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• W3092020382 abstract "The aim of the study was to investigate the demographic and clinical characteristics of Venezuelan patients with systemic sclerosis (SSc) seen in a tertiary hospital. Consecutive patients 18 years and older who fulfilled the 2013 ACR/EULAR classification criteria for SSc and who were followed up in the outpatient clinic of the Division of Rheumatology at the Hospital Universitario de Caracas were selected for the study. Demographic and clinical variables were registered at the time of inclusion using a standard protocol. Forty-eight SSc patients were included; 46 (95.8%) were female; the mean age was 55.1 ± 13.7 (mean ± SD) years and all were of Hispanic ethnicity. Thirty-one (64.6%) had limited SSc and 17 (35.4%) had diffuse SSc. The mean duration of disease was 13.4 ± 11.7 (mean ± SD) years, 16.74 ± 12.99 years for limited SSc and 7.52 ± 5.25 years for diffuse SSc (p = 0.0077). Raynaud's phenomenon was the most frequent manifestation (100%), followed by arthritis (68.8%), telangiectasia (60.4%), dyspnea (60.4%), dysphagia (58.3%) and puffy hands (56.3%). The modified Rodnan Skin Score (mRSS) and the frequency of dyspnea were higher in those with diffuse as compared to limited SSc (p = 0.0211 and p = 0.0003, respectively). We performed high-resolution computed tomography (HRCT) of the lungs in 31 patients; 14 (45.2%) had evidence of interstitial lung disease (ILD), 11 (68.8%) with diffuse SSc (p = 0.0052). The most frequent anti-nuclear antibody pattern was nucleolar, accounting for 18 (42.8%) of the cases. Anti-centromere antibodies were present in 16.7% of the cases and were associated with the limited SSc subset (p = 0.0443) and with calcinosis (p = 0.0020). Anti-topoisomerase antibodies were associated with ILD (p = 0.0077). Typical clinical and serological manifestations were present in this sample of Venezuelan patients with SSc, with an expected distribution according to disease subtype. The autoantibody profile allows clinicians to identify those patients with limited forms of the disease and those without pulmonary involvement. El objetivo de este estudio fue investigar las características demográficas y clínicas de los pacientes venezolanos con esclerosis sistémica (ES) examinados en un hospital terciario. Se seleccionaron para el estudio pacientes consecutivos mayores de 18 años, que cumplieron los criterios de la clasificación ACR/EULAR de 2013 para ES, sometidos a seguimiento en la clínica ambulatoria del Departamento de Reumatología del Hospital Universitario de Caracas. Se registraron las variables demográficas y clínicas en el momento de la inclusión, utilizando un protocolo estándar. Se incluyó a 48 pacientes con ES, de los cuales 46 (95,8%) eran mujeres; la edad media fue de 55,1 ± 13,7 (media ± DE) años, todos ellos de etnia hispana. Treinta y uno de ellos (64,6%) tenían ES limitada y 17 (35,4%) ES difusa. La duración media de la enfermedad fue de 13,4 ± 11,7 (media ± DE) años: 16,74 ± 12,99 años para ES limitada y 7,52 ± 5,25 años para ES difusa (p = 0,0077). El fenómeno de Raynaud fue la manifestación más frecuente (100%), seguida de la artritis (68,8%), la telangiectasia (60,4%), la disnea (60,4%), la disfagia (58,3%) y la hinchazón de manos (56,3%). La puntuación de la escala cutánea de Rodnan modificada (mRSS) y la frecuencia de la disnea fueron más altas en aquellos sujetos con ES difusa con respecto a los sujetos con ES limitada (p = 0,0211 y p = 0,0003, respectivamente). Realizamos una TAC de alta resolución pulmonar a 31 pacientes; 14 (45,2%) tenían evidencia de enfermedad pulmonar intersticial (EPI), 11 (68,8%) de ellos con ES difusa (p = 0,0052). El patrón más frecuente de anticuerpos anti-nucleares fue de tipo nucleolar, representando 18 (42,8%) de los casos. Los anticuerpos anti-centrómeros estuvieron presentes en el 16,7% de los casos, asociándose al subconjunto de ES limitada (p = 0,0443) con calcinosis (p = 0,0020). Los anticuerpos anti-topoisomerasa estuvieron asociados a la EPI (p = 0,0077). Las manifestaciones clínicas y serológicas típicas estuvieron presentes en esta muestra de pacientes venezolanos con ES, con una distribución prevista con arreglo al subtipo de la enfermedad. El perfil de anticuerpos permite a los clínicos identificar a aquellos pacientes con formas limitadas de la enfermedad, y aquellos con compromiso pulmonar." @default.
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• W3092020382 date "2021-12-01" @default.
• W3092020382 modified "2023-10-15" @default.
• W3092020382 title "Characterization of Venezuelan patients with systemic sclerosis: a study in a tertiary hospital in Caracas" @default.
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• W3092020382 doi "https://doi.org/10.1016/j.reuma.2020.06.006" @default.
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