Maturu Venkata Nagarjuna, Lakshman Arjun, Bal Amanjit, Dhir Varun, Sharma Aman, Garg Mandeep, Saikia Biman, Agarwal Ritesh
Department of Pulmonary Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh, Haryana and Punjab, India.
Department of Internal Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh, Haryana and Punjab, India.
Lung India. 2016 Jan-Feb;33(1):20-6. doi: 10.4103/0970-2113.173055.
Antisynthetase syndrome (AS) is an uncommon and under-recognised connective tissue disease characterized by the presence of antibodies to anti-aminoacyl t-RNA synthetase along with features of interstitial lung disease (ILD), myositis and arthritis. The aim of the current study is to describe our experience with management of AS.
This was a 2-year (2013-2014) retrospective analysis of patients diagnosed with anti-Jo-1-related AS. The presence of anti-Jo-1 antibody was tested by the immunoblot assay. All patients underwent high-resolution computed tomography of the chest, transthoracic echocardiography and evaluation for inflammatory myositis. Transbronchial lung biopsies and muscle biopsies were obtained when clinically indicated.
Nine patients (mean age: 43.8 years) were diagnosed with anti-Jo-1-related AS. The median duration of symptoms before diagnosis of AS was 6 months. All patients were negative for antinuclear antibodies by indirect immunofluorescence. The prevalence of ILD, myositis and arthritis at presentation was 100%, 77.8% and 55.6%, respectively. The most common ILD pattern was non-specific interstitial pneumonia (n = 6) followed by organizing pneumonia (n = 2) and usual interstitial pneumonia (n = 1). ILD was the sole manifestation in two patients and was subclinical in two patients. Six patients had pleuropericardial effusions, three patients had pulmonary artery hypertension and two patients had venous thromboembolism. Eight of the nine patients improved after treatment with steroids and other immunosuppressants.
Antisynthetase syndrome is an important and a treatable cause of ILD. Strong clinical suspicion is needed to achieve an early diagnosis.
抗合成酶综合征（AS）是一种罕见且未得到充分认识的结缔组织病，其特征为存在抗氨酰t - RNA合成酶抗体，并伴有间质性肺病（ILD）、肌炎和关节炎的表现。本研究的目的是描述我们在AS管理方面的经验。
这是一项对2013 - 2014年诊断为抗Jo - 1相关AS患者的为期2年的回顾性分析。通过免疫印迹法检测抗Jo - 1抗体的存在。所有患者均接受了胸部高分辨率计算机断层扫描、经胸超声心动图检查以及炎性肌炎评估。在临床指征明确时进行经支气管肺活检和肌肉活检。
9例患者（平均年龄：43.8岁）被诊断为抗Jo - 1相关AS。AS诊断前症状的中位持续时间为6个月。通过间接免疫荧光法检测，所有患者的抗核抗体均为阴性。发病时ILD、肌炎和关节炎的患病率分别为100%、77.8%和55.6%。最常见的ILD类型是非特异性间质性肺炎（n = 6），其次是机化性肺炎（n = 2）和普通间质性肺炎（n = 1）。ILD是2例患者的唯一表现，2例患者为亚临床状态。6例患者有胸膜心包积液，3例患者有肺动脉高压，2例患者有静脉血栓栓塞。9例患者中有8例在接受类固醇和其他免疫抑制剂治疗后病情改善。
抗合成酶综合征是ILD的一个重要且可治疗的病因。需要高度的临床怀疑才能实现早期诊断。
