Clinical manifestations and long-term outcome of anti-Jo1 antisynthetase patients in a large cohort of Spanish patients from the GEAS-IIM group

Trallero-Araguás, Ernesto; Grau-Junyent, Josep María; Labirua-Iturburu, Anne; García–Hernández, Francisco J.; Monteagudo-Jiménez, Manuel; Fraile-Rodríguez, Guadalupe; Bujanda, Luís; Rodríguez-Carballeira, Mónica; Sáez‐Comet, Luis; Selva-O’Callaghan, Albert

doi:10.1016/j.semarthrit.2016.03.011

Cited by 100 publications

(80 citation statements)

References 32 publications

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“…To our knowledge, this is the first study to demonstrate that serum neopterin is an independent prognostic factor for DM. An increasing number of studies have been performed to identify prognostic factors in myositis and several biomarkers or clinical complications have been suggested to be associated with poor outcome, such as anti-MDA5 antibody [4], serum ferritin [25], malignancy [29] and ILD [3,30]. In line with these studies, we also confirmed anti-MDA5 antibody, malignancy and RP-ILD to be independent risk factors, based on our multivariate model.…”

Section: Discussionsupporting

confidence: 86%

A high level of serum neopterin is associated with rapidly progressive interstitial lung disease and reduced survival in dermatomyositis

Peng

Zhang

Liang

et al. 2019

Clinical and Experimental Immunology

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Summary Neopterin is primarily synthesized and released by activated macrophages/monocytes upon stimulation with interferon‐γ and is considered as a marker for macrophage activation. This study aimed to analyze the serum levels of neopterin in patients with dermatomyositis (DM) in association with clinical manifestations, laboratory data and patient prognosis. One hundred and eighty‐two consecutive DM patients and 30 healthy controls were retrospectively enrolled into the study. Serum levels of neopterin were significantly increased in DM patients compared to healthy controls (P < 0·001). High serum neopterin levels were associated with anti‐melanoma differentiation‐associated gene (MDA5) antibody, rapidly progressive interstitial lung disease (RP‐ILD) and characteristic DM cutaneous involvement. Longitudinal assessment of serum samples revealed that the serum neopterin levels were closely correlated with disease severity (β = 30·24, P < 0·001). In addition, a significant increase in serum neopterin concentration of non‐survivors was observed when compared to that of survivors (P < 0·001). Receiver operator characteristic curves showed that serum neopterin could distinguish non‐survivors and survivors at an optimal cut‐off level of 22·1 nmol/l with a sensitivity and specificity of 0·804 and 0·625, respectively (P < 0·001). Kaplan–Meier survival curves revealed that DM patients with serum neopterin > 22·1 nmol/l had a significantly higher mortality compared to the patient group with serum neopterin < 22·1 nmol/l (log‐rank P < 0·001). Multivariate regression analysis identified high serum neopterin concentration to be an independent risk factor for poor prognosis in DM (adjusted hazard ratio = 4·619, 95% confidence interval = 2·092–10·195, P < 0·001). In conclusion, increased serum levels of neopterin were significantly associated with RP‐ILD and reduced survival in DM patients, suggesting it as a promising biomarker in disease evaluation of DM.

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Section: Discussionsupporting

confidence: 86%

A high level of serum neopterin is associated with rapidly progressive interstitial lung disease and reduced survival in dermatomyositis

Peng

Zhang

Liang

et al. 2019

Clinical and Experimental Immunology

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“…Anti-Ro52, a myositis associated antibody, the positive frequency of which was reported as 65% in an anti-Jo1 positive ASSD cohort (31). In our cohort the anti-Ro52 positive rate was 87.9% in Jo-1 group, and 91.3% in EJ group, respectively, which were signi cantly higher than the other 3 groups.…”

Section: Discussioncontrasting

confidence: 43%

Clinical features of Anti-synthetase syndrome associated interstitial lung disease: a retrospective cohort in China

