Initial digital vasculitis in a large multicenter cohort of childhood-onset systemic lupus erythematosus
Autor(a) principal: | |
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Data de Publicação: | 2017 |
Outros Autores: | , , , , , , , , , , |
Tipo de documento: | Artigo |
Idioma: | eng |
Título da fonte: | Revista Brasileira de Reumatologia (Online) |
Texto Completo: | http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0482-50042017000600583 |
Resumo: | Abstract 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-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. |
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Initial digital vasculitis in a large multicenter cohort of childhood-onset systemic lupus erythematosusDigital vasculitisChildhood-onset systemic lupus erythematosusVasculitisSledai-2KAbstract 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-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.Sociedade Brasileira de Reumatologia2017-12-01info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersiontext/htmlhttp://old.scielo.br/scielo.php?script=sci_arttext&pid=S0482-50042017000600583Revista Brasileira de Reumatologia v.57 n.6 2017reponame:Revista Brasileira de Reumatologia (Online)instname:Sociedade Brasileira de Reumatologia (SBR)instacron:SBR10.1016/j.rbre.2017.09.002info:eu-repo/semantics/openAccessSakamoto,Ana PaulaSilva,Clovis ArturSilva,Marco Felipe Castro daLopes,Anandreia SimõesRusso,Gleice Clemente SouzaSallum,Adriana Maluf EliasKozu,KatiaBonfá,EloisaSaad-Magalhães,ClaudiaPereira,Rosa Maria RodriguesLen,Claudio ArnaldoTerreri,Maria Teresaeng2017-12-08T00:00:00Zoai:scielo:S0482-50042017000600583Revistahttp://www.scielo.br/scielo.php?script=sci_serial&pid=0482-5004&lng=pt&nrm=isoONGhttps://old.scielo.br/oai/scielo-oai.php||sbre@terra.com.br1809-45700482-5004opendoar:2017-12-08T00:00Revista Brasileira de Reumatologia (Online) - Sociedade Brasileira de Reumatologia (SBR)false |
dc.title.none.fl_str_mv |
Initial digital vasculitis in a large multicenter cohort of childhood-onset systemic lupus erythematosus |
title |
Initial digital vasculitis in a large multicenter cohort of childhood-onset systemic lupus erythematosus |
spellingShingle |
Initial digital vasculitis in a large multicenter cohort of childhood-onset systemic lupus erythematosus Sakamoto,Ana Paula Digital vasculitis Childhood-onset systemic lupus erythematosus Vasculitis Sledai-2K |
title_short |
Initial digital vasculitis in a large multicenter cohort of childhood-onset systemic lupus erythematosus |
title_full |
Initial digital vasculitis in a large multicenter cohort of childhood-onset systemic lupus erythematosus |
title_fullStr |
Initial digital vasculitis in a large multicenter cohort of childhood-onset systemic lupus erythematosus |
title_full_unstemmed |
Initial digital vasculitis in a large multicenter cohort of childhood-onset systemic lupus erythematosus |
title_sort |
Initial digital vasculitis in a large multicenter cohort of childhood-onset systemic lupus erythematosus |
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 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 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.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 Pereira,Rosa Maria Rodrigues Len,Claudio Arnaldo Terreri,Maria Teresa |
dc.subject.por.fl_str_mv |
Digital vasculitis Childhood-onset systemic lupus erythematosus Vasculitis Sledai-2K |
topic |
Digital vasculitis Childhood-onset systemic lupus erythematosus Vasculitis Sledai-2K |
description |
Abstract 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-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. |
publishDate |
2017 |
dc.date.none.fl_str_mv |
2017-12-01 |
dc.type.driver.fl_str_mv |
info:eu-repo/semantics/article |
dc.type.status.fl_str_mv |
info:eu-repo/semantics/publishedVersion |
format |
article |
status_str |
publishedVersion |
dc.identifier.uri.fl_str_mv |
http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0482-50042017000600583 |
url |
http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0482-50042017000600583 |
dc.language.iso.fl_str_mv |
eng |
language |
eng |
dc.relation.none.fl_str_mv |
10.1016/j.rbre.2017.09.002 |
dc.rights.driver.fl_str_mv |
info:eu-repo/semantics/openAccess |
eu_rights_str_mv |
openAccess |
dc.format.none.fl_str_mv |
text/html |
dc.publisher.none.fl_str_mv |
Sociedade Brasileira de Reumatologia |
publisher.none.fl_str_mv |
Sociedade Brasileira de Reumatologia |
dc.source.none.fl_str_mv |
Revista Brasileira de Reumatologia v.57 n.6 2017 reponame:Revista Brasileira de Reumatologia (Online) instname:Sociedade Brasileira de Reumatologia (SBR) instacron:SBR |
instname_str |
Sociedade Brasileira de Reumatologia (SBR) |
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SBR |
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SBR |
reponame_str |
Revista Brasileira de Reumatologia (Online) |
collection |
Revista Brasileira de Reumatologia (Online) |
repository.name.fl_str_mv |
Revista Brasileira de Reumatologia (Online) - Sociedade Brasileira de Reumatologia (SBR) |
repository.mail.fl_str_mv |
||sbre@terra.com.br |
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1750318051742449664 |