Email Alert | RSS

Journal of Peking University (Health Sciences) ›› 2021, Vol. 53 ›› Issue (6): 1078-1082. doi: 10.19723/j.issn.1671-167X.2021.06.012

Previous Articles     Next Articles

Clinical and immunological characteristics of patients with anti-synthetase syndrome complicated with cardiac involvement

LUO Lan1,2,XING Xiao-yan2,XIAO Yun-shu3,CHEN Ke-yan2,ZHU Feng-yun-zhi2,ZHANG Xue-wu2,LI Yu-hui2,△()   

  1. 1. Department of Ophthalmology, Peking University People’s Hospital, Beijing 100044, China
    2. Department of Rheumatology and Immunology, Peking University People’s Hospital, Beijing 100044, China
    3. Department of Pathology, Peking University People’s Hospital, Beijing 100044, China
  • Received:2021-08-05 Online:2021-12-18 Published:2021-12-13
  • Contact: Yu-hui LI E-mail:liyuhui84@163.com
  • Supported by:
    National Natural Science Foundation of China(81801617);National Natural Science Foundation of China(81771678);National Natural Science Foundation of China(81971520);Research and Development Fund of Peking University People’s Hospital(RDX2020-03)

RICH HTML

 18   

PDF (PC)

201

Abstract:

Objective: To investigate the clinical and immunological features of cardiac involvement in patients with anti-synthetase syndrome (ASS). Methods: In the study, 96 patients diagnosed with ASS hospitalized in the Department of Rheumatology and Immunology, Peking University People’s Hospital from April 2003 to November 2020 were included. The patients were divided into two groups according to whether they were accompanied with cardiac involvement. Demographic features, clinical characteristics (Gottron’s sign/papules, muscle damage, etc.), comorbidities, laboratory indices (creatine kinase, inflammatory indicators, immunoglobulin, complement, lymphocyte subset, autoantibodies, etc.) were collected and the differences between the two groups were analyzed statistically. Results: The prevalence of cardiac involvement in the patients with ASS was 25.0% (24/96). The ASS patients complicated with cardiac involvement presented with elevated cardiac troponin I (cTnI, 75.0%, 18/24), pericardial effusion (33.3%, 8/24), reduction of left ventricular function (33.3%, 8/24) and valves regurgitation (33.3%, 8/24). The age of onset of the patients with cardiac involvement was older than that of the patients without cardiac involvement [(54.58±10.58) years vs. (48.47±13.22) years, P=0.043). Arthritis was observed less frequently in the patients with cardiac involvement than those without cardiac involvement (37.5% vs. 61.1%, P=0.044). In addition, rapidly progressive interstitial lung disease (54.2% vs. 30.6%, P=0.037) was observed more frequently in the patients with cardiac involvement than those without cardiac involvement. As compared with the ASS patients without cardiac involvement, C-reactive protein (CRP) [(13.55 (8.96, 38.35) mg/L vs. 4.60 (1.37, 17.40) mg/L, P=0.001], and lactate dehydrogenase (LDH) [408.0 (255.0, 587.0) U/L vs. 259.5 (189.8, 393.8) U/L, P=0.007] were significantly higher in the patients with cardiac involvement. Anti-Ro-52 antibody was detected more commonly in the ASS patients with cardiac involvement compared with the patients without cardiac involvement (91.7% vs. 69.4%, P=0.029). No significant differences were found in the comorbidities, alanine transaminase (ALT), aspartate transaminase (AST), creatine kinase (CK), erythrocyte sedimentation rate (ESR), ferritin (Fer), immunoglobulin G (IgG), complement 3 (C3), complement 4 (C4), lymphocyte subset between the two groups. Conclusion: Cardiac involvement is common in ASS, mainly manifested as myocardial damage. It is necessary to be aware of cardiac complications in patients with elevated CRP, elevated LDH and positive anti-Ro-52 antibody.

