TY - JOUR
T1 - Characterization of Venezuelan patients with systemic sclerosis
T2 - a study in a tertiary hospital in Caracas
AU - Rivas-Vargas, Daniel
AU - Snih, Soham Al
AU - Rodríguez, Martín A.
N1 - Publisher Copyright:
© 2020 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología
PY - 2021/12
Y1 - 2021/12
N2 - 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.
AB - 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.
KW - Demography
KW - Epidemiology
KW - Hispanic
KW - Systemic sclerosis
UR - http://www.scopus.com/inward/record.url?scp=85092219009&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85092219009&partnerID=8YFLogxK
U2 - 10.1016/j.reuma.2020.06.006
DO - 10.1016/j.reuma.2020.06.006
M3 - Article
C2 - 33039312
AN - SCOPUS:85092219009
SN - 1699-258X
VL - 17
SP - 601
EP - 606
JO - Reumatologia Clinica
JF - Reumatologia Clinica
IS - 10
ER -