Journal of Peking University (Health Sciences) ›› 2020, Vol. 52 ›› Issue (6): 995-1000. doi: 10.19723/j.issn.1671-167X.2020.06.002

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Clinical and immunological characteristics of myositis complicated with thromboembolism

Feng-yun-zhi ZHU1,Xiao-yan XING2,Xiao-fei TANG3,Yi-min LI1,Miao SHAO1,Xue-Wu ZHANG1,Yu-hui LI1,(),Xiao-lin SUN1,Jing HE1   

  1. 1. Department of Rheumatology and Immunology, Peking University People’s Hospital, Beijing 100044, China
    2. Department of Cardiac Electrophysiology, Peking University People’s Hospital, Beijing 100044, China
    3. Department of Nephrology and Rheumatology, Aerospace Center Hospital, Beijing 100069, China
  • Received:2020-08-10 Online:2020-12-18 Published:2020-12-13
  • Contact: Yu-hui LI E-mail:liyuhui84@163.com
  • Supported by:
    National Natural Science Foundation of China(81801617);Peking University People’s Hospital Research and Development Funds(RDY2018-01);Peking University People’s Hospital Research and Development Funds(RS2018-02);Peking University People’s Hospital Research and Development Funds(RDE2019-02);New Teaching Approach Funds of Peking University(2020YX006)

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Abstract:

Objective: To investigate and analyse the clinical and immunological features of patients with myositis complicated with thromboembolism. Methods: We identified a cohort of 390 myositis patients diagnosed with myositis admitted to People’s Hospital of Peking University from 2003 to 2019. The patients were retrospectively enrolled in this investigation. According to the outcome of the color Doppler ultrasound, CT pulmonary angiography, pulmonary ventilation and perfusion scan patients were divided into myositis with and without thromboembolism group. Demographic, clinical (heliotrope rash, Gottron’s sign/papules, periungual erythema, skin ulceration, subcutaneous calcinosis, Mechanic’s hands, myalgia, interstitial lung disease, pulmonary arterial hypertension), laboratory, immunological [anti-autoantibodies including melanoma differentiation associated gene 5 (anti-MDA5), anti-Mi-2, anti-transcription intermediary factor-1γ (anti-TIF-1γ, anti-nuclear matrix protein 2 (anti-NXP2), anti-small ubiquitin-like modifier activating enzyme (anti-SAE), anti-synthetase], imaging and therapeutic status data of the patients at the diagnosis of myositis with and without thromboembolism were collected and the differences in these data were analyzed. Logistic regressive analysis was used to identify the risk factors of thromboembolism. Results: In the retrospective study, 390 myositis patients were investigated. The mean age of onset was (49.6±13.4) years, male to female ratio was 0.31:1. Thromboembolism was identified in 4.62% (18/390) of the myositis patients, which was lower than the published reports. Out of 18 patients with thromboembolism, 55.6% (10/18) of them were deep venous thrombosis, followed by cerebral infarction (22.2%, 4/18), pulmonary embolism (11.1%, 2/18), renal artery embolism (5.6%, 1/18) and embolism of upper extremity (5.6%, 1/18). Fifty percent of thromboembolism events occurred 6 months after the diagnosis of myositis, 38.9% of thromboembolism events occurred 6 months within the diagnosis of myositis, 11.1% of thromboembolism events occurred 6 months before the diagnosis of myositis. As compared with the myositis patients without thromboembolism, the myositis patients complicated with thromboembolism were older [(58.3±11.7) years vs. (49.3±13.4) years, P=0.006]. C-reaction protein (CRP) (12.2 mg/L vs. 4.1 mg/L, P<0.001), ferritin (20 085.5 μg/L vs. 216.6 μg/L, P<0.001) and D-dimer (529.0 μg/L vs. 268.0 μg/L, P=0.002) were significantly higher in thromboembolism group. Diabetes (44.4% vs. 16.4%, P=0.006), coronary heart disease (22.2% vs. 3.0%, P=0.003) and surgery (16.7% vs. 3.5%, P=0.032) were observed more common in thromboembolism group than those without thromboembolism. Activated partial thromboplastin time (APTT) (26.9 s vs. 28.7 s, P=0.049) and albumin (32.4 g/L vs. 36.5 g/L, P=0.002) was lower in thromboembolism group. The risk factors of thromboembolism in the myositis patients were low level of albumin (OR=0.831, 95%CI: 0.736-0.939, P=0.003), diabetes (OR=4.468, 95%CI: 1.382-14.448, P=0.012), and coronary heart disease (OR=22.079, 95%CI: 3.589-135.837, P=0.001) were independent significant risk factors for thromboembolism in the patients with myositis. There was no significant difference in clinical manifestations, myositis-specific antibodies or myositis-associated antibodies between the two groups. Conclusion: Thromboembolism is a complication of myositis. Lower levels of albumin, diabetes, and coronary heart disease might be risk factors of thromboembolism in myositis patients.

