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Vol. 17. Issue 10.
Pages 601-606 (December 2021)
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Vol. 17. Issue 10.
Pages 601-606 (December 2021)
Original Article
Characterization of Venezuelan patients with systemic sclerosis: a study in a tertiary hospital in Caracas
Caracterización de los pacientes venezolanos con esclerosis sistémica: estudio en un hospital terciario en Caracas
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Daniel Rivas-Vargasa,
Corresponding author
rivasvargas4@gmail.com

Corresponding author.
, Soham Al Snihb, Martín A. Rodrígueza,c
a Centro Nacional de Enfermedades Reumáticas, Hospital Universitario de Caracas, Caracas, Venezuela
b Division of Rehabilitation Sciences/School of Health Professions, Division of Geriatrics/Department of Internal Medicine, University of Texas Medical Branch, Galveston, TX, United States
c Sealy Center on Aging, University of Texas Medical Branch, Galveston, TX, United States
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Tables (3)
Table 1. Sociodemographic characteristics of Venezuelan patients with systemic sclerosis.
Table 2. Clinical characteristics of Venezuelan patients with systemic sclerosis.
Table 3. Distribution of autoantibodies in Venezuelan patients with systemic sclerosis.
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Abstract
Objective

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.

Methods

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.

Results

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).

Conclusions

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.

Keywords:
Systemic sclerosis
Epidemiology
Demography
Hispanic
Resumen
Objetivo

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.

Métodos

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.

Resultados

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).

Conclusiones

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.

Palabras clave:
Esclerosis sistémica
Epidemiología
Demografía
Hispanos

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