Anti-synthetase syndrome: anti-PL-7, anti-PL-12 and anti-EJ

Souza, Fernando Henrique Carlos de; Cruellas, Marcela Gran Pina; Levy‐Neto, Maurício; Shinjo, Samuel Katsuyuki

doi:10.1016/s2255-5021(13)70048-8

Cited by 6 publications

(3 citation statements)

References 28 publications

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“…A síndrome antissintetase (SAS) é uma doença autoimune sistêmica caracterizada pela presença de miosite, artrite e pneumopatia intersticial, assim como febre, fenômeno de Raynaud e "mãos de mecânico" (CAVAGNA et al, 2015;Da Silva LMB et al, 2022;KATZAP et al, 2011;SHINJO et al, 2010;SOUZA et al, 2013;BEHRENS PINTO et al, 2020;BACCARO et al, 2020). Laboratorialmente, a doença é caracterizada pela presença de autoanticorpos Anti-aminoacil-tRNA sintetases (Anti-ARS), como Anti-Jo-1, -PL-7, -PL-12, -OJ, -EJ, -KS, -Zo e -Ha (KATZAP et al, 2011;CAVAGNA et al, 2019).…”

Section: Conceito De Síndrome Antissintetaseunclassified

“…-PL-7, -PL-12, -OJ, -EJ, -KS, -Zo e -Ha) apresentam maior heterogeneidade nas manifestações clínicas, especificamente no comprometimento muscular (BACCARO et al, 2020). Por exemplo, Anti-PL-7 e Anti-PL-12 estão mais associados a pneumopatia intersticial e menor incidência de miosite (SOUZA et al,2013), semelhante ao que se observa com o autoanticorpo Anti-KS (COJOCARU et al,2016). A presença de Anti-EJ e Anti-OJ tem sido relacionada em associação com miosite (IMBERT-MASSEAU et al, 2003), mas sua prevalência em SAS é baixa (IMBERT-MASSEAU et al,2003).…”

Section: Conceito De Síndrome Antissintetaseunclassified

“…A prevalência de miosite em SAS é estimada em 73% -94%. Apesar disso, a manifestação muscular geralmente não está presente no início da doença e pode se manifestar ao longo dos anos (SHINJO et al, 2010;SOUZA et al, 2013;HERVIER et al, 2013), dificultando o diagnóstico. A fraqueza muscular em pacientes com SAS tem um amplo espectro fenotípico, variando desde pacientes assintomáticos, com aumento da creatinofosfoquinase (CPK) ou mialgia, a pacientes acamados com fraqueza muscular grave (HERVIER et al, 2013).…”

Section: Conceito De Síndrome Antissintetaseunclassified

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Alta prevalência de padrão de miopatia necrosante em biópsias musculares de pacientes com síndrome antissintetase com autoanticorpos Anti-Jo-1 positivo

Silva¹

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Ao meu amado esposo Júlio César e ao nosso futuro filho, Heitor, obrigada pela paciência nas horas de estudos, pelo companheiro, carinho e afeto. A vocês dedico todo o meu amor.Ao meu amado pai, meu guia que tanto estimulou e despertou minha paixão pelo conhecimento, lhe dedico todo meu carinho e respeito.À minha mãe querida, que segurou minhas mãos e guiou meus passos, obrigada por todo o amor e dedicação.Ao meu amado irmão, que mesmo não estando mais fisicamente entre nós, sempre vibrou por cada uma de minhas vitórias e conquistas, obrigada por estar comigo em todos os momentos.Sem vocês, nada disso seria possível! AGRADECIMENTOSAo meu orientador, Prof. Dr. Samuel Katsuyuki Shinjo, pela paciência, pelos ensinamentos, dedicação e confiança. Saiba que é um exemplo para mim não apenas como professor, mas como uma pessoa ímpar, que levarei comigo por toda a vida.À querida Isabela Borges, que me auxiliou e participou tão ativamente de todo o processo de desenvolvimento do trabalho atual, obrigada pelo carinho, pelos ensinamentos e pela paciência nos dias de laboratório.Aos professores, colegas e funcionários do Serviço de Reumatologia e do

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Section: Conceito De Síndrome Antissintetaseunclassified

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Alta prevalência de padrão de miopatia necrosante em biópsias musculares de pacientes com síndrome antissintetase com autoanticorpos Anti-Jo-1 positivo

Silva¹

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Anti-tRNA synthetase syndrome interstitial lung disease: A single center experience

