简体中文

AC-19-胞浆致密颗粒型

同义词 胞浆均质型 cytoplasmic homogeneous
描述
整个细胞质呈现一种接近均匀且浑沌不淸的模式。
It appears to be a blurry, almost homogeneous pattern throughout the cytoplasm
抗原相关性
tRNA合成酶（PL-7，PL-12），核糖体P蛋白（RibP）、信号识别颗粒（SRP）
tRNA-synthetases (PL-7, PL-12), ribosomal P proteins (RibP), signal recognition particle (SRP)

  • 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
  • ▶在系统性红斑狼疮、抗合成酶抗体综合征 (自身免疫性肌病的一种)、间质性肺病、多发性关节炎、雷诺现象和技工手的患者中有检出；这些特征可能以多种形式或单一形式出现，尤其是间质性肺病[1-4]
    ▶如果临床怀疑为系统性红斑狼疮，推荐后续检测抗核糖体P蛋白抗体 (包括P0、P1、P2和 C22多肽)。
    ▶在一些研究中，抗RibP抗体与神经精神性狼疮有关，在儿童期发病的SLE中，其与自身免疫性溶血性贫血有关[2, 5-8]
    ▶如果疑似IIM，尤其是抗合成酶抗体综合征，推荐进行针对抗tRNA合成酶抗体的后续检测；该抗原包含于疾病特异性免疫试剂(例如炎症性肌炎谱*)中  [1, 4, 9]
    ▶如果疑似IIM，尤其是坏死性肌病，推荐进行针对抗SRP抗体的后续检测；该抗原包含于疾病特异性免疫试剂(例如炎症性肌炎谱*)中  [9]
    注：AC-19和AC-20之间的细微区别可能取决于HEp-2细胞底物和/或抗体浓度；在HEp-2细胞的间接免疫荧光筛查中可能检测不到针对RibP和tRNA合成酶的抗体。
    ▶Found in patients with systemic lupus erythematosus (SLE) and the anti-synthetase syndrome (a subset of idiopathic inflammatory myopathies (IIM)), interstitial lung disease, polyarthritis, Raynaud’s phenomenon, and mechanic’s hands; these features may occur in various combinations or as an isolated manifestation, especially interstitial lung disease [1-4].
    ▶If SLE is clinically suspected, follow-up tests for antibodies to ribosomal P phosphoproteins (P0, P1, P2, C22 RibP peptide) are recommended.
    ▶RibP antibodies have been associated in some studies with neuropsychiatric lupus, and in childhood-onset SLE with autoimmune hemolytic anemia [2, 5-8].
    ▶If IIM, in particular the anti-synthetase syndrome, is suspected, it is recommended to perform follow-up tests for antibodies to tRNA synthetases; antigens are included in disease specific immunoassays (i.e., inflammatory myopathy profile) [1, 4, 9].
    ▶If IIM, in particular necrotizing myopathy, is suspected, it is recommended to perform follow-up tests for SRP antibodies, an antigen included in some disease specific immunoassays (i.e., inflammatory myopathy profile) [9].
    Notes: The fine distinction between AC-19 and -20 may depend on the manufacturer of the HEp-2 substrates and/or antibody concentration; antibodies to both RibP as well as tRNA synthetases may be undetected in HEp-2 IFA-screening.
  • 二级信息
    Second level information


  • 抗RibP抗体:
    ▶据报道，10%的自身免疫性肝炎患者体内存在抗RibP抗体，而这些患者并没有系统性红斑狼疮的临床症状或实验室证据[10]
    ▶一项多中心研究表明，系统性红斑狼疮患者中抗RibP抗体的阳性率为8%-35% [6]
    ▶在稀释比例为1: 80或更高时，只有不到60%的抗RibP抗体阳性血清表现为AC-19核型；通常会与核仁弱阳性表现并存。
    抗tRNA合成酶抗体:
    ▶在稀释比例为1: 80或更高时，只有不到50%的抗tRNA合成酶抗体阳性血清表现为AC-19核型 [11]
    注: 大多数抗RibP抗体临床相关性研究中并没有显示与AC-19核型的相关性。



