Description and Analysis of a Novel Subtype of the Anti-Synthetase Syndrome Characterized by Frequent Attacks of Fever and Systemic Inflammation in a Single-Center Cohort Study.

Abstract

Objectives:

The aim of this study was to investigate anti-synthetase syndrome (ASyS) patients who presented with recurrent episodes of fever and systemic inflammation.

Methods:

A retrospective cohort of Chinese ASyS patients (n=126) in our center (between January 2013 and January 2020) was included. Patients presenting with concomitant autoimmune rheumatic diseases or malignancies were subsequently excluded. The number of non-infectious fever attacks and attack frequency were recorded and calculated. Patients with two or more attacks and within the upper three quartiles of attack frequency were defined as high-inflammation group. Univariate and multivariate analyses were carried out to characterize the high-inflammation subtype.

Results:

Out of 113 eligible patients with an average of 5 years follow up, 25 patients were defined as the high-inflammation group (16 for anti-Jo1, 9 for anti-PL7), with an average of 1.12 attack/patient-year. Compared to low-inflammation group (0-1 attack only and a frequency lower than 0.5 attack/patient-year), the high-inflammation group had higher occurrence of fever and rapid progressive interstitial lung disease (RPILD) as the first presentation (84% vs. 21% and 40% vs. 9%, respectively, both p<0.01). Anti-PL-7 was related to the more inflammatory phenotype (p=0.014). Cumulative disease-modifying agent exposures (>=3) were much higher in the high-inflammation group (60% vs. 26%), while biological agents, i.e., rituximab and tocilizumab, showed better "drug survival" for Jo-1+ and PL-7+ ASyS patients with high inflammation, respectively, in our cohort.

Conclusions:

ASyS with recurrent systemic inflammatory episodes reflects a subtype of more aggressive and refractory disease in the spectrum of ASyS. Increased awareness of this subtype might lead to more appropriate management.

• Authors: Shuhui Sun, Zhiwei Chen, Danting Zhang, Wenwen Xu, Wanlong Wu, Fangfang Sun, Liyang Gu, Jie Chen, Jiajie Li, Ting Li, Xiaodong Wang, Shuang Ye
• Journal: Frontiers in immunology (Front Immunol) Vol. 12 Pg. 729602 ( 2021) ISSN: 1664-3224 [Electronic] Switzerland
• PMID: 34630407 (Publication Type: Journal Article)
• Copyright: Copyright © 2021 Sun, Chen, Zhang, Xu, Wu, Sun, Gu, Chen, Li, Li, Wang and Ye.

Chemical References

• Autoantibodies
• Biological Factors
• Biomarkers
• Immunomodulating Agents
• Amino Acyl-tRNA Synthetases

Topics

• Adult
• Aged
• Amino Acyl-tRNA Synthetases (immunology)
• Autoantibodies (blood)
• Autoimmunity (drug effects)
• Biological Factors (therapeutic use)
• Biomarkers (blood)
• China
• Female
• Fever (diagnosis, drug therapy, enzymology, immunology)
• Humans
• Immunomodulating Agents (therapeutic use)
• Male
• Middle Aged
• Myositis (diagnosis, drug therapy, enzymology, immunology)
• Recurrence
• Retrospective Studies
• Time Factors
• Treatment Outcome