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Journal of Peking University (Health Sciences) ›› 2024, Vol. 56 ›› Issue (6): 980-986. doi: 10.19723/j.issn.1671-167X.2024.06.006

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Analysis of clinical features of ruccrent interstitial lung disease in patients with anti-EJ positive antisynthetase syndrome

Yujing ZHU, Lei WANG, Chengyin LYU, Wenfeng TAN, Miaojia ZHANG*()   

  1. Department of Rheumatology and Immunology, the First Affiliated Hospital of Nanjing Medical University, Nanjing 210029, China
  • Received:2024-08-01 Online:2024-12-18 Published:2024-12-18
  • Contact: Miaojia ZHANG E-mail:miaojia_zhang@163.com
  • Supported by:
    the Clinical Capacity Enhancement Project of the First Affiliated Hospital of Nanjing Medical University(JSPH-MC-2023-29)

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Abstract:

Objective: To summarize the clinical characteristics of 57 patients diagnosed with anti-glycyl tRNA synthetase (anti-EJ) positive antisynthetase syndrome (ASS), a subtype of anti-glycyl tRNA positive ASS, complicated by interstitial lung disease (ILD), and to investigate the factors asso-ciated with ILD recurrence. Methods: A retrospective analysis was conducted on the clinical data of 57 anti-EJ positive ASS patientswho were treated at the First Affiliated Hospital of Nanjing Medical University from January 1, 2020 to June 30, 2024. The data collected included demographic information, clinical characteristics, laboratory test results, chest CT findings, and pulmonary function tests. The characteristics of ILD recurrence were also analyzed. Results: All the 57 patients with anti-EJ positive ASS were diagnosed with ILD. The mean age at disease onset was (58.18±10.27) years, with a mean disease duration of 3.00 (2.00, 16.00) months. Among the patients, 70. 18% were female, 87.72% experienced a cough, 70. 18% had expectoration, 89.47% reported respiratory difficulties, and 14.04% developed respiratory failure. The results of pulmonary function test showed that the percentage of forced vital capacity (FVC) in the normal predicted value (FVC%), the percentage of forced expiratory volume in the first second (FEV1) in the normal predicted value (FEV1%) and the percentage of diffusion lung carbon monoxide (DLCO) in the normal predicted value (DLCO%) were 59.36±21.41, 58.34±19.46 and 58.17±27.95, respectively. The oxygenation index was (363.24±99.42) mmHg. Chest CT imaging showed that nonspecific interstitial pneumonia (NSIP) was the most common radiographic pattern. Among the 46 patients who completed a follow-up of more than 12 months, 21 cases (45.65%) showed recurrence of ILD. The average age of onset for the recurrence group was (61.38±8.63) years, while that for the non-recurrence group was (55.28±11.85) years, with a difference approaching statistical significance (P=0.056). Further analysis showed that the ESR (erythrocyte sedimentation rate) level was significantly higher in the recurrence group than in the non-recurrence group [(50.48±29.64) mm/h vs. 30.28±23.97) mm/h, P=0.025], and the IgM (immune globulin M) level was also significantly higher in the recurrence group (P=0.042). Moreover, the CD8+T proportion was significantly higher in the recurrence group than in the non-recurrence group (25.48±11.81 vs. 18.59± 8.53, P=0.027). Despite the fact that the recurrence group had a higher baseline age, higher ESR, IgM, and CD8+T proportion, multivariate binary logistic regression analysis showed that these indicators were not independent risk factors for ILD recurrence. Conclusion: ILD is the most common clinical manifestation in patients with anti-EJ positive ASS, with a significant impact on pulmonary function. Although the patients responded well to a combination of glucocorticoid and immunosuppressive therapies, the recurrence rate remains high, particularly in those with increased sputum production, and elevated ESR. Close monitoring and early intervention for high-risk patients are essential to improving long-term outcomes.

Key words: Antisynthetase syndrome, Interstitial lung disease, anti-glycyl tRNA antisynthetase antibody, Recurrence

CLC Number: 

  • R563

Figure 1

Flowchart of clinical data analysis anti-EJ, anti-glycyl tRNA synthetase; ASS, antisynthetase syndrome; ILD, interstitial lung disease."

Table 1

Clinical features between ruccrence group and non-ruccrence group in anti-EJ positive ASS-ILD patients"