    Autoantibodies to RibP:
    ▶RibP antibodies have been reported in 10% of autoimmune hepatitis patients without clinical or laboratory evidence of SLE [10].
    ▶The prevalence of RibP antibodies in SLE has been reported to range between 8 – 35% in a multicenter study [6].
    ▶Less than 60% of the sera positive for RibP antibodies have the AC-19 pattern at serum screening dilutions of 1:80 or higher; the coexistence of a weak nucleolar staining is relatively common.
    Autoantibodies to tRNA-synthetases:
    ▶Not all tRNA synthetase antibodies are positive in HEp-2 IFA; less than 50% of sera having tRNA synthetase antibodies have an AC-19 pattern at serum screening dilutions of 1:80 or higher [11].
    Note: Most reports describing clinical association of RibP antibodies do not actually show correlations with the AC-19 pattern as such.
  • 参考文献
  • 1.Satoh M, Tanaka S, Ceribelli A, Calise SJ, Chan EKL. A Comprehensive Overview on Myositis-Specific Antibodies: New and Old Biomarkers in Idiopathic Inflammatory Myopathy. Clin Rev Allergy Immunol. 2017;52:1-19.
    2.Sciascia S, Bertolaccini ML, Roccatello D, Khamashta MA, Sanna G. Autoantibodies involved in neuropsychiatric manifestations associated with systemic lupus erythematosus: a systematic review. J Neurol. 2014;261:1706-14.
    3.Yura H, Sakamoto N, Satoh M, Ishimoto H, Hanaka T, Ito C, Hasegawa T, Tanaka S, et al. Clinical characteristics of patients with anti-aminoacyl-tRNA synthetase antibody positive idiopathic interstitial pneumonia. Respir Med. 2017;132:189-94.
    4.Choi MY, Satoh M, Fritzler MJ. Update on autoantibodies and related biomarkers in autoimmune inflammatory myopathies. Curr Opin Rheumatol. 2023;35:383-94.
    5.Valoes CC, Molinari BC, Pitta AC, Gormezano NW, Farhat SC, Kozu K, Sallum AM, Appenzeller S, et al. Anti-ribosomal P antibody: a multicenter study in childhood-onset systemic lupus erythematosus patients. Lupus. 2017;26:484-9.
    6.Mahler M, Kessenbrock K, Szmyrka M, Takasaki Y, Garcia-De La Torre I, Shoenfeld Y, Hiepe F, Shun-le C, et al. International multicenter evaluation of autoantibodies to ribosomal P proteins. Clin Vaccine Immunol. 2006;13:77-83.
    7.Choi MY, FitzPatrick RD, Buhler K, Mahler M, Fritzler MJ. A review and meta-analysis of anti-ribosomal P autoantibodies in systemic lupus erythematosus. Autoimmun Rev. 2020;19:102463.
    8.Bonfa E, Golombek SJ, Kaufman LD, Skelly S, Weissbach H, Brot N, Elkon KB. Association between lupus psychosis and anti-ribosomal P protein antibodies. N Engl J Med. 1987;317:265-71.
    9.Betteridge Z, McHugh N. Myositis-specific autoantibodies: an important tool to support diagnosis of myositis. J Intern Med. 2016;280:8-23.
    10.Calich AL, Viana VS, Cancado E, Tustumi F, Terrabuio DR, Leon EP, Silva CA, Borba EF, et al. Anti-ribosomal P protein: a novel antibody in autoimmune hepatitis. Liver Int. 2013;33:909-13.
    11.Fritzler MJ, Choi MY, Mahler M. The Antinuclear Antibody Test in the Diagnosis of Antisynthetase Syndrome and Other Autoimmune Myopathies. J Rheumatol. 2018;45:444-5.

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