摘要目的:分析2例少见型抗合成酶综合征（ASS）的临床特点、自身抗体及预后，提高临床医师对少见型ASS的认识。方法:收集2020年7月至2022年8月在郑州大学第一附属医院风湿免疫科就诊的2例少见型ASS患者的临床资料，并进行相关文献复习。结果:2例少见型ASS患者分别是抗苯丙酰酮tRNA（抗Zo抗体）和抗酪氨酰抗体（抗Ha抗体）阳性，均以间质性肺疾病（ILD）为主要临床表现，同时合并抗Ro52抗体阳性。ANA荧光核型表现为胞质型，但与常见的几种抗氨酰tRNA合成酶（ARS）抗体的胞质致密颗粒型有所不同。经糖皮质激素和免疫抑制剂治疗后，例1因病程长、确诊较晚，死于呼吸衰竭，例2病程短，经积极治疗后肺部病变显著好转。结论:临床中遇到胞质型ANA荧光核型，尤其合并抗Ro52抗体阳性的ILD患者，需积极完善全套肌炎特异性抗体筛查，以排除少见型ASS。

abstractsObjective:To study the clinical and immunological features of two case of rare antisynthetase syndrome (ASS), so as to improve the level of diagnosis and treatment.Methods:Two cases with rare antisynthetase syndrome admitted to the First Affiliated Hospital of Zhengzhou University from July 2020 to August 2022 were collected.Results:The two rare ASS were anti-Zo antibody and anti-Ha antibody positive patients, both of which had interstitial lung disease (ILD) as the main clinical manifestation and positive anti-Ro52 antibody. Two rare antisynthetase autoantibodies manifested cytoplasmic ANA indirect immunofluorescence (IIF) staining pattern, but it is different from the cytoplasmic dense speckled pattern of several common ASS antibodies. After treatment with glucocorticoids and immunosuppressants, case 1 died of respiratory failure due to a long course of disease and late diagnosis, the lung lesions of case 2 improved significantly.Conclusion:When encountering the cytoplasmic ANA fluorescent pattern in ILD patients, especially with anti-Ro52 antibody, it is necessary to screen more myositis specific antibodies to rule out the possibility of rare ASS.