Key words: Anti-synthetase syndrome, Myocarditis, Myositis, Autoantibodies

CLC Number: 

  • R593.2

Table 1

Clinical characteristics of ASS patients with cardiac involvement"

Variables Value, n (%)
Elevated cTnI 18 (75.0)
Elevated BNP 4 (16.7)
UCG
Pericardial effusion 8 (33.3)
PAH 4 (16.7)
Reduction of LV function 8 (33.3)
Valves regurgitation 8 (33.3)
Regional wall motion abnormality 1 (4.2)
ECG
QT interval prolongation 2 (8.3)
Abnormal T waves 2 (8.3)
ST-T segment changes 2 (8.3)

Table 2

Comparison of clinical and laboratory features between ASS patients with or without cardiac involvement"

Variables ASS with cardiac involvement (n=24) ASS without cardiac involvement (n=72) P value
Demographics
Female 17 (70.8) 49 (68.1) 0.799
Age at onset/years 54.58±10.58 48.47±13.22 0.043
Duration/month 12.0 (3.5, 34.5) 5.5 (2.0, 15.2) 0.108
Clinical features
Fever 13 (54.2) 31 (43.1) 0.344
Mechanic’s hands 16 (66.7) 38 (52.8) 0.235
Gottron’s sign/papules 22 (91.7) 52 (72.2) 0.050
Heliotrope rash 8 (33.3) 14 (19.4) 0.161
V-neck sign 5 (20.8) 14 (19.4) >0.999
Shawl sign 3 (12.5) 4 (5.6) 0.497
Myalgia 9 (37.5) 39 (54.2) 0.157
Muscle weakness 14 (58.3) 40 (55.6) 0.812
Raynaud’s phenomenon 2 (8.3) 10 (13.9) 0.722
Arthritis 9 (37.5) 44 (61.1) 0.044
Complications
ILD 24 (100.0) 68 (94.4) 0.569
RP-ILD 13 (54.2) 22 (30.6) 0.037
C-ILD 11 (45.8) 46 (63.9) 0.119
Malignancy 1 (4.2) 5 (6.9) >0.999
PAH 4 (16.7) 7 (9.7) 0.579
Venous thrombosis 3 (12.5) 3 (4.2) 0.163
Risk factors
BMIa≥24 kg/m2 14 (60.9) 35 (51.5) 0.434
Cigarette 4 (16.7) 13 (18.1) >0.999
Hypertension 11 (45.8) 19 (26.4) 0.075
Diabetes 5 (20.8) 14 (19.4) >0.999
Hyperlipidemia 5 (20.8) 14 (19.4) >0.999
Laboratory features
ALT/(U/L) 40.0 (18.2, 78.0) 29.0 (18.2, 48.0) 0.383
AST/(U/L) 46.5 (20.0, 95.5) 29.5 (20.0, 45.8) 0.129
CKb /(U/L) 642 (62, 2 167) 138 (44, 661) 0.057
LDHb/(U/L) 408.0 (255.0, 587.0) 259.5 (189.8, 393.8) 0.007
ESR/(mm/h) 29.3 (17.0, 52.2) 21.0 (8.0, 39.0) 0.074
CRPc/(mg/L) 13.55 (8.96, 38.35) 4.60 (1.37, 17.40) 0.001
Ferritind/(μg/L) 172.6 (56.1, 692.7) 183.6 (66.5, 331.2) 0.837
IgGe/(g/L) 16.9 (12.3, 22.9) 14.9 (11.6, 17.1) 0.108
C3f/(g/L) 0.97±0.26 0.98±0.25 0.819
C4f/(g/L) 0.21 (0.15, 0.24) 0.21 (0.17, 0.27) 0.329
T cellg/(cells/μL) 728 (530, 1 078) 976 (559, 1 548) 0.209
CD4+T cellg/(cells/μL) 435 (212, 619) 521 (376, 834) 0.388
CD8+T cellg/(cells/μL) 269 (200, 305) 392 (210, 545) 0.233
NK cellg/(cells/μL) 145 (86, 389) 154 (76, 391) 0.950
T cellh/% 67.01±15.31 66.51±13.42 0.888
CD4+T cellh/% 38.18±17.08 38.59±10.88 0.918
CD8+T cellh/% 24.4 (18.8, 32.0) 24.1 (18.4, 33.4) 0.987
NK cellg/% 12.7 (10.6, 29.4) 12.6 (7.1, 18.0) 0.470
CD4+/CD8+h 1.45 (1.09, 2.43) 1.56 (0.95, 2.49) 0.853

