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

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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)

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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
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