Clinical features and outcomes of the patients with anti-glycyl tRNA synthetase syndrome

Zhang, Yinli; Ge, Yongpeng; Yang, Hanbo; Chen, He; Tian, Xiaowen; Zhenguo, Huang; Liu, Shengyun; Lü, Xin; Wang, Guochun

doi:10.1007/s10067-020-04979-8

Cited by 16 publications

(20 citation statements)

References 28 publications

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“…Therefore, the value of changes in follow-up data for PFT in predicting the outcome of ILD needs to be further confirmed in future studies. In terms of imaging, HRCT of patients with ASS-ILD is characterized by axial, peripheral, and coronal distributions ( 28 ). The imaging type of pulmonary interstitial disease is mainly NSIP, followed by NSIP-OP ( 28 ).…”

Section: Discussionmentioning

confidence: 99%

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Clinical features, prognostic factors, and survival of patients with antisynthetase syndrome and interstitial lung disease

Zhao

Jiang

et al. 2022

Front. Immunol.

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ObjectiveThis study aimed to analyze the clinical features and prognostic factors of imaging progression and survival in patients with antisynthetase syndrome (ASS) complicated by interstitial lung disease (ILD) in a large Chinese cohort.MethodsMedical records, imaging, and serological data of 111 patients with ASS-ILD (positive for at least one of the following autoantibodies: anti-Jo1, anti-PL7, anti-PL12, and anti-EJ) from the Affiliated Yantai Yuhuangding Hospital of Qingdao University database were retrospectively investigated. According to the changes in high-resolution computed tomography (HRCT) outcomes at 1 year follow-up, Patients were categorized into three groups: the regression, stability, and deterioration groups. Univariate analysis was performed to evaluate the possible prognostic factors of ILD outcome and death, and multivariate analysis was performed to determine the independent predictors of ASS-ILD outcome and death by logistic regression.ResultsThe number of CD3-CD19+ cells and initial glucocorticoid dosage were correlated with imaging progression, and may be independent risk factors for ILD deterioration. Dyspnea as the first symptom, hypohemoglobinemia, the serum ferritin level, oxygen partial pressure at diagnosis, and different treatment types were important factors affecting survival, and the initial serum ferritin level may be an independent risk factor for survival.ConclusionsThe clinical characteristics of patients with ASS-ILD with different antisynthetase antibody subtypes are different. An increase in the CD3-CD19+ cell level is an independent risk factor for the deterioration of HRCT imaging. Early intensive treatment with high-dose glucocorticoids can effectively improve imaging prognosis of ILD. Patients with significantly elevated serum ferritin levels should be treated intensively.

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Section: Discussionmentioning

confidence: 99%

“…In terms of imaging, HRCT of patients with ASS-ILD is characterized by axial, peripheral, and coronal distributions ( 28 ). The imaging type of pulmonary interstitial disease is mainly NSIP, followed by NSIP-OP ( 28 ). The results of this study showed that NSIP-OP is the most common ILD type of ASS-ILD, followed by NSIP.…”

Section: Discussionmentioning

confidence: 99%

Clinical features, prognostic factors, and survival of patients with antisynthetase syndrome and interstitial lung disease

Zhao

Jiang

et al. 2022

Front. Immunol.

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“…Likewise, none of the EJ+, PL-12, or OJ+ ASyS patients in our cohort fulfilled the high-inflammation subtype definition, which might simply be because of the small number of patients. As an example, in a larger single-center Chinese ASyS cohort (n=234), the authors reported 46 EJ+ patients who had similar occurrences of fever (60.9%) and RPILD (21.7%) ( 6 ). Some patients might well be labeled as high-inflammation subtype.…”

Section: Discussionmentioning

confidence: 99%

“…It is noteworthy that fever is a common and prominent clinical manifestation of ASyS, which is different from other IIMs, with a reported incidence varying from 26% to 61% (5,6). Although most disease courses of ASyS are chronic with goodto-moderate response to conventional immunosuppressive treatments, patients who presented with recurrent episodes of fever and elevated acute phase reactants due to systemic inflammation were deemed to be more aggressive and difficult to treat.…”

Section: Introductionmentioning

confidence: 99%

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

et al. 2021

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

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“…Anti-EJ antibody is the least common antibody associated with NSIP and has mainly been reported in patients with dermatomyositis-associated ILD [ 7 ]. In a study of Chinese patients with anti-glycyl tRNA synthetase syndrome, ILD-related symptoms were the most common initial manifestations and most prevalent features [ 8 ]. In another study, the prevalence of NSIP reached 94.4% [ 9 ].…”

Section: Discussionmentioning

confidence: 99%

A Rare Case of Anti-glycyl transfer RNA (tRNA) Synthetase Antibody-Related Non-specific Interstitial Pneumonia

Chhetri¹,

Gole²,

Mallari³

et al. 2022

Cureus

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This is a case of a 65-year-old female with a past medical history of type 2 diabetes mellitus (DM) and hypothyroidism who presented with a five-day history of shortness of breath, dry cough, and fatigue. Shortness of breath was exertional, and cough was intermittent. She had no exposure to COVID-19 infection. During the presentation, the patient required supplemental oxygen up to 6 liters per minute (L/m) and was tachypneic and tachycardic. Initial computed tomography (CT) of the chest revealed bilateral parenchymal disease compatible with COVID-19 pneumonia, however, the patient's COVID-19 polymerase chain reaction (PCR) test was persistently negative. Despite being treated for COVID-19 pneumonia, the patients' oxygen requirement increased, leading to the requirement of non-invasive positive pressure ventilation (BiPAP -bilevel positive airway pressure). The pulmonologist initiated a workup for possible underlying interstitial lung disease (ILD). Anti-glycyl transfer RNA (anti-EJ) antibody was positive on two occasions. The patient was started on pulse dose steroid and long-term steroid taper. The patient responded very well to the steroid and was later able to wean off the oxygen to room air. High-resolution CT which was done 3 months after the hospital stay revealed features suggestive of non-specific interstitial pneumonia (NSIP). Anti-synthetase syndrome is a rare but treatable etiology of ILD and should always be considered as a differential during workups.

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Clinical features and outcomes of the patients with anti-glycyl tRNA synthetase syndrome

Cited by 16 publications

References 28 publications

Clinical features, prognostic factors, and survival of patients with antisynthetase syndrome and interstitial lung disease

Clinical features, prognostic factors, and survival of patients with antisynthetase syndrome and interstitial lung disease

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

A Rare Case of Anti-glycyl transfer RNA (tRNA) Synthetase Antibody-Related Non-specific Interstitial Pneumonia

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