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THU0361 EPIDEMIOLOGIC VARIATION ON SCLERODERMA RENAL CRISIS AND CLINICAL FEATURES VARIATION ON SYSTEMIC SCLEROSIS PATIENTS OVER TIME: DATA FROM RESCLE REGISTRY.

X. Pla Salas,C. Tolosa,A. Guillen del Castillo, M. E. Sanchez Garcia, J. Sanchez-Redondo,E. L. Callejas-Moraga, L. Saez-Comet,J. A. Vargas-Hitos,J. A. Todoli Parra, L. Trapiella Martinez, I. Rodriguez-Pubto,M. Freire,I. Pons Martin del Campo,V. Fonollosa-Pla,C. P. Simeon-Aznar

Annals of the rheumatic diseases(2020)

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摘要
Background:Scleroderma renal crisis (SRC) prevalence is decreasing. However, no Systemic Sclerosis (SSc) patient’s registry has evaluated that decrease over time. No treatment have been able to prevent SRC development.Objectives:Primary objective: to identify SRC prevalence in 2 periods in the RESCLE(Registro deESCLErodermia) registry. Secondary objective: to idenfy which features could justify that change on SRC prevalence.Methods:Up to December 2018, 1937 SSc patients were included by 31 referral centers in RESCLE registry. SRC prevalence and incidence in diagnosed patients before and after 2003 was determined. Clinical characteristics of diagnosed patients in each period of time were analysed to identify differences between them.Results:Out of 1937 SSc, 43 (2.2%) developed SRC. Prevalence of SRC before and after 2003 was 3.5% and 1.08%. SRC Incidence: Graphic 1. Significant differences between Pre-2003 vs. Post-2003 SSc cohorts were found in univariate analysis: Table 1 and 2.Table. 1.Univariate analysisPre-2003(%)Post-2003(%)Demographic datalcSSc6359dcSSc2815ssSSc6.414Early SSc1.13.3Very early SSc1.99.6Age at SSc dx49.1(±15.2)y55.0(±15.6)yTime from SSc dx to SRC1.1(0.2-4.3)y0.6(0.1-1.5)yACR/EULAR 2013 criteria9986ComorbiditiesSmoked2037Arterial hypertension3529Diagnostic proceduresPAPs >40mmHg by Echocardiography3825Not-sclerodermal pattern at VCS8.919Lupus anticoagulant124.8Prognostic featuresOverall mortality2911ILD-related death101.6Conclusion:SRC Prevalence and Incidence has decreased. Prevalence is three-fold in diagnosed SSc cohort pre-2003 than in post-2003. The post-2003 cohort showed lesser prevalence of dcSSc subtype, earlier SSc diagnosis, less organic involvement and more intensive treatment than pre-2003 cohort. All these findings could explain the decline in the SRC prevalence.Figure:Table 2.Univariate analysisPre-2003 (%)Post-2003 (%)Clinical dataSkin sclerosis (as 1stsymptom)7.24.1Digital ulcers4931Telangiectasia6652Acroosteolysis124.6Calcinosis2914Joint contractures2813ILD4937Cardiac conduction alteration2027Left diastolic dysfunction4132Peripheral neuropathy136.1Sicca syndrome3724Treatment featuresCalcium channel blockers2340Specific vasodilators2.114Prostaglandin0.993.4Angiotensin system inhibitors5.116Corticosteroids1120Immunosuppressant315y: years old; lcSSc: limited cutaneous SSc; dcSSc: diffuse cutaneous SSc; ssSSc: sine scleroderma SSc; ILD: Interstitial lung disease; PAPs: systolic Pulmonary Arterial Pressure; VCS: Video-capillaroscopy; dx: diagnosisDisclosure of Interests:Xavier Pla Salas: None declared, Carles Tolosa Consultant of: Actelion pharmaceuticals, GSK, MSD., Alfredo Guillén del Castillo: None declared, María Esther Sánchez García: None declared, Jorge Sánchez-Redondo: None declared, Eduardo L. Callejas-Moraga: None declared, Luis Sáez-Comet: None declared, Jose Antonio Vargas-Hitos: None declared, Jose Antonio Todolí Parra: None declared, Luis Trapiella Martínez: None declared, Ignasi Rodriguez-Pubto: None declared, Mayka Freire: None declared, Isaac Pons Martin del Campo: None declared, Vicent Fonollosa-Pla Consultant of: Actelion pharmaceuticals, GSK, MSD., Carmen Pilar Simeón-Aznar Consultant of: Actelion pharmaceuticals, GSK, MSD., on behalf of RESCLE Investigators, Autoimmune Diseases Study Group (GEAS): None declared
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