenomenon received 10 sessions of LLL distant irradiation during winter months. Assessment of subjective and objective parameters was performed at baseline, one week after the last session and three months later. Variations of subjective parameters as number of daily acute episodes and severity of discomfort were assessed by a coloured visual analogue scale. A standardised cold challenge test using computed thermography of continuous temperature recordings by means of infrared telethermography was used to assess the digital blood flow. Results: A significant improvement was noticed clinically and thermographically after 6 weeks and 3 months, respectively (p < 0.0001). Conclusions: These data suggest that LLL treatment has a good short and medium term effectiveness in patients with Raynaud’s phenomenon.


2019 ◽  
Vol 4 (2) ◽  
pp. 102-110 ◽  
Author(s):  
Andreu Fernández-Codina ◽  
Esperanza Cañas-Ruano ◽  
Janet E Pope

Raynaud’s phenomenon is nearly universal in systemic sclerosis. Vasculopathy is part of systemic sclerosis. Raynaud’s phenomenon can cause of complications and impairment, especially when tissue ischemia and digital ulcers develop. There are many treatment options for Raynaud’s phenomenon in systemic sclerosis often with sparse data and few robust studies comparing the different treatment options. Recommendations from guidelines usually include calcium channel blockers as first-line pharmacological treatment. In the clinical setting, multiple variables such as financial factors, geography where access to medications varies, and patient factors, baseline hypotension, can influence the treatment for Raynaud’s phenomenon and digital ulcers. Prostacyclins and PDE-5 inhibitors are reserved for more severe Raynaud’s phenomenon or healing of digital ulcers. Prevention of digital ulcers may also include endothelin receptor blocker (bosentan) in some countries. Other treatments had less consensus. Algorithms developed by systemic sclerosis experts might be helpful in deciding which treatment to choose for each setting, using a step-wise strategy, which intends to complement guidelines. This review focuses on a practical approach to the treatment of Raynaud’s phenomenon and digital ulcers in systemic sclerosis, based on algorithms designed by systemic sclerosis experts using consensus, and we review the evidence that supports treatment from initial to second and third-line options.


2019 ◽  
Vol 80 (11) ◽  
pp. 658-664
Author(s):  
Vikrant Devgire ◽  
Michael Hughes

Raynaud's phenomenon is a common vasospastic condition which carries a significant burden of pain and hand-related disability ( Hughes and Herrick, 2016 ). The prevalence of Raynaud's phenomenon in the general population has been reported to be approximately 5% ( Garner et al, 2015 ). Raynaud's phenomenon can occur either as a primary (‘idiopathic’) phenomenon or secondary to a wide range of underlying medical conditions and drug causes. Therefore, hospital-based specialists are frequently involved in the care of patients with Raynaud's phenomenon and need to be aware of associated conditions and prescribed medications for Raynaud's phenomenon. In particular, Raynaud's phenomenon is often the earliest manifestation of an underlying autoimmune connective tissue disease (e.g. systemic sclerosis). A comprehensive clinical assessment is required including performing targeted investigations (e.g. nailfold capillaroscopy and systemic sclerosis-associated autoantibodies). Patient education and lifestyle adaptations is first-line treatment for Raynaud's phenomenon. There is a wide range of pharmacological options including oral and intravenous drug therapies available to treat Raynaud's phenomenon. Surgical intervention is sometimes required for refractory Raynaud's phenomenon and tissue ischaemia. This review describes the clinical manifestations of Raynaud's phenomenon including potential secondary causes and presents an approach to assessment and management.


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