Analysis of the clinical features of antisynthetase syndrome: a retrospective cohort study in China

Wang, Ruoming; Zhao, Yingna; Qi, Fumin; Wu, Xiuhua; Wang, Yingai; Xu, Yong; Wu, Yue; Zhang, Na; Hou, Hou; Sun, WenWen; Li, Xin; Wei, Wei

doi:10.1007/s10067-022-06404-8

Cited by 12 publications

(13 citation statements)

References 20 publications

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“…Although our study could not discover a link between individual anti‐ASS antibodies and PF‐ILD, there is research addressing the influence of anti‐ASS antibodies on ILD. Our previous research 21 found that ASS patients with anti‐EJ antibodies had high prevalence of ILD as well the tendency to represent ILD as the initial symptom. Further, the HRCT of this subgroup showed more serious imaging findings.…”

Section: Discussionmentioning

confidence: 94%

Predictive factors for progressive fibrosing interstitial lung disease in anti‐synthetase syndrome

Zhao

Zhang

et al. 2023

Int J of Rheum Dis

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Objectives Interstitial lung disease (ILD) is common in anti‐synthetase syndrome (ASS). Progressive fibrosing ILD (PF‐ILD) may develop in ILD with autoimmune features. Data on PF‐ILDs in ASS as a group are scarce. This study aimed to explore the characteristics and predictors of PF‐ILD in ASS patients. Methods This retrospective study enrolled 96 ASS‐ILD patients. Baseline clinical data were collected. PF‐ILD assessments were conducted at every hospital visit during windows of 24 months after initial diagnosis. Phenotypic, survival features and predictors of PF‐ILD were estimated through SPSS 22.0. Results The results revealed that 35.42% (34/96) were evaluated to be PF‐ILD with a median interval time of 14.73 months. Nonspecific interstitial pneumonia was the most common radiological pattern of PF‐ILD. Ground glass opacity (GGO), traction bronchiectasis and reticulation were representative high‐resolution computed tomography findings of this group. Compared with the non‐progressive group, PF‐ILD patients had higher frequencies of anti‐Ro‐52 antibodies (91.18% vs 66.13%, P = 0.007) and GGO in the lower + middle and lower + middle + upper zones of the left lung, as well as lower + middle zones in the right lung (85.30% vs 54.84%, P = 0.003; 64.71% vs 38.71%, P = 0.015; 82.35% vs 58.06%, P = 0.016). Multivariate Cox analysis identified that anti‐Ro‐52 antibody (hazards ratio [HR] 3.55, 95% CI 1.06–11.90, P = 0.040) and GGO in left lower + middle lung zones (HR 22.11, 95% CI 1.95–250.90, P = 0.012) were independent risk factors for PF‐ILD. Conclusions PF‐ILD was associated with poor prognosis. Over one‐third of ASS‐ILD patients may develop to PF‐ILD. Anti‐Ro‐52 antibody positivity and GGO in left lower + middle lung zones were independent risk factors for PF‐ILD in ASS patients.

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Section: Discussionmentioning

confidence: 94%

Predictive factors for progressive fibrosing interstitial lung disease in anti‐synthetase syndrome

Zhao

Zhang

et al. 2023

Int J of Rheum Dis

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“…Co-existence of anti-Ro-52 antibodies has been shown by several investigators to associate with worse lung disease in ASyS. 17,23,[70][71][72][73][74] However, this has not been universally observed especially in larger more diverse populations. 18,53,55,75 Based on these discrepant reports, it is unclear to what degree anti-Ro-52 contributes to worse pulmonary manifestations in ASyS.…”

Section: Pulmonary Manifestations Interstitial Lung Diseasementioning

confidence: 99%

“…Moreover, antibody levels can fluctuate through the disease course depending on disease activity and in response to treatment. [127][128][129] Table 1 Antisynthetase antibodies and prevalence Antisynthetase antibody tRNA synthetase auto-antigen Prevalence (%) [14][15][16][17][18][19][22][23][24][25][26][27]50,77,88…”

Section: Antisynthetase Antibodiesmentioning

confidence: 99%

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Review of Pulmonary Manifestations in Antisynthetase Syndrome