Zhan¹,

Yan

Wang

et al. 2020

Preprint

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Background Antisynthetase Syndrome (ASSD) is a chronic autoimmune condition characterized by antibodies directed against an aminoacycl transfer RNA synthetase (ARS) along with a group of clinical features including the classical clinical triad: inflammatory myopathy, arthritis, interstitial lung disease (ILD). ASSD is highly heterogenous due to the different organs involvement, and ILD is the main cause of mortality and function loss, which presents as different patterns when diagnosed. We designed this retrospective cohort to describe the clinical features and disease behavior of the ASSD) associated ILD. Methods Retrospectively collected the data of 108 cases of ASSD associated ILD in Beijing Chaoyang Hospital since 2017.12 to 2019.3. Data including age, gender, physical examination, laboratory test, pulmonary function and High Resolution Computer Tomography (HRCT), treatment, were obtained from the Electronic Medical Record (EMR) system. Patients were divided into 5 groups according to the distinct Aminoacyl tRNA synthetases (ARS) antibodies, and all patients had a multiple discussion team (MDT) to make a radiological and pathological diagnosis of the ILD pattern. Each patient had at least 1 follow up for no less than 6 months. Patients with missing data of MDT evaluation or follow up were excluded. Results: 108 consecutive patients were recruited in this retrospective cohort. 30 cases received bronchoscopy for a transbronchial lung biopsy. 3 had bronchoscopy for a transbronchial cryobiopsy. 33 were with JO-1 positive and 30 were with PL-7 positive. 23 with EJ positive, 13 with PL12 positive and 9 with OJ positive. JO-1 group had a significant higher rate of mechanic’s hand (57.6%) than other 4 groups, the skin involvement (Gottron Papules and/or Heliotrope Rash) was found in 38 (35%) and no difference was found among the 5 groups. Polymyositis/Dermatomyositis (PM/DM) was diagnosed in 25 (23.1%) patients and no difference was observed among the 5 groups. The anti-PL7 positive group had a higher frequency of UIP pattern (13.3%) than other 4 groups but the difference was not significant due to the small sample size, and EJ group had the most frequent OP pattern(78.2%), which was significantly higher than PL-7 group (P<0.001) and PL-12 group (P=0.025). The median follow-up time were 10.7 months. All received prednisone treatment, with or without immunosuppressants ,and at the 6-month-follow up, the JO-1 group and EJ group had the significantly higher improvement of forced vital capacity that the other 3 groups (P<0.05), and PL-7 group had the lowest FVC improvement (P<0.05). The anti-Jo1-positive group and anti-EJ-positive group had significantly higher anti-RO52-positive occurrence than other 3 groups (P<0.05).Conclusion Anti PL-7 antibody had the same frequency as anti-JO1 in ASSD-ILD, in which the ILD pattern was different with distinct anti-ARS antibodies. All ASSD-ILD responded to therapy of steroids, with or without immunosuppressants. PL-7 group had a highest occurrence of UIP pattern, and significantly lower respondence to therapy.

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“…Thus, the optimal definition of categorical decline in FVC for trial purposes may consist of either a ≥10% decline in the FVC or a 5-9% marginal decline in the FVC in association with a ≥15% decline in the DLCO for systemic sclerosis. However, such studies are lacking in IMrelated ILD (74).…”

Section: Evaluationsmentioning

confidence: 99%

Inflammatory Myopathy-Related Interstitial Lung Disease: From Pathophysiology to Treatment

Hervier

Uzunhan

2020

Front. Med.

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Inflammatory myopathies (IM) are auto-immune connective tissue diseases characterized by muscle involvement and by extramuscular manifestations. As such, pulmonary manifestations, which mainly include interstitial lung disease (ILD), often darken two out of four distinct IM, namely dermatomyositis and overlapping myositis. Being the initiation site of the disease and being the leading cause of morbidity and mortality, ILD is of major importance in this context. ILD has a heterogeneous expression among the patients, with various onset mode, various radiological pattern, various severity and finally with different prognoses, which are particularly difficult to predict at the time of IM diagnosis. Therefore, ILD is a challenging issue. Treatments are based on steroids and immunosuppressive or targeted therapies. Their respective place is yet poorly codified however and remains often based on clinician expertise. Dedicated clinical trials are lacking to date and are also difficult to build, due to difficulty of constituting large and homogeneous patient groups and to rigorously evaluate disease outcomes. Indeed, pulmonary function tests alone are being regularly defeated in IM, in which respiratory muscles are often involved. Composite scores, bringing together several lung parameters, should thus be developed and validated in the future, to better assess the disease response to treatment. This review aims to describe the current knowledge of IM immuno-pathogenesis, the clinical features associated with IM related-ILD, focusing of both severity and prognosis, and the actual therapeutic approaches.

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Clinical manifestations and long-term outcome of anti-Jo1 antisynthetase patients in a large cohort of Spanish patients from the GEAS-IIM group

Cited by 100 publications

References 32 publications

A high level of serum neopterin is associated with rapidly progressive interstitial lung disease and reduced survival in dermatomyositis

A high level of serum neopterin is associated with rapidly progressive interstitial lung disease and reduced survival in dermatomyositis

Clinical features of Anti-synthetase syndrome associated interstitial lung disease: a retrospective cohort in China

Inflammatory Myopathy-Related Interstitial Lung Disease: From Pathophysiology to Treatment

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