PRACA ORYGINALNA
Clinical pattern of systemic sclerosis in Central Ukraine. Association between clinical manifestations of systemic sclerosis and hypertension
Więcej
Ukryj
Data nadesłania: 24-01-2018
Data ostatniej rewizji: 23-02-2018
Data akceptacji: 25-02-2018
Data publikacji online: 28-02-2018
Data publikacji: 28-02-2018
Reumatologia 2018;56(1):24-30
SŁOWA KLUCZOWE
DZIEDZINY
STRESZCZENIE
Objectives:
Systemic 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 methods:
Patients 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.
Results:
In 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.
Conclusions:
The 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).
REFERENCJE (29)
1.
Roberts-Thomson P, Jones M, Hakendorf P, et al. Scleroderma in South Australia: epidemiological observations of possible pathogenic significance. Intern Med J 2001; 31: 220-229.
2.
Alamanos Y, Tsifetaki N,Voulgari PV, et al. Epidemiology of systemic sclerosis in northwest Greece 1981 to 2002. Semin Arthritis Rheum 2005; 34: 714-720.
3.
Nikpour M, Stevens MW, Herrick AL, et al. Epidemiology of systemic sclerosis. Best Pract Res Clin Rheumatol 2010; 74: 857-869.
4.
Silman AJ. Epidemiology of scleroderma. Ann Rheum Dis 1991; 50: 846-853.
5.
Dobrota R, Maurer B, Graf N, et al. Prediction of improvement in skin fibrosis in diffuse cutaneous systemic sclerosis: a EUSTAR analysis. Ann Rheum Dis 2016; 75: 1743-1748.
6.
Allanore Y, Denton CP, Krieg T, et al. Clinical characteristics and predictors of gangrene in patients with systemic sclerosis and digital ulcers in the Digital Ulcer Outcome Registry:.
7.
a prospective, observational cohort. Ann Rheum Dis 2016; 75: 1736-1740.
8.
Subcommittee for Scleroderma Criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee. Preliminary criteria for the classification of systemic sclerosis (scleroderma). Arthritis Rheum 1980; 23: 581-590.
9.
LeRoy EC, Black C, Fleischmajer R, et al. Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol 1988; 15: 202-205.
10.
LeRoy EC, Medsger TA. Criteria for the classification of early systemic sclerosis. J Rheumatol 2001; 28: 1573-1576.
11.
van den Hoogen F, Khanna D, Fransen J, et al. 2013 classification criteria for systemic sclerosis: an American College of Rheumatology/European league against rheumatism collaborative initiative. Ann Rheum Dis 2013; 72: 1747-1755.
12.
Minier T, Guiducci S, Bellando-Randone S, et al. Preliminary analysis of the very early diagnosis of systemic sclerosis (VEDOSS) EUSTAR multicentre study: evidence for puffy fingers as a pivotal sign for suspicion of systemic sclerosis. Ann Rheum Dis 2014; 73: 2087-2093.
13.
Kuryata OV, Lysunets TK, Semenov VV. Risk and predictors of development of arterial hypertension in patients with systemic sclerosis. Arterial Hypertension 2017; 53, doi:
http://dx.doi.org/10.22141/222....
14.
Hunzelmann N, Genth E, Krieg T, et al. The registry of the German Network for Systemic Scleroderma: frequency of disease subsets and patterns of organ involvement. Rheumatology 2008; 47: 1185-1192.
15.
Walker UA, Tyndall A, Czirjak L, et al. Clinical risk assessment of organ manifestations in systemic sclerosis: a report from the EULAR cleroderma Trials And Research group database. Ann Rheum Dis 2007; 66: 754-763.
16.
Agca R, Heslinga SC, Rollefstad S, et al. EULAR recommendations for cardiovascular disease risk management in patients with rheumatoid arthritis and other forms of inflammatory joint disorders: 2015/2016 update. Ann Rheum Dis 2017; 76: 17-28.
17.
Underwood JC, Cross SS. Osteoarticular and connective tissues. In: General and Systematic Pathology. Hughes DE (ed.). 5th Edition. Churchill Livingstone, Edinburgh 2009; 710-474.
18.
Argula RG, Harley RA, Silver RM, et al. Is systemic sclerosis related pulmonary arterial hypertension a distinct phenotype? A lung morphometric analysis of systemic sclerosis associated vs idiopathic pulmonary arterial hypertension. J Scleroderma Relat Disord 2017; 2 (suppl 1): s1-s20.
19.
Cannon PJ, Hassar M, Case DB, et al. The relalioship of hypertension and renal failure in scleroderma (progressive systemic sclerosis) to structural and functional abnormalities of the renal cortical circulation. Medicine 1974; 53: 1-46.
20.
Kovalchik MT, Guggenheim SJ, Silverman MH, et al. The kidney in progressive systemic sclerosis: A prospective study. Ann Intern Med 1978; 89: 881-887.
21.
Simon NM, Graham MB, Kyser FA, et al. Resolution of renal failure with malignant hypertension in scleroderma: Case report and review of the literature. Am J Med 1979; 67: 533-539.
22.
Kowal-Bielecka O, Fransen J, Avouac J et al. Update of EULAR recommendations for the treatment of systemic sclerosis. Ann Rheum Dis 2017; 76: 1327-1339.
23.
Simeón-Aznar CP, Fonollosa-Plá V, Tolosa-Vilella C, et al. Registry of the Spanish Network for Systemic Sclerosis: Clinical Pattern According to Cutaneous Subsets and Immunological Status. Semin Arthritis Rheum 2012; 41: 789-800.
24.
Mancia G, Fagard R, Narkiewicz K, et al. 2013 ESH/ESC Guidelines for the management of arterial hypertension: The Task Force for the management of arterial hypertension of the European Society of Hypertension (ESH) and of the European Society of Cardiology (ESC). Eur Heart J 2013; 34: 2159-2219.
25.
Bryan C, Knight C, Black CM, et al. Prediction of five-year survival following presentation with scleroderma. Arthritis Rheum 1999; 42: 2660-2665.
26.
Tent H, Waanders F, Krikken JA, et al. Performance of MDRD study and CKD-EPI equations for long-term follow-up of nondiabetic patients with chronic kidney disease. Nephrol Dial Transplant 2012; 27 Suppl 3: iii89-95.
27.
Shanmugam VK, Steen VD. Renal Manifestations in Scleroderma: Evidence for Subclinical Renal Disease as a Marker of Vasculopathy. Int J Rheumatol 2010; pii: 538589.
28.
Tamaki T, Mori S, Takehara K. Epidemiological study of patients with systemic sclerosis in Tokyo. Arch Dermatol Res 1991; 283: 366-371.
29.
Bellando-Randone S, Guiducci S, Matucci-Cerinic M. Very early diagnosis of systemic sclerosis. Pol Arch Med Wewn 2012; 122 Suppl 1: 18-23.
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