Table 3

Comparison of autoantibodies between ASS patients with or without cardiac involvement"

Variables ASS with cardiac involvement (n=24), n(%) ASS without cardiac involvement (n=72), n(%) P value
Anti-Jo-1 positivity 11 (45.8) 42 (58.3) 0.286
Anti-PL-7 positivity 3 (12.5) 13 (18.1) 0.752
Anti-PL-12 positivity 4 (16.7) 10 (13.9) >0.999
Anti-EJ positivity 6 (25.0) 7 (9.7) 0.121
Anti-OJ positivity 0 (0) 5 (6.9) 0.327
Anti-KS positivity 1 (4.2) 0 (0) 0.250
ANA positivity 21 (87.5) 55 (76.4) 0.246
Anti-Ro-52 positivity 22 (91.7) 50 (69.4) 0.029
[1] Jayakumar D, Zhang R, Wasserman A, et al. Cardiac manifestations in idiopathic inflammatory myopathies: An overview[J]. Cardiol Rev, 2019, 27(3):131-137.
doi: 10.1097/CRD.0000000000000241 pmid: 30585794
[2] Opinc AH, Makowski MA, Lukasik ZM, et al. Cardiovascular complications in patients with idiopathic inflammatory myopathies: Does heart matter in idiopathic inflammatory myopathies?[J]. Heart Fail Rev, 2021, 26(1):111-125.
doi: 10.1007/s10741-019-09909-8
[3] Sharma K, Orbai AM, Desai D, et al. Brief report: Antisynthetase syndrome-associated myocarditis[J]. J Card Fail, 2014, 20(12):939-945.
doi: 10.1016/j.cardfail.2014.07.012
[4] Connors GR, Christopher-Stine L, Oddis CV, et al. Interstitial lung disease associated with the idiopathic inflammatory myopathies: What progress has been made in the past 35 years?[J]. Chest, 2010, 138(6):1464-1474.
doi: 10.1378/chest.10-0180
[5] Lilleker JB, Vencovsky J, Wang G, et al. The EuroMyositis registry: An international collaborative tool to facilitate myositis research[J]. Ann Rheum Dis, 2018, 77(1):30-39.
doi: 10.1136/annrheumdis-2017-211868 pmid: 28855174
[6] Li Y, Gao X, Li Y, et al. Predictors and mortality of rapidly progressive interstitial lung disease in patients with idiopathic inflammatory myopathy: A series of 474 patients[J]. Front Med (Lausanne), 2020, 7:363.
[7] Dieval C, Deligny C, Meyer A, et al. Myocarditis in patients with antisynthetase syndrome: Prevalence, presentation, and outcomes[J]. Medicine (Baltimore), 2015, 94(26):e798.
doi: 10.1097/MD.0000000000000798
[8] Katz A, Bena J, Chatterjee S. Antisynthetase syndrome: Prevalence of serositis in autoantibody subsets [C/OL]. 2018 ACR/ARHP Annual Meeting [2021-06-17]. https://acrabstracts.org/abstract/antisynthetase-syndrome-prevalence-of-serositis-in-autoan-tibody-subsets/.
[9] Ketlogetswe KS, Aoki J, Traill TA, et al. Severe aortic regurgitation secondary to antisynthetase syndrome[J]. Circulation, 2011, 124(3):e40-e41.
[10] Marie I. Morbidity and mortality in adult polymyositis and dermatomyositis[J]. Curr Rheumatol Rep, 2012, 14(3):275-285.
doi: 10.1007/s11926-012-0249-3
[11] Wang H, Liu T, Cai YY, et al. Pulmonary hypertension in polymyositis[J]. Clin Rheumatol, 2015, 34(12):2105-2112.
doi: 10.1007/s10067-015-3095-7 pmid: 26468158
