►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.

23. Johnson SR, Fransen J, Khanna D, et al. Validation of potential classification criteria for systemic sclerosis. Arthritis Care Res 2012;64:358–67.

24. Mehra S, Walker J, Patterson K, et al. Autoantibodies in systemic sclerosis. Autoimmun Rev 2013;12:340–54.

48. Okano Y, Steen VD, Medsger TA. Autoantibody to U3 nucleolar ribonucleoprotein (fibrillarin) in patients with systemic sclerosis. Arthritis Rheum 1992;35:95–100.

49. Arnett FC, Reveille JD, Goldstein R, et al. Autoantibodies to fibrillarin in systemic sclerosis (scleroderma). An immunogenetic, serologic, and clinical analysis. Arthritis Rheum 1996;39:1151–60.

50. Tormey VJ, Bunn CC, Denton CP, et al. Anti-fibrillarin antibodies in systemic sclerosis. Rheumatology 2001;40:1157–62.

51. Nandiwada SL, Peterson LK, Mayes MD, et al. Ethnic differences in autoantibody diversity and hierarchy: More clues from a US cohort of patients with systemic sclerosis. J Rheumatol 2016;43:1816–24.

23. Johnson SR, Fransen J, Khanna D, et al. Validation of potential classification criteria for systemic sclerosis. Arthritis Care Res 2012;64:358–67.

24. Mehra S, Walker J, Patterson K, et al. Autoantibodies in systemic sclerosis. Autoimmun Rev 2013;12:340–54.

48. Okano Y, Steen VD, Medsger TA. Autoantibody to U3 nucleolar ribonucleoprotein (fibrillarin) in patients with systemic sclerosis. Arthritis Rheum 1992;35:95–100.

49. Arnett FC, Reveille JD, Goldstein R, et al. Autoantibodies to fibrillarin in systemic sclerosis (scleroderma). An immunogenetic, serologic, and clinical analysis. Arthritis Rheum 1996;39:1151–60.

50. Tormey VJ, Bunn CC, Denton CP, et al. Anti-fibrillarin antibodies in systemic sclerosis. Rheumatology 2001;40:1157–62.

51. Nandiwada SL, Peterson LK, Mayes MD, et al. Ethnic differences in autoantibody diversity and hierarchy: More clues from a US cohort of patients with systemic sclerosis. J Rheumatol 2016;43:1816–24.

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