►可见于系统性硬化症患者(48)。

►若临床疑似系统性硬化症，推荐后续检测抗U3RNP/核纤蛋白（fibrillarin）抗体。该抗原包含在疾病特异性免疫检测试剂中（例如，系统性硬化症谱*）(48)。

►若经免疫检测法确认有抗U3RNP/核纤蛋白（fibrillarin）抗体反应性，则其与弥散性系统性硬化症临床相关，且提示更易罹患肺动脉高压、骨骼肌疾病、严重心脏受累、胃肠动力障碍等(23,48-50)。

在系统性硬化症患者中，抗U3RNP/核纤蛋白（fibrillarin）抗体在非裔美国人、拉丁裔美国人中最为常见(48,49,51)。

注：尽管已有商品化的抗U3RNP/核纤蛋白（fibrillarin）抗体免疫检测试剂，但其低敏感性等技术问题应纳入考量(24)。

*炎症性肌病谱、系统性硬化症谱、自身免疫性肝病谱（扩展版）等检测试剂可能仅供临床专业实验室使用

► Found in patients with SSc (48)

► If SSc is clinically suspected, it is recommended to perform a follow-up test for anti-U3RNP/fibrillarin antibodies; the antigen is included in disease specific immunoassays (i.e., SSc profile*) (48)

► If confirmed as anti-U3RNP/fibrillarin reactivity by immunoassay, the clinical association is with diffuse SSc, increased incidence of pulmonary arterial hypertension, skeletal muscle disease, severe cardiac involvement, and gastrointestinal dysmotility (23, 48–50)

► Among SSc patients, anti-U3RNP/fibrillarin antibodies are most commonly found in African American and Latin American patients (48, 49, 51)

Notes: Although some anti-U3RNP/fibrillarin immunoassays are commercially available, technical issues relating to the limited sensitivity of these immunoassays should be taken into consideration (24).

*Availability of the inflammatory myopathy profile, the SSc profile and the (extended) liver profile may be limited to specialty clinical laboratories.

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