Wilfong

Young-Glazer

Sohn

et al. 2021

Preprint

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Background: Recognition of Anti tRNA synthetase (ARS) related interstitial lung disease (ILD) is key to ensuring patients have prompt access to immunosuppressive therapies. The purpose of this retrospective cohort study was to identify factors that may delay recognition of ARS-ILD. Methods: Patients seen at Vanderbilt University Medical Center (VUMC) between 9/17/2017-10/31/2018 were included in this observational cohort. Clinical and laboratory features were obtained via chart abstraction. Kruskal-Wallis ANOVA, Mann-Whitney U, and Fisher's exact t tests were utilized to determine statistical significance. Results: Patients with ARS were found to have ILD in 51.9% of cases, which was comparable to the frequency of ILD in systemic sclerosis (59.5%). The severity of FVC reduction in ARS (53.2%) was comparable to diffuse cutaneous systemic sclerosis (56.8%, p=0.48) and greater than dermatomyositis (66.9%, p=0.005) or limited cutaneous systemic sclerosis (lcSSc, 71.8%, p=0.005). Frank honeycombing was seen with ARS antibodies but not other myositis autoantibodies. ARS patients were more likely to first present to a pulmonary provider in a tertiary care setting (53.6%), likely due to fewer extrapulmonary manifestations. Only 33% of ARS-ILD were anti-nuclear antibody, rheumatoid factor, or anti-cyclic citrullinated peptide positive. Patients with ARS-ILD had a two-fold longer median time to diagnosis compared to other myositis-ILD patients (11.0 months, IQR 8.5 to 43 months vs. 5.0 months, IQR 3.0 to 9.0 months, p=0.003). Conclusions: ARS patients without prominent extra-pulmonary manifestations are at high risk for not being recognized as having a connective tissue disease related ILD and miscategorized as UIP/IPF without comprehensive serologies

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Demographic, clinical, laboratory data, prognostic, and treatment features of patients with antisynthetase syndrome: An international, two-center cohort study

Silva¹,

Rathore²,

Agarwal³

et al. 2022

Arch Rheumatol

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Objectives: To compare clinical, demographic, laboratory data, prognostic and treatment characteristics of patients with antisynthetase syndrome (ASSD) treated in two different centers of India and Brazil. Patients and methods: This international, two-center, retro-prospective cohort study which was conducted at two tertiary rheumatology centers (one in Brazil and one in India) between January 2000 to January 2020 included a total of 115 patients with ASSD (21 males, 94 females; mean age; at disease diagnosis at 40.3, range, 18 to 80 years). Demographic, clinical and laboratory data of the patients were recorded. Clinical involvement was evaluated. Results: Of the patients, 81 were Brazilians and 34 were of Indian origin. The Indian group exhibited a greater delay in diagnosis after the onset of symptoms compared to Brazilian patients (12 vs. 6 months, respectively; p=0.026). Brazilian patients exhibited a significantly higher prevalence of joint and lung involvement, mechanic’s hands, and Raynaud’s phenomenon. Anti-Jo-1 was the most common autoantibodies in both groups. Systemic arterial hypertension, followed by diabetes mellitus were the most prevalent comorbidities. Concerning previously used drugs, the Indian patients had a larger group of patients treated with antimalarials, whereas the Brazilian group used more azathioprine and intravenous immunoglobulin. A higher proportion of Indian patients was treated with one immunosuppressive drug (70.6%), while the Brazilian group were often treated using two immunosuppressive drugs (33%). Comparison between the severity and prognosis showed that Brazilian group had a higher number of relapses, and during follow-up, the global mortality rates were similar in both groups (6.2% for Brazilian vs. 8.8% for Indian). Conclusion: Brazilian and Indian patients with ASSD have comparable epidemiological characteristics such as age at the time of disease diagnosis, and sex distribution, and autoantibodies. Diagnostic delay is seen in Indian patients, and Brazilians exhibit a higher prevalence of joint and lung involvement, mechanic’s hands, Raynaud’s phenomenon with a higher number of relapses, although the mortality rate seems to be similar in both groups.

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Anti-synthetase syndrome: anti-PL-7, anti-PL-12 and anti-EJ

Cited by 6 publications

References 28 publications

Alta prevalência de padrão de miopatia necrosante em biópsias musculares de pacientes com síndrome antissintetase com autoanticorpos Anti-Jo-1 positivo

Alta prevalência de padrão de miopatia necrosante em biópsias musculares de pacientes com síndrome antissintetase com autoanticorpos Anti-Jo-1 positivo

Anti-tRNA synthetase syndrome interstitial lung disease: A single center experience

Demographic, clinical, laboratory data, prognostic, and treatment features of patients with antisynthetase syndrome: An international, two-center cohort study

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