Репозиторий Университета

© Author(s) (or their employer(s)) 2019. Objectives To investigate the influence of vasodilator drugs on the occurrence of features depending on myocardial ischaemia/fibrosis (ventricular arrhythmias, Q waves, cardiac blocks, pacemaker implantation, left ventricular ejection fraction (LVEF) <55%, and/or congestive heart failure and sudden cardiac death) in systemic sclerosis (SSc). Methods 601 patients with SSc were enrolled from 1 December 2012 to 30 November 2015 and had a second visit 0.5-4 years apart. 153 received no vasodilators; 448 received vasodilator therapy (ie, calcium channel blockers and/or ACE inhibitors or angiotensin II receptor blockers or combinations of them), 89 of them being also treated with either endothelin receptor antagonists or PDE5 inhibitors or prostanoids. Associations between the occurrence of myocardial disease manifestations and any demographic, disease and therapeutic aspect were investigated by Cox regression analysis. A Cox frailty survival model with centre of enrolment as random effect was performed. Results During 914 follow-up patient-years, 12 ventricular arrhythmias, 5 Q waves, 40 cardiac blocks, 6 pacemaker implantations and 19 reduced LVEF and/or congestive heart failure (CHF) occurred. In multivariate Cox regression analysis, vasodilator therapy was associated with a lower incidence of ventricular arrhythmias (p=0.03); low-dose acetylsalicylic acid (ASA) with a lower incidence of cardiac blocks and/or Q waves and/or pacemaker implantation (p=0.02); active disease with a higher incidence of LVEF <55% and/or CHF and cardiac blocks and/or Q waves and/or pacemaker implantation (p=0.05). Conclusions The present study might suggest a preventative effect on the occurrence of distinct myocardial manifestations by vasodilator therapy and low-dose ASA.

© 2018, Pediatria Ltd.. All rights reserved. Objective of the research: to dentify risk factors for growth retardation in patients with juvenile scleroderma (JSD). The presented data show that the most unfavorable factors for growth retardation in patients with JSD are the systemic form of the disease, debut age less than 4 years. Other unfavorable factors of growth retardation developing are father's height less than 175 cm and a mother's height less than 165 cm. Analysis of the effect of glucocorticoid saverage daily dose during the year on the growth rate in patients with JSD, normalization of growth rates was noted with its decrease to 0,2 mg/kg/day or less.

© 2018 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología Case history of a small girl outlet with epilepsy, followed by scleroderma skin damage and uveitis, neurovasculitis with white matter foci in brain on the side of skin lesion in two months, immunologic disease activity. Resistance to conventional immunosuppressive therapy forced us to initiate the treatment with tocilizumab. It was well tolerated and led to significant improvement of brain, ocular and skin manifestations.

© 2018 Media Sphera Publishing Group. All rights reserved. Scleromyxedema is a rare mucinosis with a primary skin lesion due to diffuse mucin deposition, sclerosis, and lichenoid eruptions in the absence of hypothyroidism. The paper describes scleromyxedema cases and gives recommendations for the histological diagnosis of the disease by histochemical reactions to detect acid and neutral glycosaminoglycans.