Vasculite digital inicial em uma grande coorte multicêntrica de pacientes com lúpus eritematoso sistêmico de início na infância
Autor(a) principal: | |
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Data de Publicação: | 2017 |
Outros Autores: | , , , , , , , , , , |
Tipo de documento: | Artigo |
Idioma: | eng por |
Título da fonte: | Repositório Institucional da UNESP |
Texto Completo: | http://dx.doi.org/10.1016/j.rbr.2017.05.008 http://hdl.handle.net/11449/175511 |
Resumo: | 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 São 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‐2 K 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. |
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Vasculite digital inicial em uma grande coorte multicêntrica de pacientes com lúpus eritematoso sistêmico de início na infânciaInitial digital vasculitis in a large multicenter cohort of childhood‐onset systemic lupus erythematosusChildhood‐onset systemic lupus erythematosusDigital vasculitisSledai‐2 KVasculitisObjectives 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 São 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‐2 K 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.Universidade Federal de São Paulo (Unifesp) Unidade de Reumatologia PediátricaUniversidade de São Paulo (USP) Faculdade de Medicina Unidade de Reumatologia PediátricaUniversidade de São Paulo (USP) Faculdade de Medicina Divisão de ReumatologiaUniversidade Estadual Paulista (Unesp) Faculdade de Medicina de Botucatu Hospital das Clínicas de BotucatuUniversidade Estadual Paulista (Unesp) Faculdade de Medicina de Botucatu Hospital das Clínicas de BotucatuUniversidade Federal de São Paulo (UNIFESP)Universidade de São Paulo (USP)Universidade Estadual Paulista (Unesp)Sakamoto, Ana PaulaSilva, Clovis ArturSilva, Marco Felipe Castro daLopes, Anandreia SimõesRusso, Gleice Clemente SouzaSallum, Adriana Maluf EliasKozu, KatiaBonfá, EloisaSaad‐Magalhães, Claudia [UNESP]Pereira, Rosa Maria RodriguesLen, Claudio ArnaldoTerreri, Maria Teresa2018-12-11T17:16:07Z2018-12-11T17:16:07Z2017-11-01info:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/article583-589application/pdfhttp://dx.doi.org/10.1016/j.rbr.2017.05.008Revista Brasileira de Reumatologia, v. 57, n. 6, p. 583-589, 2017.1809-45700482-5004http://hdl.handle.net/11449/17551110.1016/j.rbr.2017.05.0082-s2.0-850345894172-s2.0-85034589417.pdf2-s2.0-85034589417.pdfs2.0-S0482500417309026.pdfScopusreponame:Repositório Institucional da UNESPinstname:Universidade Estadual Paulista (UNESP)instacron:UNESPengporRevista Brasileira de Reumatologia0,340info:eu-repo/semantics/openAccess2023-11-09T06:13:57Zoai:repositorio.unesp.br:11449/175511Repositório InstitucionalPUBhttp://repositorio.unesp.br/oai/requestopendoar:29462023-11-09T06:13:57Repositório Institucional da UNESP - Universidade Estadual Paulista (UNESP)false |
dc.title.none.fl_str_mv |
Vasculite digital inicial em uma grande coorte multicêntrica de pacientes com lúpus eritematoso sistêmico de início na infância Initial digital vasculitis in a large multicenter cohort of childhood‐onset systemic lupus erythematosus |
title |
Vasculite digital inicial em uma grande coorte multicêntrica de pacientes com lúpus eritematoso sistêmico de início na infância |
spellingShingle |
Vasculite digital inicial em uma grande coorte multicêntrica de pacientes com lúpus eritematoso sistêmico de início na infância Sakamoto, Ana Paula Childhood‐onset systemic lupus erythematosus Digital vasculitis Sledai‐2 K Vasculitis |
title_short |
Vasculite digital inicial em uma grande coorte multicêntrica de pacientes com lúpus eritematoso sistêmico de início na infância |
title_full |
Vasculite digital inicial em uma grande coorte multicêntrica de pacientes com lúpus eritematoso sistêmico de início na infância |
