►不同程度地存在于多种系统性自身免疫性风湿疾病中,特别是干燥综合征、系统性红斑狼疮、亚急性皮肤红斑狼疮、新生儿红斑狼疮、先天性心脏传导阻滞、皮肌炎、系统性硬化症和系统性硬化症-自身免疫性肌病重叠综合征(15)
►对于临床疑似干燥综合征、系统性红斑狼疮、亚急性皮肤性红斑狼疮、新生儿红斑狼疮或先天性心脏传导阻滞的患者,建议对抗SS-A/Ro(Ro60)和抗SS-B/La抗体进行后续筛查;在大多数实验室中,这些抗原都包含在常规的ENA谱检测中(15)
►抗SSA/Ro自身抗体是干燥综合征分类标准的一部分(标准不区分 Ro60和Ro52/TRIM21)(25)
►对于临床疑似系统性硬化症、自身免疫性肌病或较轻的系统性红斑狼疮的患者,建议进一步检测抗Mi-2、TIF1γ和Ku自身抗体;这些抗原通常包含在疾病特异性免疫检测(例如,炎症性肌病谱*)中(26)
►抗Mi-2和TIF1γ自身抗体与皮肌炎相关;皮肌炎患者中抗TIF1γ自身抗体虽然在所有的AC-4核型中很少见,但与老年患者的恶性肿瘤密切相关(26,27)
►抗Ku抗体与系统性硬化症-自身免疫性肌病重叠综合征和系统性红斑狼疮-系统性硬化症-自身免疫性肌病重叠综合征相关(26)
注:HEp-2间接免疫荧光检测中可能检测不到抗SS-A/Ro(Ro60)和自身免疫性肌病特异性自身抗体(28)
*炎症性肌病谱、系统性硬化症谱和(扩展的)肝病谱的应用可能仅限于专业的临床实验室。
► If SjS, SLE, subacute cutaneous lupus erythematosus, neonatal lupus erythematosus, or congenital heart block is clinically suspected, it is recommended to perform follow-up tests for anti-SS-A/Ro (Ro60) and anti-SS-B/La antibodies; in most laboratories these antigens are included in the routine ENA profile (15)
► Autoantibodies to SS-A/Ro are part of the classification criteria for SjS (the criteria do not distinguish between Ro60 and Ro52/TRIM21) (25)
► If SSc, AIM, or to a lesser extend SLE, is clinically suspected, it is recommended to perform follow-up tests for detecting autoantibodies to Mi-2, TIF1γ, and Ku; these antigens are typically included in disease specific immunoassays (i.e., inflammatory myopathy profile*) (26)
► Autoantibodies to Mi-2 and TIF1γ are associated with DM; autoantibodies to TIF1γ in patients with DM, although rare in the overall AC-4 pattern, is strongly associated with malignancy in old patients (26, 27)
► Autoantibodies to Ku are associated with SSc-AIM and SLE-SSc-AIM overlap syndromes (26)
Notes: Anti-SS-A/Ro (Ro60) and AIM-specific autoantibodies may be undetected in HEp-2 IIFA-screening (28)
*Availability of the inflammatory myopathy profile, the SSc profile and the (extended) liver profile may be limited to specialty clinical laboratories.