Clinical and epidemiological characteristics and treatment of Raynaud's phenomenon in rheumatic diseases

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.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1584.1-1585
Author(s):  
I. Gaisin ◽  
Z. Bagautdinova ◽  
R. Valeeva ◽  
N. Maximov ◽  
O. Desinova ◽  
...  

Background:Systemic sclerosis (SSc) is a connective tissue disease (CTD) most frequently associated with Raynaud’s phenomenon – RP (96%), followed by mixed CTD (MCTD) (86%), systemic lupus erythematosus – SLE (31%), undifferentiated CTD (30%), rheumatoid arthritis – RA (22%) and Sjogren’s disease – SD (13%)1. RP can manifest as a classical triple-colour change with pallor (ischaemic phase) followed by cyanosis (deoxygenation) and erythema (reperfusion)2,1. However, this triple-colour change only occurs in 19% of cases2,3. Majority of patients report an episodic double-colour change, consisting of pallor and cyanosis, pallor and erythema or cyanosis and erythema2. In a 4.8-year follow-up, 37.2% of RP patients developed rheumatic diseases (RD), 8.1% had other causes, in 54,7% RP remained primary4.Objectives:To study the incidence and manifestations of secondary RP in RD.Methods:A questionnaire survey conducted in 230 patients with RD.Results:RP was detected in 45.6% of RD patients (n=105), 54.4% of patients with RD had no RP (n=125). RP was 4 times more frequent in females than in males (F:M 4:1). In RP group, 87 patients (82%) had autoimmune RD: SSc (55.2%), SLE (17.1%), RA (6%),dermatomyositis (3.8%), cross syndrome (3.8%), MCTD (1.9%), SD (0.9%).Only 84% of RP patients had positive answers to all three questions that characterizeRP (1. Is there an unusual sensitivity of fingers to cold? 2. Do fingers change colorwhen exposed to cold? 3. Do they turn white and/or bluish?). Biphasic color changes (whitening-blueness; whitening-redness; blue-redness) were observed in 33 (31.4%) patients with RP, three-phase changes – in 32 patients (30.5%). Blueness of fingers to cold was more frequent in SLE than in SSc (p=0.027).Redness of fingers to cold occurred more often in cross syndrome, MCTD, SD, RA, vasculitis than in SSc (p<0.001) and in vasculitis than in SLE (p=0.035). In SSc patients, whitening of fingers to cold was more common than redness (p=0.037) and two-/three-phase changes of fingers color in the cold were more frequent than single-phase changes (p<0.001).The frequency of RP attacks was detected more than once a day in 44 (42%) patients. In 73% of cases, RP did not show signs of deep digital ischemia. Digital ulcers (active) were observed in 13 (12.3%) patients, fractures in a finger area – 23 (21.9%), digital scars – 15 (14.2%), phalange amputations – 7 (6.6%).Conclusion:Patients with RD and secondary RP most often have SSC (55%), less often – SLE (17%), RA (6%), DM (3%). In SSc and SLE patients, Raynaud’s reddening of fingers to cold is less common than in other RD. In SSc, two-/three-phase changes of fingers color in the cold are more frequent than single-phase changes. In SLE, fingers turn blue in the cold more often than in SSc.References:[1]Prete M, Fatone MC, Favoino E, Perosa F. Raynaud’s phenomenon: from molecular pathogenesis to therapy.Autoimmun Rev2014;13:655–67.[2]Linnemann B, Erbe M. Raynaud’s phenomenon – assessment and differential diagnoses.Vasa2015;44:166–77.[3]Heidrich H, Helmis J, Fahrig C, Hovelmann R, Martini N. Clinical characteristics of primary, secondary and suspected secondary Raynaud’s syndrome and diagnostic transition in the long-term follow-up. A retrospective study in 900 patients.Vasa2008;37 (Suppl. 73):3–25.[4]Pavlov–Dolijanovic S, Damjanov NS, VujasinovicStupar NZ, Radunovic GL, Stojanovic RM, Babic D. Late appearance and exacerbation of primary Raynaud’s phenomenon attacks can predict future development of connective tissue disease: a retrospective chart review of 3035 patients.RheumatolInt2013;33:921–6.Acknowledgments:Professor LP. Anan’eva, Professor RT. AlekperovDisclosure of Interests:Ilshat Gaisin Speakers bureau: Boehringer Ingelheim, KRKA, Berlin-Chemie Menarini, Sanofi, Zukhra Bagautdinova: None declared, Rosa Valeeva: None declared, Nikolay Maximov Speakers bureau: Pfizer, KRKA, Oxana Desinova: None declared, Rushana Shayakhmetova: None declared, Irina Sabelnikova: None declared, Anna Tukmacheva: None declared, Larisa Gibadullina: None declared, Natalya Burlaeva: None declared, Elena Agareva: None declared, Yulia Ochkurova: None declared, Tatyana Bragina: None declared, Ksenia Alexandrova: None declared, Elvira Reutova: None declared


2015 ◽  
Vol 4 (1) ◽  
pp. 13-15
Author(s):  
Binit Vaidya ◽  
Manisha Bhochhibhoya