Clinical parameters Overall (n=57) Ruccrence (n=21) Non-ruccrence (n=25) P
Female 40 (70.18) 17 (80.95) 18 (72.00) 0.514
Age/years 58.18±10.27 61.38±8.63 55.28±11.85 0.056
ILD diagnosis time/months 3.00 (2.00, 16.00) 3.00 (2.00, 10.00) 2.50 (1.75, 24.00) 0.461
Cough 50 (87.72) 20 (95.24) 21 (84.00) 0.351
Sputum 40 (70.18) 18 (85.71) 15 (60.00) 0.099
Dyspnea 51 (89.47) 19 (90.48) 23 (92.00) 1.000
Respiratory failure 8 (14.04) 2 (9.52) 3 (12.00) 1.000
Fever 13 (22.81) 3 (14.29) 7 (28.00) 0.306
Mechanic’s hands 16 (28.07) 5 (23.81) 7 (28.00) 1.000
DM rash 16 (28.07) 6 (28.57) 9 (36.00) 0.754
Myocardial injury 14 (24.56) 6 (28.57) 8 (32.00) 1.000
Myalgia 20 (35.09) 4 (19.05) 7 (28.00) 0.514
Arthritis 11 (19.30) 5 (23.81) 4 (16.00) 0.711
Ro-52 51 (89.47) 18 (85.71) 23 (92.00) 0.648
Treatment (baseline)
TAC 30 (52.63) 11 (52.38) 14 (56.00) 1.000
CYC 15 (26.32) 5 (23.81) 6 (24.00) 1.000
MMF 4 (7.18) 4 (19.05) 0 (0.00) 0.024
Glucocorticoid dosage/(mg/d) 63.68±45.73 72.14±50.01 53.40±28.32 0.118

Table 2

Comparison of baseline laboratory parameters and pulmonary function between the ruccrence group and non-ruccrence group"

Category Overall (n=57) Ruccrence (n=21) Non-ruccrence (n=25) P
WBC/(×109/L) 8.40±4.21 9.74±3.67 8.78±3.17 0.346
lymphocyte count/(×109/L) 1.42±0.87 1.34±0.60 1.56±1.13 0.423
ESR/(mm/h) 40.63±27.80 50.48±29.64 30.28±23.97 0.014
CRP/(mg/L) 12.77±16.48 12.74±14.96 9.85±12.33 0.476
SF/(μg/L) 302.34±253.00 282.96±215.12 256.89±184.98 0.665
CK/(U/L) 80.00 (32.00, 375.50) 45.00 (24.50, 78.00) 238.50 (32.50, 1 291.50) 0.428
LDH/(U/L) 292.00 (245.50, 378.00) 271.00 (244.50, 277.75) 368.00 (269.75, 419.75) 0.275
IgG/(g/L) 12.40 (9.86, 15.20) 11.35 (10.83, 13.13) 13.05 (10.33, 22.73) 0.193
IgM/(g/L) 1.87 (1.17, 3.06) 2.02 (1.62, 3.68) 1.68 (0.98, 2.85) 0.042
IgA/(g/L) 2.64±1.07 2.57±1.20 2.73±1.06 0.626
CD3+ T/% 61.17±12.41 59.98±17.00 61.77±9.33 0.654
CD4+ T/% 39.26±11.43 36.23±12.55 41.6±10.75 0.125
CD8+ T/% 21.36±10.38 25.48±11.81 18.59±8.53 0.027
Oxygen index/mmHg 363.24±99.42 360.69±99.58 386.11±106.34 0.477
FVC% 59.36±21.41 60.45±25.13 64.71±17.38 0.578
FEV1% 58.34±19.46 58.05±21.49 64.07±16.83 0.381
DLCO% 58.17±27.95 60.67±32.52 56.78±22.61 0.694

Table 3

Comparison of laboratory parameters at baseline and at reccurence in 21 patients with ASS"

Category Baseline Ruccrence P
WBC/(×109/L) 9.74±3.67 9.55±3.21 0.797
lymphocyte count/(×109/L) 1.34±0.50 1.20±0.62 0.462
Neutrophils count/(×109/L) 7.66±3.49 7.72±3.03 0.928
Hb/(g/L) 123.33±13.44 128.67±11.70 0.925
PLT/(×109/L) 269.95±77.29 258.17±69.44 0.422
CK/(U/L) 82.00 (45.00, 216.50) 93.00 (35.00, 134.00) 0.068
LDH/(U/L) 342.95±135.26 287.24±91.68 0.095
SF/(μg/L) 248.50 (145.80, 323.37) 314.32 (162.85, 314.32) 0.622
CRP/(mg/L) 7.98 (3.51, 10.85) 18.20 (7.26, 29.32) 0.053
ESR/(mm/h) 50.48±29.64 31.14±14.57 0.003
IgG/(g/L) 14.59±4.78 11.62±3.68 0.004
IgA/(g/L) 2.57±1.20 2.02±1.22 0.002
IgM/(g/L) 2.85±2.36 1.79±1.06 0.005

Table 4

Chest CT classification of ASS-ILD patients with positive anti-EJ antibodies"

Group Overall (n=57) Ruccrence (n=21) Non-ruccrence (n=25)
NISP 40 (70.18) 16 (76.19) 19 (76.00)
OP 4 (7.02) 1 (4.76) 1 (4.00)
UIP 6 (10.53) 2 (9.52) 3 (12.00)
NSIP+OP 2 (3.51) 0 (0.00) 1 (4.00)
NSIP+UIP 4 (7.02) 2 (9.52) 1 (4.00)
LIP 1 (1.75) 0 (0.00) 1 (4.00)

Table 5

Multifactorial binary Logistic analysis of ILD relapse"

Items β s t P OR OR(95%CI)
ESR 0.023 0.013 2.850 0.091 1.023 0.996, 1.050
IgM 0.203 0.293 0.482 0.488 1.226 0.690, 2.176
CD8+ T 0.065 0.036 3.273 0.070 1.067 0.995, 1.144
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