[12] Labirua-Iturburu A, Selva-O’Callaghan A, Vincze M, et al. Anti-PL-7 (anti-threonyl-tRNA synthetase) antisynthetase syndrome: Clinical manifestations in a series of patients from a European multicenter study (EUMYONET) and review of the literature[J]. Medicine (Baltimore), 2012, 91(4):206-211.
doi: 10.1097/MD.0b013e318260977c
[13] Marie I, Hatron PY, Dominique S, et al. Short-term and long-term outcome of anti-Jo1-positive patients with anti-Ro52 antibody[J]. Semin Arthritis Rheum, 2012, 41(6):890-899.
doi: 10.1016/j.semarthrit.2011.09.008
[14] La Corte R, Lo Mo Naco A, Locaputo A, et al. In patients with antisynthetase syndrome the occurrence of anti-Ro/SSA antibodies causes a more severe interstitial lung disease[J]. Autoimmunity, 2006, 39(3):249-253.
pmid: 16769659
[15] Xing X, Li A, Li C. Anti-Ro52 antibody is an independent risk factor for interstitial lung disease in dermatomyositis[J]. Respir Med, 2020, 172(12):106134.
doi: 10.1016/j.rmed.2020.106134
[1] XIAO Yun-shu,ZHU Feng-yun-zhi,LUO Lan,XING Xiao-yan,LI Yu-hui,ZHANG Xue-wu,SHEN Dan-hua. Clinical and immunological characteristics of 88 cases of overlap myositis [J]. Journal of Peking University (Health Sciences), 2021, 53(6): 1088-1093.
[2] ZHANG Pu-li,YANG Hong-xia,ZHANG Li-ning,GE Yong-peng,PENG Qing-lin,WANG Guo-chun,LU Xin. Value of serum YKL-40 in the diagnosis of anti-MDA5-positive patients with dermatomyositis complicated with severe pulmonary injury [J]. Journal of Peking University (Health Sciences), 2021, 53(6): 1055-1060.
[3] YI Wen-xia,WEI Cui-jie,WU Ye,BAO Xin-hua,XIONG Hui,CHANG Xing-zhi. Long-term rituximab treatment of refractory idiopathic inflammatory myopathy: A report of 3 cases [J]. Journal of Peking University (Health Sciences), 2021, 53(6): 1191-1195.
[4] Feng-yun-zhi ZHU,Xiao-yan XING,Xiao-fei TANG,Yi-min LI,Miao SHAO,Xue-Wu ZHANG,Yu-hui LI,Xiao-lin SUN,Jing HE. Clinical and immunological characteristics of myositis complicated with thromboembolism [J]. Journal of Peking University (Health Sciences), 2020, 52(6): 995-1000.
[5] Jing ZHAO,Feng SUN,Yun LI,Xiao-zhen ZHAO,Dan XU,Ying-ni LI,Yu-hui LI,Xiao-lin SUN. Significance of anti-tubulin-α-1C autoantibody in systemic sclerosis [J]. Journal of Peking University (Health Sciences), 2020, 52(6): 1009-1013.
[6] Yu-zhou GAN,Yu-hui LI,Li-hua ZHANG,Lin MA,Wen-wen HE,Yue-bo JIN,Yuan AN,Zhan-guo LI,Hua YE. Comparison of clinical and immunological features between clinically amyopathic dermatomyositis and typical dermatomyositis [J]. Journal of Peking University (Health Sciences), 2020, 52(6): 1001-1008.
[7] Yi-ming ZHENG,Hong-jun HAO,Yi-lin LIU,Jing GUO,Ya-wen ZHAO,Wei ZHANG,Yun YUAN. Correlation study on anti-Ro52 antibodies frequently co-occur with other myositis-specific and myositis-associated autoantibodies [J]. Journal of Peking University (Health Sciences), 2020, 52(6): 1088-1092.
