Pulmonary pathologic manifestations of anti-glycyl-tRNA synthetase (anti-EJ)-related inflammatory myopathy

Schneider, Frank; Yousem, Samuel A.; Bi, David; Gibson, Kevin F.; Oddis, Chester V.; Aggarwal, Rohit

doi:10.1136/jclinpath-2014-202367

Cited by 32 publications

(18 citation statements)

References 23 publications

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“…In most patients with EJ-ILD clinical disease behavior was reversible or progressive and irreversible disease. Although anti-EJ antibodies correlated well with ILD, there are a few reports which describe the clinical, radiological and pathological findings of EJ-ILD and no reports describe any detailed clinical courses [16, 26]. To our knowledge, this is the first study to describe the detailed clinical features of biopsy-proven EJ-ILD with long-term follow-up period.…”

Section: Discussionmentioning

confidence: 92%

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Long-term clinical course of anti-glycyl tRNA synthetase (anti-EJ) antibody-related interstitial lung disease pathologically proven by surgical lung biopsy

et al. 2016

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BackgroundAnti-glycyl-tRNA synthetase (anti-EJ) antibody is occasionally positive in patients with interstitial lung disease (ILD). We aimed to define the clinical, radiological and pathological features of patients with anti-EJ antibody-positive ILD (EJ-ILD).MethodsWe retrospectively analyzed the medical records of 12 consecutive patients with EJ-ILD who underwent surgical lung biopsy.ResultsThe median follow-up time was 74 months (range, 17–115 months). The median age was 62 years (range, 47–75 years). Seven of 12 patients were female. Eight patients presented with acute onset. Six patients eventually developed polymyositis/dermatomyositis. On high-resolution computed tomography, consolidation and volume loss were predominantly observed in the middle or lower lung zone. Nine patients presented pathologically nonspecific interstitial pneumonia with organizing pneumonia, alveolar epithelial injury and prominent interstitial cellular infiltrations whereas the other three patients were diagnosed with unclassifiable interstitial pneumonia. Although all patients but one improved with the initial immunosuppressive therapy, five patients relapsed. When ILD relapsed, four of the five patients were treated with corticosteroid monotherapy. Four of the six patients without relapse have been continuously treated with combination therapy of corticosteroid and immunosuppressant.ConclusionsPatients with EJ-ILD often had acute onset of ILD with lower lung-predominant shadows and pathologically nonspecific interstitial pneumonia or unclassifiable interstitial pneumonia with acute inflammatory findings. Although the disease responded well to the initial treatment, relapse was frequent. Because of the diversity of the clinical courses, combination therapy of corticosteroid and immunosuppressant should be on the list of options to prevent relapse of EJ-ILD.

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Section: Discussionmentioning

confidence: 92%

“…However, there are only a few reports describing the clinical features of patients with ILD with positive-anti-EJ antibody (EJ-ILD) [15, 16]. The aim of this retrospective study was to define the clinical, radiological and pathological features of patients with EJ-ILD with long-term follow-up.…”

Section: Introductionmentioning

confidence: 99%

Long-term clinical course of anti-glycyl tRNA synthetase (anti-EJ) antibody-related interstitial lung disease pathologically proven by surgical lung biopsy

et al. 2016

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“…It is possible that the prevalence of the non-Jo1 ARS group is higher in different cohorts such as patients labelled as 'idiopathic' IP, particularly in those with UIP and AIP (DAD) subtypes [27]. Data from several studies suggest that UIP and AIP are commonly seen in non-Jo1 ASS, in particular anti-EJ, anti-PL7 and anti-PL12 patients, where lung disease presents early in disease course and is the predominant manifestation [22,[27][28][29][30][31][32]. In a large series of Japanese patients positive for anti-Jo-1, anti-EJ, anti-PL-7, anti-PL-12, anti-KS or anti-OJ highlighted the degree of heterogeneity within ASS in terms of distribution and onset of myositis, lung disease and skin lesions [33].…”

