Author(s): Sakamoto, Ana Paula ; Silva, Clovis Artur ; Castro da Silva, Marco Felipe ; Lopes, Anandreia Simoes ; Souza Russo, Gleice Clemente ; Elias Sallum, Adriana Maluf ; Kozu, Katia ; Bonfa, Eloisa ; Saad-Magalhaes, Claudia [UNESP] ; Rodrigues Pereira, Rosa Maria ; Len, Claudio Arnaldo ; Terreri, Maria Teresa
Date: 2018
Persistent ID: http://hdl.handle.net/11449/163567
Origin: Oasisbr
Subject(s): Digital vasculitis; Childhood-onset systemic lupus erythematosus; Vasculitis; Sledai-2K
Made available in DSpace on 2018-11-26T17:42:33Z (GMT). No. of bitstreams: 0 Previous issue date: 2017-11-01
Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq)
Federico Foundation
Nucleo de Apoio a Pesquisa Saude da Crianca e do Adolescente of USP (NAP-CriAd)
Objectives: To assess clinical digital vasculitis (DV) as an initial manifestation of childhood onset systemic lupus erythematosus (cSLE) within a large population. Methods: Multicenter cross-sectional study including 852 cSLE patients (ACR criteria) followed in ten Pediatric Rheumatology centers in Sao Paulo State, Brazil. Results: DV was observed in 25/852 (3%) cSLE patients. Periungual hemorrhage was diagnosed in 12 (48%), periungual infarction in 7 (28%), tip finger ulceration in 4 (16%), painful nodules in 1 (4%) and gangrene in 1 (4%). A poor outcome, with digital resorption, occurred in 5 (20%). Comparison of patients with and without DV revealed higher frequency of malar rash (80% vs. 53%, p = 0.008), discoid rash (16% vs. 4%, p = 0.017), photosensitivity (76% vs. 45%, p = 0.002) and other cutaneous vasculitides (80% vs. 19%, p <0.0001), whereas the frequency of overall constitutional features (32% vs. 61%, p = 0.003), fever (32% vs. 56%, p = 0.020) and hepatomegaly (4% vs. 23%, p = 0.026) were lower in these patients. Frequency of female gender, severe multi-organ involvement, autoantibodies profile and low complement were alike in both groups (p >0.05). SLEDAI-2K median, DV descriptor excluded, was significantly lower in patients with DV compared to those without this manifestation [10 (0-28) vs. 14 (0-58), p = 0.004]. Visceral vasculitis or death were not observed in this cSLE cohort. The frequency of cyclophosphamide use (0% vs. 18%, p = 0.014) was significantly lower in the DV group. Conclusion: Our large multicenter study identified clinical DV as one of the rare initial manifestation of active cSLE associated with a mild multisystemic disease, in spite of digital resorption in some of these patients. (C) 2017 Published by Elsevier Editora Ltda.
Univ Fed Sao Paulo UNIFESP, Unidade Reumatol Pediat, Sao Paulo, SP, Brazil
Univ Sao Paulo, Fac Med, Unidade Reumatol Pediat, Sao Paulo, SP, Brazil
Univ Sao Paulo, Fac Med, Div Reumatol, Sao Paulo, SP, Brazil
Univ Estadual Paulista, UNESP, Fac Med Botucatu, Hosp Clin Botucatu, Botucatu, SP, Brazil
Univ Estadual Paulista, UNESP, Fac Med Botucatu, Hosp Clin Botucatu, Botucatu, SP, Brazil
CNPq: CNPq 303422/2015-7
CNPq: 301805/2013-0
CNPq: 305068/2014-8
CNPq: 301479/2015
CNPq: 303752/2015-7
