Anti-synthetase syndrome in ANA and anti-Jo-1 negative patients presenting with idiopathic interstitial pneumonia
Anti-synthetase syndrome in ANA and anti-Jo-1 negative patients presenting with idiopathic interstitial pneumonia
复制标题
DOI：
10.1016/j.rmed.2009.05.001
复制
发表时间：
2009-11-01
影响因子：
4.3
通讯作者：
Brown, Kevin K.
中科院分区：
文献类型：
--
作者：
Fischer, Aryeh;Swigris, Jeffrey J.;Brown, Kevin K.
目的：描述的临床特征的“特发性”间质性肺炎的患者被诊断为抗合成酶综合征的基础上的临床特征和阳性抗PL-7或PL-12 antibodies.Methods：在24个月的时间内，我们评估了37例谁提出了临床。抗合成酶（AS）综合征的特征，阴性抗Jo-1抗体，以及评估其他抗tRNA合成酶（抗tRS）抗体。结果：9例（24%）患者根据临床表现和其他抗tRS抗体（7例抗PL-7抗体，2例抗PL-12抗体）确诊为非抗Jo-1阳性AS综合征。所有患者均以呼吸困难为首发症状，以ILD为首发表现。在三名患者中发现了CPK升高，但只有两名患者出现肌无力。肺生理学显示限制（强制生命体征。能力预测的60%）和受损的气体转移（一氧化碳扩散能力预测的40%）。所有患者的胸部HRCT扫描结果相似，基底动脉异常占优势，模式提示非特异性间质性肺炎和机化性肺炎。免疫调节疗法用于治疗ILD反应是可变的，但有些科目明显improved.Conclusion：抗PL-7和PL-12抗体可能是更常见的“特发性”间质性肺炎比以前考虑的患者，应检查AS综合征的功能，尽管抗核或抗Jo-1抗体的阴性屏幕的患者。需要进一步的研究来提高对抗PL-7或抗PL-12阳性AS综合征的认识，包括其预后和最佳治疗方法。(C)2009爱思唯尔有限公司保留所有权利。
Objectives: To describe the clinical features of patients presenting with "idiopathic" interstitial pneumonia that were diagnosed with anti-synthetase syndrome based on clinical features and positive anti-PL-7 or PL-12 antibodies.Methods: Over a 24-month period, we evaluated 37 patients who presented with clinical. features of anti-synthetase (AS) syndrome, negative anti-Jo-1 antibodies, and who were assessed for other anti-tRNA synthetase (anti-tRS) antibodies. All data were abstracted from the medical record.Results: Nine (24%) were confirmed to have non-anti-Jo-1 positive AS syndrome based on clinical features and the presence of other anti-tRS antibodies (seven with anti-PL-7, two with anti-PL-12 antibodies). All presented with dyspnea as the initial symptom and with ILD as the first manifestation. Elevated CPK was identified in three patients but only two had muscle weakness. Pulmonary physiology revealed restriction (forced vital. capacity 60% of predicted) and impaired gas transfer (diffusing capacity for carbon monoxide 40% of predicted). All, had similar findings on thoracic HRCT scans, with basilar predominance of abnormalities and patterns suggestive of nonspecific interstitial pneumonia and organizing pneumonia. Immunomodulatory therapies were used to treat the ILD-responses were variable, but some subjects clearly improved.Conclusion: Anti-PL-7 and PL-12 antibodies may be more common among patients presenting with "idiopathic" interstitial pneumonia than formerly considered and should be checked in patients with features of AS syndrome despite a negative screen for anti-nuclear or anti-Jo-1 antibodies. Further research is needed to advance understanding of anti-PL-7 or anti-PL-12-positive AS syndrome, including its prognosis and optimal approaches to therapy. (C) 2009 Elsevier Ltd. All rights reserved.