Pathological characteristics of skeletal muscle in anti-synthase syndrome patients
10.3760/cma.j.c141217-20190711-00254
- VernacularTitle:抗合成酶综合征骨骼肌肌肉病理特征分析
- Author:
Xiaolan TIAN
1
;
Hongyan LIU
;
Lining ZHANG
;
Yamei ZHANG
;
Wenli LI
;
Qingyan LIU
;
Qinglin PENG
;
Guochun WANG
;
Xin LU
Author Information
1. 中日友好医院风湿免疫科，北京 100029
- From:
Chinese Journal of Rheumatology
2020;24(9):597-603,F3
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To investigate the pathological characteristics of skeletal muscle and its association with anti-aminoacyl-tRNA synthetases(ARS) antibodies and clinical features in patients with anti-synthetase syndrome (ASS).Methods:Patients diagnosed as ASS at China-Japan Friendship Hospital from 2008 to 2018 were involved in this study. Immunohistochemistry staining of MHC-Ⅰ, CD3, CD4, CD8 and CD20 molecule were performed in all muscle specimen taken from these patients. According to the muscular pathological characteristics, all patients could be divided into six pathological subgroups: pathologic DM(pDM), pathologic PM (pPM), unspecified myositis(USM), necrotizing myopathies (NAM), only MHC-Ⅰexpression (MHC-Ⅰ) and normal pathology groups. Immunoblotting assay was used to detect anti-ARS antibodies. Statistical analysis was performed using Statistical Product and Service Solutions (SPSS) software. T-test, Mann-Whitney U test and Analysis of Variance (ANOVA) were used for the comparison of measurement data. Chi-square test or Fisher's test were used for categorical data. P<0.05 was considered statistically significant. Results:A total of 77 patients underwent muscle biopsy and anti-ARS antibodies test, with the average age of (50±12) years and disease course of 9(3-24) months. The prevalence of anti-Jo-1, anti-PL-7, anti-PL-12, anti-EJ and anti-OJ antibodies in these patients was 47%(36 cases), 29%(22 cases), 12%(9 cases), 13%(10 cases) and 0 respectively. Among all the ASS patients, the most common pathological type was USM(37/77, 48%), followed by pDM(14/77, 18%), the normal pathology(13/77, 17%), NAM(10/77, 13%) and MHC-Ⅰ (3/77, 4%) groups. There were no pPM subtypes in all groups. The frequency of pDM types was significantly different among the anti-ARS antibodies groups( χ2=9.075, P=0.028). The anti-EJ-positive patients had a higher frequency of pDM compared with anti-PL-7-group(40% vs 4%, χ2=6.555, P=0.024). Meanwhile, the CD20 expression in muscle tissue was observed in 30% of anti-EJ-positive patients and 4% of anti-Jo-1-positive ones. There was statistically significant difference in the positive rate of CD20 expression among anti-ARS antibodies groups ( χ2=12.837, P<0.01). Conclusion:The muscle pathological characteristics of ASS are polymorphic and relate to the anti-ARS antibodies. Performing muscle biopsy and distinguishing pathological types are helpful for the diagnosis and stratification of ASS.
