ObjectivesSystemic sclerosis (SSc) is a rare disease of connective tissue, manifestations of which may vary in different geographical areas. We aimed to describe the clinical portrait of patients with SSc in Dnipropetrovsk region and to investigate how initial clinical and laboratory characteristics are connected with the presence of hypertension in SSc onset.Material and methodsPatients were enrolled to this study from the registry of SSc patients, established in the Rheumatology Department, Mechnikov Dnipropetrovsk Regional Clinic, Dnipro. This registry contains histories of new cases of SSc from 1993 to 2014. Patients are followed-up and receive treatment according to EULAR and local standards. Diagnosis of SSc was based on ACR and EULAR Criteria for systemic Sclerosis. Two patients developed scleroderma renal crisis during follow-up. This report is a cross-sectional study. We analysed only data of the first visit to a rheumatologist.ResultsIn total 148 patients (median age [IQR] – 47 [40; 52] years) fulfilled the inclusion criteria. Male/female ratio was 1 : 20.1. The most frequent clinical signs were Raynaud’s phenomenon and arthritis. The prevalence of skin lesion in dcSSc patients was twice as high as in lcSSc patients. Pulmonary fibrosis occurred significantly more commonly in dcSSc patients. Hypertension occurred in 26–33% in both groups. Patients with hypertension at the SSc onset were seven years older than normotensive patients. More hypertensive patients were classified as lcSSc. Mean GFR was dramatically lower in hypertensive patients.ConclusionsThe most common clinical form in our study was diffuse cutaneous subset of SSc. Hypertension in patients with SSc may be associated with local cutaneous subset of SSc and renal impairment. The strongest predictors of clinical form of SSc are signs of fibrosis (skin lesion and pulmonary fibrosis) and inflammation (arthritis and elevated CRP).
Системна склеродермія (ССД)-системне захворювання сполучної тканини невідомої етіології із широким спектром клінічних проявів. Пацієнти із запальними захворюваннями суглобів, до яких можна віднести ССД, мають підвищений ризик кардіоваскулярних (КВ) ускладнень порівняно із загальною популяцією [1]. Серед причин смерті, не пов'язаних із ССД, КВ-ускладнення (зокрема, серцева недостатність) посідають друге місце [2, 3]. Оцінка КВ-ризиків проводиться з використанням таких шкал, як Фремінгемська або SCORE. Проте в низці досліджень була продемонстрована неточність прогнозу цих шкал для пацієнтів із ревматоїдним артритом (РА) [4, 5]. Можливо, рівень запалення та застосування протизапальних засобів впливають на показники, за допомогою яких оцінюють КВ-ризик у загальній популяції [1]. Робоча група EULAR у 2016 р. розробила рекомендації з ведення КВ-ризиків для пацієнтів із РА [1], але для пацієнтів із ССД такі дослідження ще не проводились. Артеріальна гіпертензія (АГ) є потужним фактором, що впливає на КВ-ризики, який піддається модифікації [6-8] та відносно просто діагностується. Як і у випадку з РА, при ССД спостерігається підвищений рівень запальної активності. Зважаючи на
Background:Sjogren’s syndrome (SS) is a rare autoimmune disease with multisystem manifestations. Diagnostics of SS is intricate due to its low prevalence and deed of invasive tests for diagnosis confirmation.Objectives:To investigate influence of the first symptom of SS on the time of diagnosis establishment.Methods:The study was conducted at Dnipropetrovsk Mechnikov Regional Hospital, Dnipro, Ukraine. 23 patients (1 male and 22 females, mean age 54 [47;61] years) with SS that received medical care at Rheumatology Department from 2007th to 2017th were enrolled to the study. Diagnosis of SS was provided according to American-European Consensus Group criteria (2002). We analyzed time from the first symptom appearance to diagnosis of SS establishment. Symptoms of the disease onset were classified into 4 groups: fever (body temperature>37 C), arthritis (swelling and/or tenderness of ≥1 joints), signs of salivary glands injury (swelling of salivary glands, dry mouth syndrome) and other signs of autoimmune diseases (Rhaynaud’s phenomenon, rash, myalgia).Results:Median time between the first symptoms and SS diagnostics was 8 [2;17] years. Time to SS diagnostics depending on the first manifestation is in the table below.Table 1 Time of SS diagnostics depending on the first symptom.SymptomPresentAbsentLog-rank test (p)Fever3 [1;8]9,5 [2,2;17]0,06Arthritis17 [6;22,5]6,5 [2;11,5]0,06Salivary glands injury11 [6;19,5]4,5 [2;10]0,15Symptoms of autoimmune diseases4 [2;8]10,5 [2;18]0,09Presence of fever or symptoms of autoimmune diseases led to faster diagnostics of SS. Presence of salivary glands injury didn’t influence time of SS diagnostics, while arthritis enlarged it (Figure 1).Conclusion:Fever and signs of autoimmune diseases may be useful in diagnostics of SS. Greater alertness of symptoms of salivary glands injury is needed.References[1] Vitali C, Bombardieri S, Jonsson R, et al. Classification criteria for Sjögren’s syndrome: a revised version of the European criteria proposed by the American-European Consensus Group. Ann Rheum Dis. 2002;61(6):554-8.Disclosure of Interests:None declared
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