Purpose

Antisynthetase syndrome (ASS) is a rare systemic autoimmune condition associated to the presence of anti-aminoacyl-tRNA synthetase antibodies. Interstitial lung disease (ILD) is the most prevalent manifestation of ASS and is a major determinant of morbidity and mortality. The aim of this study was to describe the radiological characteristics of patients with ASS-associated-ILD in our institution.

Materials and methods

Medical records from 2014 to 2020 were retrospectively reviewed and patients with a diagnosis of ASS and evidence of ILD on HRCT were included. HRCT images were reviewed by two thoracic radiologists in consensus. Five HRCT patterns were defined: cellular non-specific interstitial pneumonia (NSIP), organizing pneumonia (OP), mixed NSIP/OP pattern, acute interstitial pneumonia (AIP) pattern and fibrotic pattern. Descriptive statistics was calculated for all variables.

Results

Twenty-two patients with ASS who met inclusion criteria were included. The disease presented with the typical triad of ASS in 45% of patients, 55% had ILD only at the onset. Cellular NSIP was present in 27% of patients, OP in 23%, mixed NSIP/OP in 9%, AIP in 18% and a fibrotic pattern in 23%.

Conclusion

HRCT findings in ASS-associated ILD are often non-specific; nevertheless, it is important to consider this diagnosis, especially in patients presenting with acute onset of symptoms.

中文翻译：

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抗合成酶综合征患者的间质性肺病：一项回顾性病例系列研究。

目的

抗合成酶综合征 (ASS) 是一种罕见的全身性自身免疫性疾病，与抗氨酰-tRNA 合成酶抗体的存在有关。间质性肺病 (ILD) 是 ASS 最普遍的表现，是发病率和死亡率的主要决定因素。本研究的目的是描述我们机构中 ASS 相关 ILD 患者的放射学特征。

材料和方法

回顾性分析了 2014 年至 2020 年的医疗记录，并纳入了诊断为 ASS 且 HRCT 显示 ILD 证据的患者。HRCT 图像由两位胸部放射科医师一致审查。定义了五种 HRCT 模式：细胞非特异性间质性肺炎 (NSIP)、机化性肺炎 (OP)、混合 NSIP/OP 模式、急性间质性肺炎 (AIP) 模式和纤维化模式。计算所有变量的描述性统计数据。

结果

符合入选标准的 22 名 ASS 患者被纳入。该病在 45% 的患者中表现为典型的 ASS 三联征，55% 的患者仅在发病时出现 ILD。27% 的患者存在细胞 NSIP，23% 存在 OP，9% 存在混合 NSIP/OP，18% 存在 AIP，23% 存在纤维化模式。

结论

ASS 相关 ILD 的 HRCT 结果通常是非特异性的；尽管如此，重要的是要考虑这一诊断，尤其是对于症状急性发作的患者。