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Journal of Peking University (Health Sciences) ›› 2024, Vol. 56 ›› Issue (2): 284-292. doi: 10.19723/j.issn.1671-167X.2024.02.013

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A unicenter real-world study of the correlation factors for complete clinical response in idiopathic inflammatory myopathies

Zhanhong LAI1,Jiachen LI1,Zelin YUN1,Yonggang ZHANG2,Hao ZHANG3,Xiaoyan XING1,Miao SHAO1,Yuebo JIN1,Naidi WANG1,Yimin LI4,Yuhui LI1,*(),Zhanguo LI1,*()   

  1. 1. Department of Rheumatology and Immunology, Peking University People's Hospital, Beijing 100044, China
    2. Department of Rheumatology and Immunology, Baoding First Hospital, Baoding 071000, Hebei, China
    3. Department of Rheumatology and Immunology, Dalian Municipal Central Hospital, Dalian 116089, Liaoning, China
    4. Department of Rheumatology and Immunology, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou 310003, China
  • Received:2023-09-04 Online:2024-04-18 Published:2024-04-10
  • Contact: Yuhui LI,Zhanguo LI E-mail:liyuhui84@163.com;Li99@bjmu.edu.cn

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Abstract:

Objective: To investigate the correlation factors of complete clinical response in idiopathic inflammatory myopathies (IIMs) patients receiving conventional treatment. Methods: Patients diagnosed with IIMs hospitalized in Peking University People's Hospital from January 2000 to June 2023 were included. The correlation factors of complete clinical response to conventional treatment were identified by analyzing the clinical characteristics, laboratory features, peripheral blood lymphocytes, immunological indicators, and therapeutic drugs. Results: Among the 635 patients included, 518 patients finished the follow-up, with an average time of 36.8 months. The total complete clinical response rate of IIMs was 50.0% (259/518). The complete clinical response rate of dermatomyositis (DM), anti-synthetase syndrome (ASS) and immune-mediated necrotizing myopathy (IMNM) were 53.5%, 48.9% and 39.0%, respectively. Fever (P=0.002) and rapid progressive interstitial lung disease (RP-ILD) (P=0.014) were observed much more frequently in non-complete clinical response group than in complete clinical response group. The aspartate transaminase (AST), lactate dehydrogenase (LDH), D-dimer, erythrocyte sedimentation rate (ESR), C-reaction protein (CRP) and serum ferritin were significantly higher in non-complete clinical response group as compared with complete clinical response group. As for the treatment, the percentage of glucocorticoid received and intravenous immunoglobin (IVIG) were significantly higher in non-complete clinical response group than in complete clinical response group. Risk factor analysis showed that IMNM subtype (P=0.007), interstitial lung disease (ILD) (P=0.001), eleva-ted AST (P=0.012), elevated serum ferritin (P=0.016) and decreased count of CD4+T cells in peripheral blood (P=0.004) might be the risk factors for IIMs non-complete clinical response. Conclusion: The total complete clinical response rate of IIMs is low, especially for IMNM subtype. More effective intervention should be administered to patients with ILD, elevated AST, elevated serum ferritin or decreased count of CD4+T cells at disease onset.

Key words: Idiopathic inflammatory myopathies, Clinical response, Interstitial lung disease, Risk factors, Autoantibodies

CLC Number: 

  • R593.26

Figure 1

Flow diagram of study design IIMs, idiopathic inflammatory myopathies; EULAR, European League Against Rheumatism; ACR, American College of Rheumatology."

Table 1

Comparison of clinical characteristics between IIMs CCR group and NCCR group"

Variables CCR (n=259) NCCR (n=259) P value
Female 195 (75.3) 190 (73.4) 0.615
Age at onset/years 49.3±15.0 50.2±13.6 0.460
DM 146 (56.4) 127 (49.0) 0.095
ASS 60 (23.2) 63 (24.3) 0.757
IMNM 46 (17.8) 72 (27.8) 0.006
Fever 75 (29.0) 109 (42.1) 0.002
Dyspnea 103 (39.8) 91 (35.1) 0.276
Gottron’s sign/papules 158 (61.0) 166 (64.1) 0.468
Mechanic’s hands 72 (27.8) 82 (31.7) 0.336
Heliotrope rash 99 (38.2) 110 (42.5) 0.325
ILD 159 (61.4) 189 (73.0) 0.005
  RP-ILD 84 (32.4) 111 (42.9) 0.014
  C-ILD 72 (27.8) 67 (25.9) 0.620

