Clinical impact of myositis-specific autoantibodies on long-term prognosis of juvenile idiopathic inflammatory myopathies: multicentre study

Rheumatology ◽  
2021 ◽  
Author(s):  
Yuichi Yamasaki ◽  
Norimoto Kobayashi ◽  
Shinji Akioka ◽  
Kazuko Yamazaki ◽  
Shunichiro Takezaki ◽  
...  
Keyword(s):  
Matrix Protein ◽  
Multiple Drug ◽  
Idiopathic Inflammatory Myopathies ◽  
Inflammatory Myopathies ◽  
Gamma Subunits ◽  
Specific Autoantibody ◽  
Long Term Prognosis ◽  
Using Data ◽  
Drug Free

Abstract Objectives This study aimed to investigate the clinical characteristics, treatment and prognosis of juvenile idiopathic inflammatory myopathies (JIIM) in Japan for each myositis-specific autoantibody (MSA) profile. Methods A multicentre, retrospective study was conducted using data of patients with JIIM at nine paediatric rheumatology centres in Japan. Patients with MSA profiles, determined by immunoprecipitation using stored serum from the active stage, were included. Results MSA were detected in 85 of 96 cases eligible for the analyses. Over 90% of the patients in this study had one of the following three MSA types: anti-melanoma differentiation-associated protein 5 (MDA5) (n = 31), anti-transcriptional intermediary factor 1 alpha and/or gamma subunits (TIF1γ) (n = 25) and anti-nuclear matrix protein 2 (NXP2) (n = 25) antibodies. Gottron papules and periungual capillary abnormalities were the most common signs of every MSA group in the initial phase. The presence of interstitial lung disease (ILD) was the highest risk factor for patients with anti-MDA5 antibodies. Most patients were administered multiple drug therapies: glucocorticoids and MTX were administered to patients with anti-TIF1γ or anti-NXP2 antibodies. Half of the patients with anti-MDA5 antibodies received more than three medications including i.v. CYC, especially patients with ILD. Patients with anti-MDA5 antibodies were more likely to achieve drug-free remission (29 vs 21%) and less likely to relapse (26 vs 44%) than others. Conclusion Anti-MDA5 antibodies are the most common MSA type in Japan, and patients with this antibody are characterized by ILD at onset, multiple medications including i.v. CYC, drug-free remission, and a lower frequency of relapse. New therapeutic strategies are required for other MSA types.

Abstract 17315: Low Occurrence of Left Ventricular Reverse Remodeling and Poor Long-term Prognosis in Dilated Cardiomyopathy with Higher Immunohistological Expression of Myocardial Tenascin-C

Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Tetsuro Yokokawa ◽  
Yasuo Sugano ◽  
Takafumi Nakayama ◽  
Masao Takigami ◽  
Toshiyuki Nagai ◽  
...  
Keyword(s):  
Dilated Cardiomyopathy ◽  
Matrix Protein ◽  
Left Ventricular ◽  
Extracellular Matrix Protein ◽  
Reverse Remodeling ◽  
Tenascin C ◽  
Left Ventricular Reverse Remodeling ◽  
Lv Remodeling ◽  
Long Term Prognosis

Background: Tenascin-C (TN-C) is an extracellular matrix protein, and may regulate matrix organization during tissue remodeling. Although serum TN-C is reported as a prognostic biomarker in patients with dilated cardiomyopathy (DCM), the clinical significance of myocardial expression of TN-C remains undetermined. The objective of this study is to clarify the significance of myocardial TN-C expression on left ventricular (LV) remodeling and long-term prognosis in patients with DCM. Methods: Eighty consecutive patients in 2005 and 2006, who were diagnosed with DCM by excluding ischemic cardiomyopathy and secondary cardiomyopathy by coronary angiography and right ventricular endomyocardial biopsy, were analyzed. Those patients were followed up to 73±34 months, and their clinical data were obtained. Immunohistochemistry for TN-C was performed on stored biopsy specimens to examine the association of TN-C deposition with the occurrence of LV reverse remodeling as well as long-term mortality. Immunostained area of myocardial TN-C was measured densitometrically and calculated in percent by the fraction of TN-C stained area to the whole myocardium (TN-C area). LV reverse remodeling was defined as LV end-diastolic dimension ≤55 mm and fractional shortening ≥25% by 60 months after diagnosis. Results: TN-C area was 1.2±1.6% on average. Twenty-two patients (28%) underwent LV reverse remodeling. Patients with LV reverse remodeling showed less TN-C area at diagnosis than those without (TN-C area; 0.6±0.5 vs. 1.5±1.8%, p=0.021). Patients were divided into two groups according to the extent of TN-C area; high TN-C group (TN-C area≥1.5%, n=19) and low TN-C group (TN-C area<1.5%, n=61). LV reverse remodeling occurred less frequently in high TN-C group compared with low TN-C group (5% vs 33%, p=0.01). Eleven patients (14%) died during the observation period. Kaplan-Meier analysis revealed high TN-C group had worse prognosis than low TN-C group (p=0.009). Conclusions: Patients with higher immunohistological expression of myocardial TN-C in DCM were characterized with lower occurrence of later LV reverse remodeling and poorer long-term prognosis. Myocardial TN-C may have some crucial role in LV remodeling process in patients with DCM.

