National registry of patients with juvenile idiopathic inflammatory myopathies in Hungary—Clinical characteristics and disease course of 44 patients with juvenile dermatomyositis

Autoimmunity ◽  
2006 ◽  
Vol 39 (3) ◽  
pp. 223-232 ◽  
Author(s):  
T. Constantin ◽  
T. Constantin ◽  
A. Ponyi ◽  
T. Constantin ◽  
A. Ponyi ◽  
...  
Keyword(s):  
Clinical Characteristics ◽  
Juvenile Dermatomyositis ◽  
National Registry ◽  
Idiopathic Inflammatory Myopathies ◽  
Disease Course ◽  
Inflammatory Myopathies

Juvenile dermatomyositis and other idiopathic inflammatory myopathies of childhood

The Lancet ◽  
2008 ◽  
Vol 371 (9631) ◽  
pp. 2201-2212 ◽  
Author(s):  
Brian M Feldman ◽  
Lisa G Rider ◽  
Ann M Reed ◽  
Lauren M Pachman
Keyword(s):  
Juvenile Dermatomyositis ◽  
Idiopathic Inflammatory Myopathies ◽  
Inflammatory Myopathies

A25: The Association of Immunogenetic and Environmental Factors with Disease Course in Patients with Juvenile Idiopathic Inflammatory Myopathies

2014 ◽  
Vol 66 ◽  
pp. S39-S40 ◽  
Author(s):  
G. Esther A. Habers ◽  
Adam M. Huber ◽  
Gulnara Mamyrova ◽  
Terrance P. O'Hanlon ◽  
Sharon Adams ◽  
...  
Keyword(s):  
Environmental Factors ◽  
Idiopathic Inflammatory Myopathies ◽  
Disease Course ◽  
Inflammatory Myopathies

scholarly journals Anti-NXP2 autoantibodies in adult patients with idiopathic inflammatory myopathies: possible association with malignancy

2012 ◽  
Vol 71 (5) ◽  
pp. 710-713 ◽  
Author(s):  
Yuki Ichimura ◽  
Takashi Matsushita ◽  
Yasuhito Hamaguchi ◽  
Kenzo Kaji ◽  
Minoru Hasegawa ◽  
...  
Keyword(s):  
Lupus Erythematosus ◽  
Juvenile Dermatomyositis ◽  
Metastatic Cancer ◽  
Japanese Patients ◽  
Adult Patients ◽  
Idiopathic Inflammatory Myopathies ◽  
Inflammatory Myopathies ◽  
Systemic Lupus ◽  
Serum Samples ◽  
Clinical Associations

ObjectivesMyositis-specific autoantibodies (MSAs) are useful tools for identifying clinically homogeneous subsets and predicting prognosis of patients with idiopathic inflammatory myopathies (IIM) including polymyositis (PM) and dermatomyositis (DM). Recent studies have shown that anti-NXP2 antibody (Ab) is a major MSA in juvenile dermatomyositis (JDM). In this study the frequencies and clinical associations of anti-NXP2 Ab were evaluated in adult patients with IIM.MethodsClinical data and serum samples were collected from 507 adult Japanese patients with IIM (445 with DM and 62 with PM). Eleven patients with JDM, 108 with systemic lupus erythematosus, 433 with systemic sclerosis and 124 with idiopathic pulmonary fibrosis were assessed as disease controls. Serum was examined for anti-NXP2 Ab by immunoprecipitation and western blotting using polyclonal anti-NXP2 Ab.ResultsSeven patients (1.6%) with adult DM and one (1.6%) with adult PM were positive for anti-NXP2 Ab. Except for two patients with JDM, none of the disease controls were positive for this autoantibody. Among eight adult patients with IIM, three had internal malignancies within 3 years of diagnosis of IIM. Another patient with DM also had a metastatic cancer at the diagnosis. All of the carcinomas were at an advanced stage (stage IIIb–IV).ConclusionsWhile less common than in juvenile IIM, anti-NXP2 Ab was found in adult IIM. Anti-NXP2 Ab may be associated with adult IIM with malignancy.

scholarly journals Anti-melanoma-associated Antigen A1 Autoantibodies Predict the Outcome of Patients With Idiopathic Inflammatory Myopathies Related Interstitial Lung Disease

2020 ◽  
Author(s):  
Yawen Shen ◽  
Wei Jiang ◽  
Hanbo Yang ◽  
He Chen ◽  
Qinglin Peng ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Lupus Erythematosus ◽  
Estimating Equation ◽  
Good Prognosis ◽  
Trna Synthetase ◽  
Fisher Exact Test ◽  
Idiopathic Inflammatory Myopathies ◽  
Disease Course ◽  
Inflammatory Myopathies

