临床相关性
一级信息 关于临床相关性和缩写列表 |
Clinical Relevance
First level information About Clinical Relevance & List of Abbreviations |
►可见于系统性硬化症、系统性硬化症-自身免疫性肌病重叠综合征,及有其他系统性自身免疫性风湿疾病临床表现的患者(44-46)。 ►若临床疑似局限皮肤型系统性硬化症,推荐进一步检测抗Th/To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*)(44,45)。 ►若临床疑似系统性硬化症-自身免疫性肌病重叠综合征,推荐进一步检测抗PM/Scl抗体。该抗原可能包含在常规ENA谱中,也包含在疾病特异性免疫检测试剂中(例如,炎症性肌病谱*与系统性硬化症谱*)。大体上,抗PM/Scl抗体会在AC-8核型基础上出现弥散的细胞核致密颗粒荧光(46)。 ►其他已被识别的抗原包括B23/核仁磷酸蛋白(nucleophosmin)、No55/SC65、C23/核仁素(nucleolin)等,但相应自身抗体的临床意义尚不明确。目前尚无针对这些自身抗体的商品化免疫检测试剂。 注:尽管已有商品化的抗Th/To抗体免疫检测试剂,但应充分考虑其低敏感性等技术问题(44,47)。 *炎症性肌病谱、系统性硬化症谱及肝病谱(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, SSc-AIM overlap syndrome, and patients with clinical manifestations of other SARD (44–46) ► If limited cutaneous SSc is clinically suspected, it is recommended to perform a follow-up test for anti-Th/To antibodies; the antigen is included in disease specific immunoassays (i.e., SSc profile*) (44, 45) ► If SSc-AIM overlap syndrome is clinically suspected, it is recommended to perform a follow-up test for anti-PM/Scl antibody reactivity; the antigen may be included in the routine ENA profile and is included in disease specific immunoassays (i.e., inflammatory myopathy profile* and the SSc profile*); in general, anti-PM/Scl antibodies yield a diffuse nuclear fine speckled staining in addition to the AC-8 pattern (46) ► Other antigens recognized include B23/nucleophosmin, No55/SC65, and C23/nucleolin, but the clinical significance of these autoantibodies is not well established; specific immunoassays for these autoantibodies are currently not commercially available Notes: Although some anti-Th/To antibody immunoassays are commercially available, technical issues relating to the limited sensitivity of these immunoassays should be taken in to consideration (44, 47). *Availability of the inflammatory myopathy profile, the SSc profile and the (extended) liver profile may be limited to specialty clinical laboratories. |
一级信息参考文献 | First level information references |
44. Mahler M, Fritzler MJ, Satoh M. Autoantibodies to the mitochondrial RNA processing (MRP) complex also known as Th/To autoantigen. Autoimmun Rev 2015;14:254–7. 45. Ceribelli A, Cavazzana I, Franceschini F, et al. Anti-Th/To are common antinucleolar autoantibodies in Italian patients with scleroderma. J Rheumatol 2010;37:2071–5. 46. Mahler M, Raijmakers R. Novel aspects of autoantibodies to the PM/Scl complex: clinical, genetic and diagnostic insights. Autoimmun Rev 2007;6:432–7. 47. Mahler M, Gascon C, Patel S, et al. Rpp25 is a major target of autoantibodies to the Th/To complex as measured by a novel chemiluminescent assay. Arthritis Res Ther 2013;15. |
44. Mahler M, Fritzler MJ, Satoh M. Autoantibodies to the mitochondrial RNA processing (MRP) complex also known as Th/To autoantigen. Autoimmun Rev 2015;14:254–7. 45. Ceribelli A, Cavazzana I, Franceschini F, et al. Anti-Th/To are common antinucleolar autoantibodies in Italian patients with scleroderma. J Rheumatol 2010;37:2071–5. 46. Mahler M, Raijmakers R. Novel aspects of autoantibodies to the PM/Scl complex: clinical, genetic and diagnostic insights. Autoimmun Rev 2007;6:432–7. 47. Mahler M, Gascon C, Patel S, et al. Rpp25 is a major target of autoantibodies to the Th/To complex as measured by a novel chemiluminescent assay. Arthritis Res Ther 2013;15. |
二级信息 | Second level information |
►因抗Th/To反应性导致的AC-8核型也可见于系统性红斑狼疮、未分化的结缔组织病(例如,患者有风湿性临床特征,但无法诊断为系统性自身免疫性风湿疾病)、无皮肤硬化的系统性硬化症、特发性间质性肺病或肺动脉高压患者(20,21)。 ►对于因抗PM/Scl反应性导致的AC-8核型,其患者可能在自身免疫性肌病和系统性硬化症临床特征以外,出现多种系统性红斑狼疮和干燥综合征临床表现(22)。 |
► The AC‐8 pattern that is the result of the anti‐Th/To reactivity is also seen in patients with SLE, UCTD (i.e., patients with rheumatic symptoms without a SARD diagnosis), SSc sine scleroderma, idiopathic interstitial lung disease or pulmonary hypertension (20, 21) ► Patients with autoantibodies revealing the AC‐8 pattern due to anti‐PM/Scl reactivity may have, in addition to the clinical features of AIM and SSc, various clinical manifestations of SLE and SjS (22) |
二级信息参考文献 | Second level information references |
20. Fischer A, Pfalzgraf FJ, Feghali‐Bostwick CA, et al. Anti‐Th/To‐Positivity in a Cohort of Patients with Idiopathic Pulmonary Fibrosis. J Rheumatol 2006;33:1600‐1605. 21. Fischer A, Meehan RT, Feghali‐Bostwick CA, et al. Unique characteristics of systemic sclerosis sine scleroderma‐associated interstitial lung disease. Chest 2006;130:976‐981. 22. Mahler M, Raijmakers R. Novel aspects of autoantibodies to the PM/Scl complex: clinical, genetic and diagnostic insights. Autoimmun Rev 2007;6:432‐437. |
20. Fischer A, Pfalzgraf FJ, Feghali‐Bostwick CA, et al. Anti‐Th/To‐Positivity in a Cohort of Patients with Idiopathic Pulmonary Fibrosis. J Rheumatol 2006;33:1600‐1605. 21. Fischer A, Meehan RT, Feghali‐Bostwick CA, et al. Unique characteristics of systemic sclerosis sine scleroderma‐associated interstitial lung disease. Chest 2006;130:976‐981. 22. Mahler M, Raijmakers R. Novel aspects of autoantibodies to the PM/Scl complex: clinical, genetic and diagnostic insights. Autoimmun Rev 2007;6:432‐437. |
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