Hegde Arun, Marwah Vikas, V Shrinath, Choudhary Robin, Malik Virendra
Department of Rheumatology, Command Hospital, Lucknow, India.
Department of Respiratory Medicine, AICTS, Pune, India.
Mediterr J Rheumatol. 2022 Dec 31;33(4):437-443. doi: 10.31138/mjr.33.4.437. eCollection 2022 Dec.
Anti-Jo1 syndrome is one of the most common amongst the various anti synthetase syndromes (ASS), which forms a subgroup of the idiopathic inflammatory myositis (IIM). It is characterised by myositis, interstitial lung disease (ILD), fever, Raynaud's phenomenon, and mechanic's hands; associated with the presence of anti-Jo1 antibodies in serum. Being an orphan disease, the clinical diagnosis is often delayed.
In this retrospective study, all patients diagnosed as Anti-Jo1 syndrome, from two tertiary care hospitals in Western Maharashtra, between 01 January 2019 - 31 December 2020, were enrolled. The parameters studied included demographic data, clinical features at presentation, laboratory parameters, spirometry, and radiographic findings, along with treatment instituted.
A total of 17 patients (8 males, 9 females) qualified for inclusion in the study. The mean age of diagnosis was 40 (±13) years with mean time to diagnosis being 2 years (± 0.6 years), from first clinical presentation. The most common presenting symptoms encountered were arthritis (n = 12, 70.5%), fever (n = 16, 70.5%), myositis (n=11, 64.7%) and breathlessness (n=10, 58.8%).10 patients had ILD at presentation on high resolution computerised tomography of chest (n=10, 58.8%) with restrictive lung defect on spirometry. Six patients required induction of immunosuppression using pulse methylprednisolone (n=6) and Rituximab (n=6), while 11 were managed with oral steroids. Mycophenolate mofetil (n=10) and Azathioprine (n=7) were used as maintenance immunosuppression.
Anti-Jo1 syndrome is a myositis syndrome, presenting with a multitude of clinical features. Steroids and disease modifying anti rheumatic drugs form mainstay of therapy.
抗 Jo1 综合征是各种抗合成酶综合征（ASS）中最常见的一种，抗合成酶综合征是特发性炎性肌病（IIM）的一个亚组。其特征为肌炎、间质性肺病（ILD）、发热、雷诺现象和技工手；血清中存在抗 Jo1 抗体。作为一种罕见病，临床诊断往往会延迟。
在这项回顾性研究中，纳入了 2019 年 1 月 1 日至 2020 年 12 月 31 日期间在西马哈拉施特拉邦的两家三级医疗机构被诊断为抗 Jo1 综合征的所有患者。研究的参数包括人口统计学数据、就诊时的临床特征、实验室参数、肺功能测定和影像学检查结果，以及所采取的治疗措施。
共有 17 名患者（8 名男性，9 名女性）符合纳入本研究的标准。诊断时的平均年龄为 40（±13）岁，从首次临床表现到诊断的平均时间为 2 年（±0.6 年）。最常见的首发症状为关节炎（n = 12，70.5%）、发热（n = 16，70.5%）、肌炎（n = 11，64.7%）和呼吸困难（n = 10，58.8%）。10 例患者在胸部高分辨率计算机断层扫描时表现为间质性肺病（n = 10，58.8%），肺功能测定显示为限制性肺缺陷。6 例患者需要使用静脉注射甲泼尼龙（n = 6）和利妥昔单抗（n = 6）进行免疫抑制诱导，而 11 例患者采用口服类固醇治疗。霉酚酸酯（n = 10）和硫唑嘌呤（n = 7）用作维持免疫抑制治疗。
抗 Jo1 综合征是一种肌炎综合征，具有多种临床特征。类固醇和改善病情的抗风湿药物是治疗的主要手段。