Abstract Background The discovery of novel autoantibody in patients with idiopathic inflammatory myopathies (IIMs) is of great significance for clinical subtype stratification and potential pathogenesis. Previous literatures have found that tumor-associated antigens were involved in the pathogenesis of IIM. Therefore, in this study, we aimed to explore the prevalence and clinical association of autoantibodies against melanoma-associated antigen A1 (MAGE-A1) in patients with IIM. Methods ELISA was performed to detect anti-MAGE-A1 autoantibodies in patients with IIM, systemic lupus erythematosus, rheumatoid arthritis, primary Sjögren syndrome, systemic sclerosis, and healthy controls, and the results were confirmed using the dot-immunoblotting assay. The association between anti-MAGE-A1 autoantibody and clinical characteristics was analyzed in IIM patients. T test, Mann-Whitney U test, double-sided Pearson's Chi-square, Fisher exact test, Spearman correlation analysis as well as the generalized estimating equation were applied in the statistical analyses. Results Anti-MAGE-A1 autoantibodies were detected in 5.03% (29/576) of all IIM patients, 4.60% (18/390) of dermatomyositis, 7.20% (5/69) of amyopathic dermatomyositis, and 5.10% (6/117) of polymyositis/immune-mediated necrotizing myopathy patients. The frequency of interstitial lung disease (ILD) was higher in anti-MAGE-A1-positive patients than those in anti-MAGE-A1-negative ones (82.8% vs 55%, p = 0.003). Anti-MAGE-A1-positive patients with IIM associated ILD had less dyspnea, more commonly asymptomatic ILD, and higher percentage of force vital capacity and diffusing lung capacity of carbon monoxcide of predicted values when compared to negative ones. The anti-aminoacyl-tRNA synthetase (ARS) coexisting anti-MAGE-A1-positive patients had common monocyclic disease course, while only anti-ARS-positive and anti-ARS coexisting anti-Ro52-positive patients had a more polycyclic disease course. None of anti-ARS-positive patients coexisting anti-MAGE-A1 antibody died during 10-year follow-up evaluation. Conclusion Anti-MAGE-A1 is a novel autoantibody associated with ILD in IIM patients. Anti-MAGE-A1-positive IIM patients with ILD appear to have good prognosis. Detection of anti-MAGE-A1 autoantibody may be useful in predicting the outcome of IIM patients with ILD.

scholarly journals Anti-Ro52 autoantibodies are associated with interstitial lung disease and more severe disease in patients with juvenile myositis

2019 ◽  
Vol 78 (7) ◽  
pp. 988-995 ◽  
Author(s):  
Sara Sabbagh ◽  
Iago Pinal-Fernandez ◽  
Takayuki Kishi ◽  
Ira N Targoff ◽  
Frederick W Miller ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Disease ◽  
Connective Tissue Disease ◽  
Clinical Features ◽  
Clinical Characteristics ◽  
Juvenile Dermatomyositis ◽  
Severe Disease ◽  
Trna Synthetase ◽  
Disease Course

ObjectivesAnti-Ro52 autoantibodies are associated with more severe interstitial lung disease (ILD) in adult myositis patients with antiaminoacyl transfer (t)RNA synthetase autoantibodies. However, few studies have examined anti-Ro52 autoantibodies in juvenile myositis. The purpose of this study was to define the prevalence and clinical features associated with anti-Ro52 autoantibodies in a large cohort of patients with juvenile myositis.MethodsWe screened sera from 302 patients with juvenile dermatomyositis (JDM), 25 patients with juvenile polymyositis (JPM) and 44 patients with juvenile connective tissue disease–myositis overlap (JCTM) for anti-Ro52 autoantibodies by ELISA. Clinical characteristics were compared between myositis patients with and without anti-Ro52 autoantibodies.ResultsAnti-Ro52 autoantibodies were found in 14% patients with JDM, 12% with JPM and 18% with JCTM. Anti-Ro52 autoantibodies were more frequent in patients with antiaminoacyl tRNA synthetase (64%, p<0.001) and anti-MDA5 (31%, p<0.05) autoantibodies. After controlling for the presence of myositis-specific autoantibodies, anti-Ro52 autoantibodies were associated with the presence of ILD (36% vs 4%, p<0.001). Disease course was more frequently chronic, remission was less common, and an increased number of medications was received in anti-Ro52 positive patients.ConclusionsAnti-Ro52 autoantibodies are present in 14% of patients with juvenile myositis and are strongly associated with anti-MDA5 and antiaminoacyl tRNA synthetase autoantibodies. In all patients with juvenile myositis, those with anti-Ro52 autoantibodies were more likely to have ILD. Furthermore, patients with anti-Ro52 autoantibodies have more severe disease and a poorer prognosis.

Antisynthetase syndrome: A distinct disease spectrum

2020 ◽  
Vol 5 (3) ◽  
pp. 178-191
Author(s):  
Kun Huang ◽  
Rohit Aggarwal
Keyword(s):  
Clinical Characteristics ◽  
Trna Synthetase ◽  
Idiopathic Inflammatory Myopathies ◽  
Inflammatory Myopathies ◽  
Antisynthetase Syndrome ◽  
Aminoacyl Trna Synthetase ◽  
Trna Synthetases ◽  
Disease Spectrum ◽  
Aminoacyl Trna Synthetases ◽  
Distinct Disease

The discovery of novel autoantibodies related to idiopathic inflammatory myopathies (collectively referred to as myositis) has not only advanced our understanding of the clinical, serological, and pathological correlation in the disease spectrum but also played a role in guiding management and prognosis. One group of the myositis-specific autoantibodies is anti-aminoacyl-tRNA synthetase (anti-ARS or anti-synthetase) which defines a syndrome with predominant interstitial lung disease, arthritis, and myositis. Autoantibodies to eight aminoacyl-tRNA synthetases have been identified with anti-Jo1 the most common in all of idiopathic inflammatory myopathies. Disease presentation and prognosis vary depending on which anti-aminoacyl-tRNA synthetase antibody is present. In this review, we will discuss the clinical characteristics, overlap features with other autoimmune diseases, prognostic factors, and management of the antisynthetase syndrome.

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