title_fullStr |
Vasculite digital inicial em uma grande coorte multicêntrica de pacientes com lúpus eritematoso sistêmico de início na infância |
title_full_unstemmed |
Vasculite digital inicial em uma grande coorte multicêntrica de pacientes com lúpus eritematoso sistêmico de início na infância |
title_sort |
Vasculite digital inicial em uma grande coorte multicêntrica de pacientes com lúpus eritematoso sistêmico de início na infância |
author |
Sakamoto, Ana Paula |
author_facet |
Sakamoto, Ana Paula Silva, Clovis Artur Silva, Marco Felipe Castro da Lopes, Anandreia Simões Russo, Gleice Clemente Souza Sallum, Adriana Maluf Elias Kozu, Katia Bonfá, Eloisa Saad‐Magalhães, Claudia [UNESP] Pereira, Rosa Maria Rodrigues Len, Claudio Arnaldo Terreri, Maria Teresa |
author_role |
author |
author2 |
Silva, Clovis Artur Silva, Marco Felipe Castro da Lopes, Anandreia Simões Russo, Gleice Clemente Souza Sallum, Adriana Maluf Elias Kozu, Katia Bonfá, Eloisa Saad‐Magalhães, Claudia [UNESP] Pereira, Rosa Maria Rodrigues Len, Claudio Arnaldo Terreri, Maria Teresa |
author2_role |
author author author author author author author author author author author |
dc.contributor.none.fl_str_mv |
Universidade Federal de São Paulo (UNIFESP) Universidade de São Paulo (USP) Universidade Estadual Paulista (Unesp) |
dc.contributor.author.fl_str_mv |
Sakamoto, Ana Paula Silva, Clovis Artur Silva, Marco Felipe Castro da Lopes, Anandreia Simões Russo, Gleice Clemente Souza Sallum, Adriana Maluf Elias Kozu, Katia Bonfá, Eloisa Saad‐Magalhães, Claudia [UNESP] Pereira, Rosa Maria Rodrigues Len, Claudio Arnaldo Terreri, Maria Teresa |
dc.subject.por.fl_str_mv |
Childhood‐onset systemic lupus erythematosus Digital vasculitis Sledai‐2 K Vasculitis |
topic |
Childhood‐onset systemic lupus erythematosus Digital vasculitis Sledai‐2 K Vasculitis |
description |
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 São 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‐2 K 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. |
publishDate |
2017 |
dc.date.none.fl_str_mv |
2017-11-01 2018-12-11T17:16:07Z 2018-12-11T17:16:07Z |
dc.type.status.fl_str_mv |
info:eu-repo/semantics/publishedVersion |
dc.type.driver.fl_str_mv |
info:eu-repo/semantics/article |
format |
article |
status_str |
publishedVersion |
dc.identifier.uri.fl_str_mv |
http://dx.doi.org/10.1016/j.rbr.2017.05.008 Revista Brasileira de Reumatologia, v. 57, n. 6, p. 583-589, 2017. 1809-4570 0482-5004 http://hdl.handle.net/11449/175511 10.1016/j.rbr.2017.05.008 2-s2.0-85034589417 2-s2.0-85034589417.pdf 2-s2.0-85034589417.pdf s2.0-S0482500417309026.pdf |
url |
http://dx.doi.org/10.1016/j.rbr.2017.05.008 http://hdl.handle.net/11449/175511 |
identifier_str_mv |
Revista Brasileira de Reumatologia, v. 57, n. 6, p. 583-589, 2017. 1809-4570 0482-5004 10.1016/j.rbr.2017.05.008 2-s2.0-85034589417 2-s2.0-85034589417.pdf s2.0-S0482500417309026.pdf |
dc.language.iso.fl_str_mv |
eng por |
language |
eng por |
dc.relation.none.fl_str_mv |
Revista Brasileira de Reumatologia 0,340 |
dc.rights.driver.fl_str_mv |
info:eu-repo/semantics/openAccess |
eu_rights_str_mv |
openAccess |
dc.format.none.fl_str_mv |
583-589 application/pdf |
dc.source.none.fl_str_mv |
Scopus reponame:Repositório Institucional da UNESP instname:Universidade Estadual Paulista (UNESP) instacron:UNESP |
instname_str |
Universidade Estadual Paulista (UNESP) |
instacron_str |
UNESP |
institution |
UNESP |
reponame_str |
Repositório Institucional da UNESP |
collection |
Repositório Institucional da UNESP |
repository.name.fl_str_mv |
Repositório Institucional da UNESP - Universidade Estadual Paulista (UNESP) |
repository.mail.fl_str_mv |
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1797789658638188544 |