Key words: Myositis, Autoimmune diseases, Thromboembolism, Autoantibodies

CLC Number: 

  • R593.2

Table 1

General characteristics of the patients with myositis"

Variables Myositis (n=390)
Demographics
Age at onset/years, x-±s 49.6±13.4
Female, n(%) 297 (76.2)
Duration/months, M (P25, P75) 6 (2, 20)
Types of TE, n(%)
DVT 10 (55.6)
Cerebral infarction 4 (22.2)
PE 2 (11.1)
Others 2 (11.1)

Figure 1

Time points of the occurrence of thromboembolism in 18 patients with myositis"

Table 2

Comparison of demographics between myositis patients with or without thromboembolism"

Variables Myositis with TE (n=18) Myositis without TE (n=372) P
Demographics
Female, n(%) 14 (77.8) 283 (76.1) 0.565
Age at onset/years, x-±s 58.3±11.7 49.3±13.4 0.006
Duration/months, M (P25, P75) 9 (3.3, 42.0) 6 (2.0, 18.5) 0.276
Survival, n(%) 17 (94.4) 357 (96.0) 0.538
PM, n(%) 3 (16.7) 45 (12.1) 0.584
DM, n(%) 8 (44.4) 211 (56.7) 0.584
CADM, n(%) 7 (38.9) 116 (31.2) 0.584
Risk factors
BMI/(kg/m2), M (P25, P75) 23.8 (23.0, 26.1) 23.5 (21.3, 25.8) 0.373
Cigarette, n(%) 6 (33.3) 57 (15.3) 0.053
Hypertension, n(%) 7 (38.9) 85 (22.9) 0.151
Diabetes, n(%) 8 (44.4) 61 (16.4) 0.006
Coronary heart disease, n(%) 4 (22.2) 11 (3.0) 0.003
Malignancy, n(%) 3 (16.7) 31 (8.3) 0.200
Varicose veins, n(%) 0 5 (1.3) 1.000
Surgery, n(%) 3 (16.7) 13 (3.5) 0.032

Table 3

Clinical and laboratory features between myositis patients with or without thromboembolism"

Variables Myositis with TE (n=18) Myositis without TE (n=372) P
Clinical features, n(%)
Heliotrope rash 11 (61.1) 171 (46.0) 0.208
Gottron’s sign (papules) 11 (61.1) 238 (64.0) 0.805
Periungual erythema 1 (5.6) 69 (18.6) 0.217
Skin ulceration 1 (5.6) 17 (4.6) 0.581
Subcutaneous calcinosis 1 (5.6) 6 (1.6) 0.284
Mechanic’s hands 2 (11.1) 103 (27.7) 0.173
Myalgia 1 (5.6) 38 (10.2) 1.000
ILD 13 (72.2) 237 (63.7) 0.462
PAH 6 (33.3) 61 (16.4) 0.100
Laboratory features
CK/(U/L), M (P25, P75) 41.0 (22.5, 944.8) 116.0 (40.0, 674.0) 0.141
CRP/(mg/L), M (P25, P75) 12.2 (7.2, 39.0) 4.1 (1.4, 14.3) <0.001
ESR/(mm/h), M (P25, P75) 25.0 (9.3, 56.8) 23.0 (11.0, 42.0) 0.779
Ferritina /(μg/L), M (P25, P75) 20 085.5 (1 039.3, 28 069.3) 216.6 (83.0, 879.7) <0.001
Elevated cTnI, n(%) 6 (35.3) 34 (21.8) 0.729
D-dimer/(μg/L), M (P25, P75) 529.0 (258.0, 1156.5) 268.0 (122.0, 492.0) 0.002
Fibrinogen/(g/L), M (P25, P75) 3.6 (2.4, 4.6) 3.1 (2.6, 3.6) 0.492
PT/s, M (P25, P75) 10.9 (10.4, 11.8) 11.0 (10.3, 11.7) 0.965
APTT/s, M (P25, P75) 26.9 (25.9, 29.0) 28.7 (26.7, 31.3) 0.049
Alb/(g/L), M (P25, P75) 32.4 (28.5, 35.8) 36.5 (33.0, 39.0) 0.002
MSAsb, n(%)
Anti-MDA5 positivity 2/14 (14.3) 43/221 (19.5) 1.000
Anti-Mi-2 positivity 2/14 (14.3) 12/221 (5.4) 0.199
Anti-TIF-1γ positivity 1/14 (7.1) 14/221 (6.3) 1.000
Anti-NXP2 positivity 1/14 (7.1) 13/221 (5.9) 0.587
Anti-SAE positivity 1/14 (7.1) 5/221 (2.3) 0.311
Anti-synthetase positivity 3/14 (21.4) 76/221 (34.2) 0.394
MAAsb, n(%)
Anti-PM-Scl75/100 positivity 1/14 (7.1) 15/221 (6.8) 1.000
Anti-Ro-52 positivity 9/14 (64.3) 105/221 (47.5) 0.276
Anti-Ku positivity 0/14 (0) 9/221 (4.1) 1.000
Glucocorticoid pulse therapy, n(%) 5 (27.8) 83 (22.3) 0.569
Cyclophosphamide, n(%) 10 (55.6) 145 (39.0) 0.217
Other immunosuppressants, n(%) 4 (22.2) 71 (19.1) 0.760
Anticoagulant drug, n(%) 1 (5.6) 9 (2.4) 0.380

Table 4

Risk factors of thromboembolism in patients with myositis"

Variables OR 95%CI P
Albumin 0.831 0.736-0.939 0.003
Diabetes 4.468 1.382-14.448 0.012
Coronary heart disease 22.079 3.589-135.837 0.001
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