Abstract

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.

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