[8] Jing XU,Jing XU,He LI,Jie TANG,Jian-long SHU,Jing ZHANG,Lian-jie SHI,Sheng-guang LI. Dermatomyositis combined with IgA vasculitis: A case report [J]. Journal of Peking University(Health Sciences), 2019, 51(6): 1173-1177.
[9] Hong-xia YANG,Xiao-lan TIAN,Wei JIANG,Wen-li LI,Qing-yan LIU,Qing-lin PENG,Guo-chun WANG,Xin LU. Clinical and pathological characteristics of immune mediated necrotizing myopathy [J]. Journal of Peking University(Health Sciences), 2019, 51(6): 989-995.
[10] Yi-ying YANG,Xiao-xia ZUO,Hong-lin ZHU,Si-jia LIU. Advances in epigenetic markers of dermatomyositis/polymyositis [J]. Journal of Peking University(Health Sciences), 2019, 51(2): 374-377.
[11] YU Jian-feng, JIN Yue-bo, HE Jing, AN Yuan, LI Zhan-guo. Changes of serum Krebs von den Lungen-6 levels in interstitial lung disease associated with dermatomyositis and secondary Sjögren’s syndrome: a case report [J]. Journal of Peking University(Health Sciences), 2017, 49(5): 910-914.
[12] XU Jing, LIU Jing,ZHU Lei, ZHANG Xue-wu, LI Zhan-guo. Significance of glucose-6-phosphate isomerase assay in early diagnosis of rheumatoid arthritis [J]. Journal of Peking University(Health Sciences), 2016, 48(6): 942-946.
[13] LIU Shuang, AN Yuan, JIA Yuan, LI Zhan-Guo. A case of clinical overlap syndrome of rheumatoid arthritis and amyopathic dermatomyositis with multiple pulmonary injuries [J]. Journal of Peking University(Health Sciences), 2014, 46(5): 805-808.
[14] WANG Lin-Lin, YANG Na, YUAN Yue, REN Ai-Guo. Establishment and evaluation of enzyme-linked immunosorbent assay for measuring human autoantibody IgG to folate receptor [J]. Journal of Peking University(Health Sciences), 2014, 46(3): 483-487.
[15] LIU Yuan, WANG Yong-Fu, WANG Kai-Li, 吕Feng-Feng . Prevalence and significance of immunoglobulin G-anti-cyclic citrullinated peptide antibodies in primary Sjögren’s syndrome patients [J]. Journal of Peking University(Health Sciences), 2014, 46(3): 478-482.
Viewed
Full text


Abstract

Cited
  Shared   
  Discussed   
[1] Author. English Title Test[J]. Journal of Peking University(Health Sciences), 2010, 42(1): 1 -10 .
[2] . [J]. Journal of Peking University(Health Sciences), 2009, 41(2): 188 -191 .
[3] . [J]. Journal of Peking University(Health Sciences), 2009, 41(3): 376 -379 .
[4] . [J]. Journal of Peking University(Health Sciences), 2009, 41(4): 459 -462 .
[5] . [J]. Journal of Peking University(Health Sciences), 2010, 42(1): 82 -84 .
[6] . [J]. Journal of Peking University(Health Sciences), 2007, 39(3): 319 -322 .
[7] . [J]. Journal of Peking University(Health Sciences), 2007, 39(3): 333 -336 .
[8] . [J]. Journal of Peking University(Health Sciences), 2007, 39(3): 337 -340 .
[9] . [J]. Journal of Peking University(Health Sciences), 2007, 39(3): 225 -328 .
[10] . [J]. Journal of Peking University(Health Sciences), 2007, 39(4): 346 -350 .
Copyright © Journal of Peking University (Health Sciences), All Rights Reserved.
Powered by Beijing Magtech Co. Ltd