Clinical Profiles and Prognosis of Patients with Distinct Antisynthetase Autoantibodies

Shi, Jingli; Li, Shanshan; Yang, Hanbo; Zhang, Yamei; Peng, Qinglin; Lü, Xin; Wang, Guochun

doi:10.3899/jrheum.161480

Cited by 145 publications

(175 citation statements)

References 39 publications

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“…Our study could not discover a link between individual anti-ARS antibodies and the long-term clinical course of ILD, in contrast to previous reports on an association between worse prognosis and anti-PL-7 antibody [14, 21]. This is because the lower frequency of PM/DM and the higher frequency of IPAF in our cohort may have led to results different from those of previous studies.…”

Section: Discussioncontrasting

confidence: 56%

Predictive Factors for the Long-Term Deterioration of Pulmonary Function in Interstitial Lung Disease Associated with Anti-Aminoacyl-tRNA Synthetase Antibodies

et al. 2018

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Background: Little has been reported on long-term pulmonary function trends among patients with interstitial lung disease associated with anti-aminoacyl-tRNA synthetase antibodies (ARS-ILD). Objectives: To clarify the factors predictive of progression in ARS-ILD based on patients’ initial clinical and radiological features. Methods: The clinical courses of 88 patients with > 1 year of follow-up data on pulmonary function tests (PFTs) were retrospectively analyzed. Disease behavior was categorized into three groups: (1) improved or (2) worsened (defined as increases or decreases, respectively, of > 10% in forced vital capacity and > 15% in %diffusing capacity of lung carbon monoxide) or (3) stable based on PFT changes compared between 1-year results as the initial data and results at 3 years to assess the long-term course. Results: In the initial course of 75 patients with ARS-ILD who received anti-inflammatory therapy within 6 months after diagnosis, 48 patients (64.0%) improved and 6 patients (8.0%) worsened. The radiological patterns in the patients with ARS-ILD included nonspecific interstitial pneumonia (NSIP) in 46.7% and NSIP with organizing pneumonia overlap in 52.0% of the cases. One-third of the initially improved patients who worsened over the long-term course were assigned to the unstable group. By multivariate logistic analysis, middle lobe traction bronchiectasis was a significant predictive factor for the patients in the unstable group. Conclusions: Most patients with ARS-ILD receiving anti-inflammatory therapy had improved or remained stable in the first year. However, over the long-term course, some patients worsened despite their initial improvement. Even though the extent of the disease is limited, middle lobe traction bronchiectasis in ARS-ILD may be a useful predictor of poor long-term disease behavior.

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Section: Discussioncontrasting

confidence: 56%

Predictive Factors for the Long-Term Deterioration of Pulmonary Function in Interstitial Lung Disease Associated with Anti-Aminoacyl-tRNA Synthetase Antibodies

et al. 2018

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“…Meanwhile, patients with anti-PL-7, anti-EJ, and anti-Jo-1 autoantibodies would develop myositis later if they only showed ILD at the time of onset. Compared with patients without anti-PL-7, Chinese ASSD patients with anti-PL-7 were more likely to develop rapidly progressive ILD and their survival rate decreased more rapidly at the early stage of long-term follow-up 70 . Furthermore, by analyzing the log-rank test and Cox proportional hazards ratio, researchers demonstrated that non-Jo-1 autoantibody patients had worse survival compared with Jo-1-positive patients 71 .…”

Section: Arss and Autoimmune Diseasesmentioning

confidence: 91%

“…Autoimmune diseases ARSs served as autoantigens in ASSD patients Anti-ARS autoantibody specificity was related to the clinical features, disease severity, and even survival of ASSD patients - [65][66][67][68][69][70][71] HisRS and AsnRS…”

Section: Referencesmentioning

confidence: 99%

Roles of aminoacyl-tRNA synthetases in immune regulation and immune diseases

Nie

Sun

et al. 2019

Cell Death Dis

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Aminoacyl-tRNA synthetases (ARSs) play a vital role in protein synthesis by linking amino acids to their cognate transfer RNAs (tRNAs). This typical function has been well recognized over the past few decades. However, accumulating evidence reveals that ARSs are involved in a wide range of physiological and pathological processes apart from translation. Strikingly, certain ARSs are closely related to different types of immune responses. In this review, we address the infection and immune responses induced by pathogen ARSs, as well as the potential anti-infective compounds that target pathogen ARSs. Meanwhile, we describe the functional mechanisms of ARSs in the development of immune cells. In addition, we focus on the roles of ARSs in certain immune diseases, such as autoimmune diseases, infectious diseases, and tumor immunity. Although our knowledge of ARSs in the immunological context is still in its infancy, research in this field may provide new ideas for the treatment of immune-related diseases.

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“…Though antisynthetase syndrome was not previously considered to be associated with an increased risk of malignancy, a retrospective review of 124 patients with antisynthetase syndrome recently showed a malignancy risk of 6.5%. 4 Overall, the data regarding the association of malignancy and antisynthetase syndrome are confl icting, and this needs further study. Therefore, an age-appropriate malignancy screen is recommended.…”

Section: Association With Cancer Riskmentioning

confidence: 99%

Hyperkeratotic fissured plaques on both hands: Mechanic’s hands

Bishnoi

Parsad

2018

CCJM

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A housewife in her 50s presented with photosensitivity and recent-onset roughness on her index fi ngers and thumbs. Examination showed asymptomatic, bilaterally symmetrical, hyperkeratotic, scaly, fi ssured plaques with hyperpigmentation on the radial aspect of the index fi nger of both hands, extending to the ulnar aspect of the thumbs and sparing the palmar aspect of both (Figure 1). Palms, soles, and other fi ngers were normal.Nail fold capillaroscopy did not reveal telangiectasia or ragged cuticles. Further examination of the skin showed confl uent macular violaceous erythema on the eyelids (suggestive of the heliotrope sign), V area of the neck, upper arms, and back.She also had a low-grade intermittent fever for the past 2 months, as well as difficulty in getting up from a squatting position and combing her hair, dyspnea on exertion, blue discoloration of the fi ngers on exposure to cold, and intermittent pain, stiffness, and swelling in the small joints of both hands that was worse in the morning and seemed to be relieved by activity. She had no history of dysphagia or nasal regurgitation of food. Strength against resistance was reduced in both arms and knee extensors. A diagnosis of dermatomyositis with "mechanic's hands" was considered.Laboratory testing was negative for antinuclear antibodies and showed elevated creatine kinase and positive anti-Jo-1 antibodies. High-resolution computed tomography of the chest showed evidence of interstitial lung disease. Features were consistent with antisynthetase syndrome and dermatomyositis. An age-appropriate malignancy screen was normal. THE CLINICAL PICTURE

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Clinical Profiles and Prognosis of Patients with Distinct Antisynthetase Autoantibodies

Abstract: Our study investigates the clinical phenotypes and outcomes of patients with antisynthetase syndrome with distinct antisynthetase antibodies and highlights the link between the anti-PL7 antibody and RP-ILD.

Cited by 145 publications

References 39 publications

Predictive Factors for the Long-Term Deterioration of Pulmonary Function in Interstitial Lung Disease Associated with Anti-Aminoacyl-tRNA Synthetase Antibodies

Predictive Factors for the Long-Term Deterioration of Pulmonary Function in Interstitial Lung Disease Associated with Anti-Aminoacyl-tRNA Synthetase Antibodies

Roles of aminoacyl-tRNA synthetases in immune regulation and immune diseases

Hyperkeratotic fissured plaques on both hands: Mechanic’s hands

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