Characteristics of interstitial lung disease in SS-A positive/Jo-1 positive inflammatory myopathy patients
Characteristics of interstitial lung disease in SS-A positive/Jo-1 positive inflammatory myopathy patients
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DOI：
10.1007/s00296-009-0884-9
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发表时间：
2009-06-01
影响因子：
4
通讯作者：
Danko, K.
中科院分区：
文献类型：
--
作者：
Vancsa, Andrea;Csipo, I.;Danko, K.
肌炎（IIM）患者发生间质性肺病（ILD）的最强预测因素是存在不同的抗合成酶抗体。本研究的目的是比较抗SS-A阳性和阴性抗合成酶综合征（ASS）患者的临床特征、影像学表现和治疗反应。对315名IIM患者进行了前瞻性研究，其中包括27名抗Jo-1阳性ASS患者。平均病程为46.6（范围4-198）个月。所有患者均符合IIM的分类标准。所有患者均于确诊时及免疫抑制剂治疗后6个月行胸片、肺功能及HRCT检查。所有患者均行常规实验室检查，RF、ANA、抗ENA、抗SS-A、抗组氨酰转移RNA抗体（Jo-1）。发现70.4%的ASS患者存在ILD。抗SS-A阴性的ASS组更易发生肺泡炎，对免疫抑制剂治疗反应良好（p < 0.05）。HRCT扫描显示SS-A阳性组纤维化程度较重。15.8%的患者死于肺部或心脏并发症。总之，抗SS-A和抗Jo-1抗体的共存可能是需要更积极治疗的IIM患者中更粗糙和严重ILD的良好预测因子。
The strongest predictive factor for the development of interstitial lung disease (ILD) in myositis (IIM) patients is the presence of different antisynthetase antibodies. The aim of this study was to compare the clinical characteristics, radiological findings and therapeutic response between the anti-SS-A positive and negative antisynthetase syndrome (ASS) patients. A prospective study of 315 IIM patients was conducted including 27 anti-Jo-1 positive ASS patients. Mean disease duration was 46.6 (range 4-198) months. All patients fulfilled the classification criteria for IIM. All patients underwent chest radiography, pulmonary function tests and HRCT at he time of diagnosis and 6 months after the immunosuppressive therapy. Routine laboratory tests, RF, ANA, anti-ENA, anti-SS-A, anti-histidyl-transfer RNA antibody (Jo-1) measurements were performed in all patients. ILD was found to be present in 70.4% of ASS patients. The anti-SS-A negative ASS group had a more frequent association with alveolitis and responded well to immunosuppressive therapy (p < 0.05). HRCT scan showed more fibrosis in the SS-A positive group. 15.8% of patients died due to pulmonary or cardiac complications. In conclusion, coexistence of anti-SS-A and anti-Jo-1 antibody may be a good predictor for a more coarse and severe ILD in IIM patients who require a more aggressive approach in therapy.