Back grounds and Aims: Microvascular involvement of nailfold often occurs in systemic rheumatic diseases, especially scleroderma and related conditions. Nailfold capillaroscopy is easily performed non-invasive, simple, repeatable, sensitive, safe and inexpensive method to examine nailfold architecture by microscope.The normal capillaries are thin, symmetrical and have hair-pin appearance. Anomalies include dilated capillary loops, dropouts, tortuosity, avascular area, arborifications, megacapillaries, infracts,thrombsis and haemorrhages. This study aims to see the morphological changes in microcirculation in nailfolds of patients with Raynaud’s, which can be helpful in predicting the presence of connective tissue disorder.Methods: It is an observational study conducted at National Center for Rheumatic Diseases, Kathmandu from 26th March 2014 to 30th March 2015. Nailfolds of 8 fingers of both hands in 105 patients were examined using a hand held microscope.Results: Of 105 patients, 91 patients (86.6%) had abnormal morphological changes while 14 (13.4%) had no significant changes in microvessels. Normal patients had no underlying connective tissue disease(CTD) but those with abnormal findings had proven underlying CTD which included Lupus 6.5%, Scleroderma 17.5%, Rheumatoid Arthritis 14.2%, Mixed CTD7.6%, Undifferentiated CTD1.0%, Undifferentiated inflammatory arthritis 14.2%, Psorasis 1.0%, Rheumatoid Arthritis with scleroderma overlap 4.3%. No primary diagnosis was found in 32.9% of patients.Conclusion: Nailfold capillaroscopy allows direct examination of microvascular structure of a patient. Abnormal findings in a patient with Raynaud’s phenomenon might help to identify the presence of or future evolution into systemic connective tissue disorder.Journal of Advances in Internal Medicine 2015;04(01):13-15


1991 ◽  
Vol 10 (3) ◽  
pp. 311-315 ◽  
Author(s):  
E. V. Tsianos ◽  
C. Berecos ◽  
E. Stavropoulos ◽  
E. Kazazis ◽  
I. C. Danielides ◽  
...  

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.


2003 ◽  
Vol 73 (1) ◽  
pp. 3-7 ◽  
Author(s):  
M. E. Mavrikakis ◽  
J. P. Lekakis ◽  
M. Papamichael ◽  
K. S. Stamatelopoulos ◽  
Ch. C. Kostopoulos ◽  
...  

Previous studies have shown that patients with Raynaud’s phenomenon secondary to systemic sclerosis present abnormal endothelial function; the mechanisms responsible for the endothelial dysfunction are unknown but increased vascular oxidative stress could be a possible cause. The hypothesis that a potent water-soluble antioxidant can reverse endothelial dysfunction in these patients was tested in the present study. We examined 11 female patients with Raynaud’s phenomenon secondary to systemic sclerosis and ten healthy control women by ultrasound imaging of the brachial artery to assess flow-mediated (endothelium-dependent) and nitrate-induced (endothelium-independent) vasodilatation. Flow-mediated dilatation and nitrate-induced dilatation were significantly reduced in patients with Raynaud’s phenomenon, indicating abnormal endothelial and smooth muscle cell function. Patients with Raynaud’s phenomenon entered a double-blind, randomized, crossover placebo-controlled trial and received orally 2 g of ascorbic acid or placebo; vascular studies were repeated two hours after ascorbic acid or placebo administration. Flow-mediated dilatation did not improve after ascorbic acid (1.6 ± 2.2% to 2.2 ± 2.5%, ns) or placebo administration (1.2 ± 1,9% to 1.7 ± 1.4%, ns); also nitrate-induced dilatation was similar after ascorbic acid or placebo (16 ± 7.4% vs 17 ± 8%, ns), suggesting no effect of ascorbic acid on endothelial and vascular smooth muscle function. In conclusion, ascorbic acid does not reverse endothelial vasomotor dysfunction in the brachial circulation of patients with Raynaud’s phenomenon secondary to systemic sclerosis. The use of different antioxidants or different dosing of ascorbic acid may be required to show a beneficial effect on endothelial vasodilator function.


VASA ◽  
2005 ◽  
Vol 34 (4) ◽  
pp. 243-249 ◽  
Author(s):  
Drinda ◽  
Neumann ◽  
Pöhlmann ◽  
Vogelsang ◽  
Stein ◽  
...  

Background: Prostanoids are used in the treatment of Raynaud’s phenomenon and acral perfusion disorders secondary to collagenosis. In subjective terms, intravenous administration of these agents produces success in more than 50% of patients. The therapeutic outcome of clinical administration of alprostadil or iloprost may vary from individual to individual. Patients and methods: The following variables were analysed in a cross-over study in 27 patients with collagenosis and Raynaud’s phenomenon: plasma viscosity and erythrocyte aggregation (rheological variables), partial pressure of oxygen and laser Doppler flowmetry in the finger region, and lymphocyte phenotyping and interleukin (IL) determinations (immunological variables). Results: Laser Doppler flowmetry revealed significant differences between patients with secondary Raynaud’s phenomenon and a control group of 25 healthy subjects. Laser Doppler readings did not change significantly as a result of the treatments. Therapy with iloprost produced a reduction in IL-1beta, L-selectin (CD 62 L) and IL-6. Conclusion: The change in immunological variables due to iloprost may explain the long-term effects of prostaglandins in the treatment of Raynaud’s phenomenon. From our results it is not possible to infer any preference for iloprost or alprostadil.


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