Section: Anti-synthetase Syndromementioning

confidence: 99%

The Clinical Features of Myositis-Associated Autoantibodies: a Review

Gunawardena

2015

Clinic Rev Allerg Immunol

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The idiopathic inflammatory myopathies (IIM) are a group of autoimmune diseases traditionally defined by clinical manifestations including skeletal muscle weakness, skin rashes, elevated skeletal muscle enzymes, and neurophysiological and/or histological evidence of muscle inflammation. Patients with myositis overlap can develop other features including parenchymal lung disease, inflammatory arthritis, gastrointestinal manifestations and marked constitutional symptoms. Although patients may be diagnosed as having polymyositis (PM) or dermatomyositis (DM) under the IIM spectrum, it is quite clear that disease course between subgroups of patients is different. For example, interstitial lung disease may predominate in some, whereas cutaneous complications, cancer risk, or severe refractory myopathy may be a significant feature in others. Therefore, tools that facilitate diagnosis and indicate which patients require more detailed investigation for disease complications are invaluable in clinical practice. The expanding field of autoantibodies (autoAbs) associated with connective tissue disease (CTD)-myositis overlap has generated considerable interest over the last few years. Using an immunological diagnostic approach, this group of heterogeneous conditions can be separated into a number of distinct clinical phenotypes. Rather than diagnose a patient as simply having PM, DM or overlap CTD, we can define syndromes to differentiate disease subsets that emphasise clinical outcomes and guide management. There are now over 15 CTD-myositis overlap autoAbs found in patients with a range of clinical manifestations including interstitial pneumonia, cutaneous disease, cancer-associated myositis and autoimmune-mediated necrotising myopathy. This review describes their diagnostic utility, potential role in disease monitoring and response to treatment. In the future, routine use of these autoAb will allow a stratified approach to managing this complex set of conditions.

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“…33,34 Overall, because the other anti-ARS antibodies make up less than 2% of patients with the antisynthetase syndrome, little is known about their specific phenotypes; however, smaller case series highlight the association of several of these less frequently observed anti-ARS antibodies with ILD. [35][36][37] Beyond these autoantibody specificities, anti-CADM-140/anti-MDA-5 antibodies are associated with a phenotype similar to the antisynthetase syndrome, with a high risk of rapidly progressive ILD. 38 Rarely found in other connective tissue disorders, anti-MDA-5 antibodies target interferon-induced helicase-1, a molecule that recognizes single-stranded RNA viruses as part of the innate immune response.…”

Section: Pulmonary Complications Of Inflammatory Myopathymentioning

confidence: 99%

“…5,52 Among patients with the antisynthetase syndrome, individual antibody specificity does not seem to correlate with histologic pattern, 21 although several studies have indicated a higher than expected prevalence of UIP and DAD compared with nonsynthetase subgroups. 33,35 Overall, these studies must be interpreted cautiously, because the estimated prevalence of histopathologic subtypes is influenced by specific serologically/clinically defined phenotypes as well as referral bias related to disease severity.…”

Section: Histopathologymentioning

confidence: 99%

Pulmonary Complications of Inflammatory Myopathy

Miller¹,

Glassberg²,

Ascherman³

2015

Rheumatic Disease Clinics of North America

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Pulmonary pathologic manifestations of anti-glycyl-tRNA synthetase (anti-EJ)-related inflammatory myopathy

Abstract: Identifying ARS-associated autoantibodies in ILD patients with or without myopathy is desirable because patients may respond well to immunosuppressive therapy, and their prognosis is better than that of patients with idiopathic forms of DAD or UIP.

Cited by 32 publications

References 23 publications

Long-term clinical course of anti-glycyl tRNA synthetase (anti-EJ) antibody-related interstitial lung disease pathologically proven by surgical lung biopsy

Long-term clinical course of anti-glycyl tRNA synthetase (anti-EJ) antibody-related interstitial lung disease pathologically proven by surgical lung biopsy

The Clinical Features of Myositis-Associated Autoantibodies: a Review

Pulmonary Complications of Inflammatory Myopathy

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