Síndrome antissintetase anti-Jo-1 / Anti-Jo-1 antisynthetase syndrome

OBJETIVO: Devido à escassez de estudos populacionais, apresentamos um estudo epidemiológico em síndrome antissintetase anti-Jo-1 (SAS). PACIENTES E MÉTODOS: Estudo coorte retrospectivo realizado em um centro de 1980 a 2010. Dados clínico-laboratoriais e demográficos foram obtidos dos prontuários médicos. Todos os pacientes preenchiam critério de Bohan e Peter (1975) e apresentavam anti-Jo-1, além de envolvimento articular, muscular e pulmonar. Dezoito pacientes com SAS anti-Jo-1 foram analisados. RESULTADOS: A média de idade ao início da doença foi de 39,9 ± 15,7 anos, e a média da duração da doença, 9,7 ± 7,0 anos. Todos os pacientes eram brancos, e 94,4 por cento eram mulheres. Sintomas constitucionais ocorreram em metade dos casos. Envolvimento cutâneo e do trato gastrointestinal ocorreram, respectivamente, em 66,6 por cento e 55,6 por cento dos casos. Não houve casos de envolvimento neurológico ou cardíaco. Metade dos pacientes apresentava pneumopatia incipiente, opacidade em vidro-fosco e fibrose pulmonar basal. Houve um caso de tuberculose, três de herpes zoster e um linfoma não Hodgkin. Um óbito ocorreu devido ao choque séptico (broncopneumonia hospitalar). Todos os pacientes receberam prednisona (1mg/kg/dia) e 12 (66,7 por cento) receberam pulsoterapia com metil prednisolona (1 g/dia, 3 dias). Diferentes imunossupressores foram utilizados como poupadores de corticosteroide, dependendo da tolerância, efeitos colaterais e/ou refratariedade da doença. Embora a recidiva da doença (clínica e/ou laboratorial) tenha ocorrido em 87,5 por cento dos casos, 12 dos 16 pacientes (75 por cento) estavam com a remissão da doença no desfecho do presente estudo. CONCLUSÃO: A maioria dos pacientes eram mulheres brancas e com alta taxa de recidiva da doença.

OBJECTIVE: Given a lack of population-based studies, we report an epidemiological-clinic study of anti-Jo-1 antisynthetase syndrome (ASS). PATIENTS AND METHODS: To study a retrospective cohort of a single-center from 1980 to 2010. Clinical-laboratory and demographic data were obtained from medical files. All patients fulfilled the Bohan and Peter criteria (1975) and presented anti-Jo-1, articular, muscle and lung involvement. Eighteen patients with anti-Jo-1 ASS were analyzed. RESULTS: The mean age at disease onset was 39.9 ± 15.7 years and average disease duration was 9.7 ± 7.0 years. All subjects were white, and 94.4 percent were female. Constitutional symptoms occurred in 50 percent of cases. There was cutaneous and gastrointestinal tract involvement in 66.6 percent and 55.6 percent of cases, respectively. No cases manifested neurologic or cardiac involvement. Half of the patients showed incipient pneumopathy, ground-glass opacities and basal pulmonary fibrosis. There was one case of tuberculosis, three of herpes zoster and one of non-Hodgkin lymphoma. One death occurred due to sepsis shock (hospital bronchopneumonia). All patients received prednisone (1mg/kg/day) and 12 (66.7 percent) participants received methyl prednisolone pulse therapy (1g/day, 3 days). Various immunosuppressants were used as corticosteroid tapers, depending on tolerance, side effects and/or refractoriness. Although disease relapse (clinical and/or laboratory) occurred in 87.5 percent of cases, 12 out of 16 patients (75 percent) were in disease remission at study endpoint. CONCLUSION: In the present study, almost all patients were white females and the disease relapse rate was high.

Prokaryotic expression and preparation of polyantibody of human histydyl-tRNA synthetase related gene / 华中科技大学学报（医学）（英德文版）

The aim of this study was to express and purify human histydyl-tRNA synthetase related gene and to prepare its polyantibody. The open reading frame was amplified by PCR, and then recombined into prokaryotic expression vector pQE30 and transformed into E. coli M15 for expression. The expressed products were induced by IPTG after the reconstructed pQE30 was transferred into M15. After purified by Ni affinity chromatography, the product was identified to be a single band by SDS-PAGE. The rabbits were inoculated with purified products. High-titer polyantibody was successfully prepared. Highly-purified expression product and prepared polyantibody may provide a good basis for further study.

Successful Combination Therapy of Cyclosporine and Methotrexate for Refractory Polymyositis with Anti-Jo-1 Antibody: A Case Report

Although corticosteroids have been the initial agent for the treatment of inflammatory myopathies (IM), immunosuppressive agents such as azathioprine, methotrexate, cyclophosphamide, or cyclosporine are commonly required to control the disease except mild cases. On the other hand, the efficacy of combination therapy of cyclosporine and methotrexate in severe rheumatoid arthritis has been proven without serious side effects. However, in treatment-resistant myositis, the experience of such a therapy is very limited, and has not been described in refractory polymyositis with anti-Jo-1 antibody. Here, we report a young female patient with recalcitrant polymyositis and anti-Jo-1 antibody who was successfully treated with the combination therapy of cyclosporine and methotrexate. At first, the myositis did not respond to several agents, such as corticosteroid, monthly pulse cyclophosphamide, azathioprine, or cyclosporine. Methotrexate was initially avoided as treatment regimen because of its potential pulmonary toxicity in the case with preexisting lung disease.