THU0361 EPIDEMIOLOGIC VARIATION ON SCLERODERMA RENAL CRISIS AND CLINICAL FEATURES VARIATION ON SYSTEMIC SCLEROSIS PATIENTS OVER TIME: DATA FROM RESCLE REGISTRY.
Authors: X. Pla Salas, C. Tolosa, A. Guillén del Castillo, M. E. Sánchez García, J. Sánchez-Redondo, E. L. Callejas-Moraga, L. Sáez-Comet, J. A. Vargas-Hitos, J. A. Todolí Parra, L. Trapiella Martínez, I. Rodriguez-Pubto, M. Freire, I. Pons Martin del Campo, V. Fonollosa-Pla, C. P. Simeón-Aznar
Journal: Annals of the Rheumatic Diseases (2020)

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 (R egistro de ESCLE rodermia) 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 analysis Pre-2003(% ) Post-2003(% ) Demographic data lcSSc 63 59 dcSSc 28 15 ssSSc 6.4 14 Early SSc 1.1 3.3 Very early SSc 1.9 9.6 Age at SSc dx 49.1(±15.2)y 55.0(±15.6)y Time from SSc dx to SRC 1.1(0.2-4.3)y 0.6(0.1-1.5)y ACR/EULAR 2013 criteria 99 86 Comorbidities Smoked 20 37 Arterial hypertension 35 29 Diagnostic procedures PAPs &gt;40mmHg by Echocardiography 38 25 Not-sclerodermal pattern at VCS 8.9 19 Lupus anticoagulant 12 4.8 Prognostic features Overall mortality 29 11 ILD-related death 10 1.6 Conclusion: 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 analysis Pre-2003 (% ) Post-2003 (% ) Clinical data Skin sclerosis (as 1 st symptom) 7.2 4.1 Digital ulcers 49 31 Telangiectasia 66 52 Acroosteolysis 12 4.6 Calcinosis 29 14 Joint contractures 28 13 ILD 49 37 Cardiac conduction alteration 20 27 Left diastolic dysfunction 41 32 Peripheral neuropathy 13 6.1 Sicca syndrome 37 24 Treatment features Calcium channel blockers 23 40 Specific vasodilators 2.1 14 Prostaglandin 0.99 3.4 Angiotensin system inhibitors 5.1 16 Corticosteroids 11 20 Immunosuppressant 3 15 y: 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: diagnosis Disclosure 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