Figure 2

Comparison of laboratory features (A) and MSAs/MAAs (B) between IIMs CCR group and NCCR group * P < 0.05, NCCR group compared with CCR group. a, 319 values missing; b, 319 values missing; c, 293 values missing; d, 320 values missing; e, 326 values missing; f, 280 values missing; g, 317 values missing; h, 314 values missing; i, 324 values missing; j, 331 values missing; k, 167 values missing. IIMs, idiopathic inflammatory myopathies; CCR, complete clinical response; NCCR, non-complete clinical response; MSAs, myositis specific antibodies; MAAs, myositis associated antibodies; DM, dermatomyositis; ASS, anti-synthetase syndrome; IMNM, immune-mediated necroti-zing myopathy; ILD, interstitial lung disease; RP-ILD, rapid progressive interstitial lung disease; C-ILD, chronic interstitial lung disease; Anti TIF-1γ, anti-transcriptional intermediary factor 1γ; Anti MDA5, anti-melanoma differentiation-associated protein 5; Anti NXP2, anti-nuclear matrix protein 2; Anti SAE1, anti-small ubiquitin-like modifier activating enzyme 1; Anti Jo-1, anti-histidyl-tRNA synthetase; Anti SRP, anti-signal recognition particle; Anti PL-7, anti-threonyl-tRNA synthetase; Anti PL-12, anti-alanyl-tRNA synthetase; Anti EJ, anti-glycyl-tRNA synthetase; Anti OJ, anti-isoleucyl-tRNA synthetase."

Table 2

Comparison of laboratory features between IIMs CCR group and NCCR group"

Variables CCR (n=259) NCCR (n=259) P value
WBC/(×109/L) 7.53±3.37 7.81±3.66 0.363
Lymphocyte/(×109/L) 1.47±0.84 1.36±0.84 0.146
PLT/(×109/L) 228.0±84.5 223.0±83.1 0.483
ALT/(U/L) 33.0 (20.3, 56.0) 45.5 (20.0, 103.0) 0.509
AST/(U/L) 34.0 (24.2, 69.0) 63.5 (31.0, 132.0) 0.008
LDH/(U/L) 289 (232, 519) 429 (290, 631) 0.001
CK/(U/L) 77.0 (34.5, 251.0) 76.0 (26.8, 775.0) 0.627
D-dimer/(μg/L) 322 (195, 487) 425 (282, 714) 0.002
ESR/(mm/L) 17.5 (8.9, 41.8) 36.5 (12.8, 58.3) 0.003
CRP/(mg/L) 3.43 (0.63, 20.40) 8.03 (2.00, 28.50) 0.002
Ferritin/(μg/L)a 229 (83, 1 116) 566 (272, 1 588) 0.020
IgG/(g/L) 14.50±6.43 13.90±5.37 0.214
C3/(g/L) 1.00±0.27 1.01±0.27 0.760
C4/(g/L) 0.25±0.12 0.24±0.10 0.575

Figure 3

Comparison of peripheral blood lymphocytes between IIMs CCR group and NCCR group IIMs, idiopathic inflammatory myopathies; CCR, complete clinical response; NCCR, non-complete clinical response; T cell, T lymphocyte cell; B cell, B lymphocyte cell; NK cell, natural killer cell; TNF, tumor necrosis factor; IFN, interferon; IL, interleukin."

Table 3

Comparison of initial treatment between IIMs CCR group and NCCR group"

Variables CCR (n=259) NCCR (n=259) P value
Glucocorticoid, n (%) 206 (79.5) 226 (87.3) 0.018
  Prednisone ≤ 15 mg/da 63 (24.4) 49 (19.0) 0.135
  15 mg/d＜prednisone＜30 mg/da 9 (3.5) 9 (3.5) ＞0.999
  Prednisone ≥ 30 mg/da 119 (46.1) 125 (48.4) 0.597
  Pulse IVMP 67 (25.9) 75 (29.0) 0.431
Immunosuppressants, n (%) 166 (64.1) 156 (60.2) 0.365
  CTX 96 (37.1) 94 (36.3) 0.855
  CSA 54 (20.8) 51 (19.7) 0.743
  TAC 10 (3.9) 17 (6.6) 0.166
  MMF 20 (7.7) 11 (4.2) 0.095
JAKi, n (%) 2 (0.8) 8 (3.1) 0.055
IVIG, n (%) 25 (9.7) 42 (16.2) 0.026

Figure 4

Multivariate Logistic regression analysis of factors related to IIMs CCR IIMs, idiopathic inflammatory myopathies; CCR, complete clinical response; IMNM, immune-mediated necrotizing myopathy; ILD, interstitial lung disease; AST, aspartate aminotransferase; T cell, T lymphocyte cell."

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