Ghanbar,

Danoff

2024

Semin Respir Crit Care Med

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Antisynthetase syndrome (ASyS) is now a widely recognized entity within the spectrum of idiopathic inflammatory myopathies. Initially described in patients with a triad of myositis, arthritis, and interstitial lung disease (ILD), its presentation can be diverse. Additional common symptoms experienced by patients with ASyS include Raynaud's phenomenon, mechanic's hand, and fever. Although there is a significant overlap with polymyositis and dermatomyositis, the key distinction lies in the presence of antisynthetase antibodies (ASAs). Up to 10 ASAs have been identified to correlate with a presentation of ASyS, each having manifestations that may slightly differ from others. Despite the proposal of three classification criteria to aid diagnosis, the heterogeneous nature of patient presentations poses challenges. ILD confers a significant burden in patients with ASyS, sometimes manifesting in isolation. Notably, ILD is also often the initial presentation of ASyS, requiring pulmonologists to remain vigilant for an accurate diagnosis. This article will comprehensively review the various aspects of ASyS, including disease presentation, diagnosis, management, and clinical course, with a primary focus on its pulmonary manifestations.

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“…Interestingly, emerging data have identified several novel autoantibodies targeting other ARSs 8 9. A growing number of studies on ASS patients have attempted to relate anti-ARS specificity to distinct clinical features,10–12 and particular presentations with specific anti-ARS antibodies have been reported, such as anti-Jo1 patients presenting with a higher frequency of joint manifestations,13 14 and those with anti-PL7/PL12 antibodies presenting with more severe lung involvement 14 15. However, the association of anti-ARS antibodies with prognosis remains unclear.…”

Section: Introductionmentioning

confidence: 99%

Novel endotypes of antisynthetase syndrome identified independent of anti-aminoacyl transfer RNA synthetase antibody specificity that improve prognostic stratification

Wu,

Xiao,

Zhang

et al. 2024

Ann Rheum Dis

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ObjectivesTo systemically analyse the heterogeneity in the clinical manifestations and prognoses of patients with antisynthetase syndrome (ASS) and evaluate the transcriptional signatures related to different clinical phenotypes.MethodsA total of 701 patients with ASS were retrospectively enrolled. The clinical presentation and prognosis were assessed in association with four anti-aminoacyl transfer RNA synthetase (ARS) antibodies: anti-Jo1, anti-PL7, anti-PL12 and anti-EJ. Unsupervised machine learning was performed for patient clustering independent of anti-ARS antibodies. Transcriptome sequencing was conducted in clustered ASS patients and healthy controls.ResultsPatients with four different anti-ARS antibody subtypes demonstrated no significant differences in the incidence of rapidly progressive interstitial lung disease (RP-ILD) or prognoses. Unsupervised machine learning, independent of anti-ARS specificity, identified three endotypes with distinct clinical features and outcomes. Endotype 1 (RP-ILD cluster, 23.7%) was characterised by a high incidence of RP-ILD and a high mortality rate. Endotype 2 (dermatomyositis (DM)-like cluster, 14.5%) corresponded to patients with DM-like skin and muscle symptoms with an intermediate prognosis. Endotype 3 (arthritis cluster, 61.8%) was characterised by arthritis and mechanic’s hands, with a good prognosis. Transcriptome sequencing revealed that the different endotypes had distinct gene signatures and biological processes.ConclusionsAnti-ARS antibodies were not significant in stratifying ASS patients into subgroups with greater homogeneity in RP-ILD and prognoses. Novel ASS endotypes were identified independent of anti-ARS specificity and differed in clinical outcomes and transcriptional signatures, providing new insights into the pathogenesis of ASS.

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Analysis of the clinical features of antisynthetase syndrome: a retrospective cohort study in China

Cited by 12 publications

References 20 publications

Predictive factors for progressive fibrosing interstitial lung disease in anti‐synthetase syndrome

Predictive factors for progressive fibrosing interstitial lung disease in anti‐synthetase syndrome

Review of Pulmonary Manifestations in Antisynthetase Syndrome

Novel endotypes of antisynthetase syndrome identified independent of anti-aminoacyl transfer RNA synthetase antibody specificity that improve prognostic stratification

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