►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 (33, 86, 87)

►如果临床上疑似为系统性红斑狼疮，推荐后续检测抗核糖体P蛋白抗体 (包括P0、P1、P2和 C22多肽)；这些抗原可能包含于常规的 ENA谱中

►在一些研究中，抗RibP抗体与神经精神性狼疮和儿童期发病的SLE合并自身免疫性溶血性贫血有关(86,88,89)

►如果疑似自身免疫性肌病，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 (即，肌炎谱*)中 (26, 33)

►如果疑似自身免疫性肌病，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(即，肌炎谱*)中 (26)

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。

*肌炎谱、系统性硬化症谱和自身免疫性肝病谱(扩展版)可能仅限于专业临床实验室应用。

► Found in patients with SLE and the anti-synthetase syndrome (a subset of AIM), 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 (33, 86, 87)

► If SLE is clinically suspected, follow-up tests for antibodies to ribosomal P phosphoproteins (P0, P1, P2, C22 RibP peptide) are recommended; these antigens may be included in the routine ENA profile

► Anti-RibP antibodies have been associated in some studies with neuropsychiatric lupus, and in childhood-onset SLE with autoimmune hemolytic anemia (86, 88, 89)

► If AIM, 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*) (26, 33)

► If AIM, in particular necrotizing myopathy, is suspected, it is recommended to perform follow-up tests for anti-SRP antibodies; the antigen is included in disease specific immunoassays (i.e., inflammatory myopathy profile*) (26)

Notes: The fine distinction between AC-19 and -20 may depend on HEp-2 substrates and/or antibody concentration; antibodies to both RibP as well as tRNA synthetases may be undetected in HEp-2 IIFA-screening.

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

26. Betteridge Z, McHugh N. Myositis-specific autoantibodies: an important tool to support diagnosis of myositis. J Intern Med 2016;280:8–23.

33. Satoh M, Tanaka S, Ceribelli A, et al. A comprehensive overview on myositis-specific antibodies: new and old biomarkers in idiopathic inflammatory myopathy. Clin Rev Allergy Immunol 2017;52:1–19.

86. Sciascia S, Bertolaccini ML, Roccatello D, et al. Autoantibodies involved in neuropsychiatric manifestations associated with systemic lupus erythematosus: a systematic review. J Neurol 2014;261:1706–14.

87. Yura H, Sakamoto N, Satoh M, et al. Clinical characteristics of patients with antiaminoacyl-tRNA synthetase antibody positive idiopathic interstitial pneumonia. Respir Med 2017;132:189–94.

88. Valões CC, Molinari BC, Pitta AC, et al. Anti-ribosomal P antibody: a multicenter study in childhood-onset systemic lupus erythematosus patients. Lupus 2017;26:484–9.

89. Mahler M, Kessenbrock K, Szmyrka M, et al. International multicenter evaluation of autoantibodies to ribosomal P proteins. Clin Vaccine Immunol 2006;13:77–83.

26. Betteridge Z, McHugh N. Myositis-specific autoantibodies: an important tool to support diagnosis of myositis. J Intern Med 2016;280:8–23.

33. Satoh M, Tanaka S, Ceribelli A, et al. A comprehensive overview on myositis-specific antibodies: new and old biomarkers in idiopathic inflammatory myopathy. Clin Rev Allergy Immunol 2017;52:1–19.

86. Sciascia S, Bertolaccini ML, Roccatello D, et al. Autoantibodies involved in neuropsychiatric manifestations associated with systemic lupus erythematosus: a systematic review. J Neurol 2014;261:1706–14.

87. Yura H, Sakamoto N, Satoh M, et al. Clinical characteristics of patients with antiaminoacyl-tRNA synthetase antibody positive idiopathic interstitial pneumonia. Respir Med 2017;132:189–94.

88. Valões CC, Molinari BC, Pitta AC, et al. Anti-ribosomal P antibody: a multicenter study in childhood-onset systemic lupus erythematosus patients. Lupus 2017;26:484–9.

89. Mahler M, Kessenbrock K, Szmyrka M, et al. International multicenter evaluation of autoantibodies to ribosomal P proteins. Clin Vaccine Immunol 2006;13:77–83.

抗RibP的自身抗体:

►据报道，10%的无系统性红斑狼疮症状和实验室证据的自身免疫性肝炎患者体内存在抗RibP抗体 (33)

►一项多中心研究表明，抗RibP抗体在系统性红斑狼疮的患病率为8%-35% (34)

►不到60%的抗RibP抗体阳性血清在血清筛查稀释度为1: 80或更高时表现为AC-19核型；核仁弱阳性并存的现象相对常见

抗tRNA合成酶的自身抗体:

►不到50%的抗tRNA合成酶抗体阳性血清中在血清筛查稀释度为1: 80或更高时表现为AC-19核型 (35)

注: 大多数抗Ribp抗体临床相关性研究中并没有显示与AC-19模式的相关性。

Autoantibodies to RibP:

► Anti-RibP antibodies have been reported in 10% of AIH patients without clinical or laboratory evidence of SLE (33)

► The prevalence of anti-RibP antibodies in SLE has been reported to range between 8 – 35% in a multicenter study (34)

► Less than 60% of the sera positive for anti-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:

► Less than 50% of sera having anti-tRNA synthetase antibodies have an AC-19 pattern at serum screening dilutions of 1/80 or higher (35)

Note: Most reports describing clinical association of anti-RibP antibodies do not actually show correlations with the AC-19 pattern as such.

33. Calich AL, Viana VS, Cancado E, et al. Anti-ribosomal P protein: a novel antibody in autoimmune hepatitis. Liver Int 2013;33:909-13.

34. Mahler M, Kessenbrock K, Szmyrka M, et al. International multicenter evaluation of autoantibodies to ribosomal P proteins. Clin Vaccine Immunol 2006;13:77-83.

35. Fritzler MJ, Choi MY, Mahler M. The anti-nuclear antibody (ANA) test in the diagnosis of anti-synthetase syndrome and other autoimmune myopathies (AIM). J Rheumatol 2018;45:444-5.

33. Calich AL, Viana VS, Cancado E, et al. Anti-ribosomal P protein: a novel antibody in autoimmune hepatitis. Liver Int 2013;33:909-13.

34. Mahler M, Kessenbrock K, Szmyrka M, et al. International multicenter evaluation of autoantibodies to ribosomal P proteins. Clin Vaccine Immunol 2006;13:77-83.

35. Fritzler MJ, Choi MY, Mahler M. The anti-nuclear antibody (ANA) test in the diagnosis of anti-synthetase syndrome and other autoimmune myopathies (AIM). J Rheumatol 2018;45:444-5.