scholarly journals Anti-nuclear matrix protein 2 antibody-positive idiopathic inflammatory myopathies represent extensive myositis without dermatomyositis-specific rash

2021 ◽  
Author(s):  
Yuki Ichimura ◽  
Miwako Shobo ◽  
Sae Inoue ◽  
Mari Okune ◽  
Akemi Maeda ◽  
...  
Keyword(s):  
Nuclear Matrix ◽  
Matrix Protein ◽  
Japanese Patients ◽  
Adult Patients ◽  
Idiopathic Inflammatory Myopathies ◽  
Inflammatory Myopathies ◽  
Standardized Incidence Ratio ◽  
Nuclear Matrix Protein ◽  
Skin Manifestations ◽  
Subcutaneous Edema

AbstractObjectiveMyositis-specific autoantibodies (MSAs) define distinct clinical subsets of idiopathic inflammatory myopathies (IIMs). The anti-nuclear matrix protein 2 (NXP2) antibody, a MSA detected in juvenile/adult IIMs, has been reported to be associated with a high risk of subcutaneous calcinosis, subcutaneous edema, and internal malignancies. The study aimed to clarify the clinical features of anti-NXP2 antibody-positive IIMs in detail.MethodsThis multi-center retrospective observational study on 76 anti-NXP2 antibody-positive patients. The antibody was detected via a serological assay using immunoprecipitation and western blotting. The patients were selected from 162 consecutive Japanese patients with IIMs.ResultsThe cohort of anti-NXP2 antibody-positive IIMs included 29 juvenile patients and 47 adult patients. Twenty-seven (35.5%) patients presented with polymyositis phenotype without dermatomyositis-specific skin manifestations (heliotrope rash and Gottron sign/papules); this was more common in the adults than children (48.9% vs. 15.8%, P < 0.01). Nine (11.8%) patients had subcutaneous calcinosis, and 20 (26.3%) patients had subcutaneous edema. In addition, the proportion of patients with muscle weakness extending to the distal limbs was high (36 patients [47.4%]) in this cohort. Adult patients had a higher prevalence of malignancy than the general population (age-standardized incidence ratio of malignancies: 22.4).ConclusionAnti-NXP2 antibody-positive IIMs, which include dermatomyositis sine dermatitis, are characterized by atypical skin manifestations and extensive muscular involvement.

scholarly journals OP0136 THE INFLUENCE OF LONG-TERM EXERCISE AND IN VITRO EXERCISE-MIMICKING STIMULATION ON THE PRODUCTION OF MYOKINES AND CYTOKINES IN MYOTUBES OF PATIENTS WITH CHRONIC IDIOPATHIC INFLAMMATORY MYOPATHIES

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 88.2-88
Author(s):  
M. Vokurková ◽  
L. Vernerová ◽  
M. Špiritović ◽  
H. Štorkánová ◽  
S. Oreska ◽  
...  
Keyword(s):  
Gene Expression ◽  
Inflammatory Cytokines ◽  
Culture Medium ◽  
Muscle Cells ◽  
Activin A ◽  
Idiopathic Inflammatory Myopathies ◽  
Healthy Controls ◽  
Inflammatory Myopathies

Background:It has been demonstrated several times that endurance exercise has beneficial effects on the condition of patients with idiopathic inflammatory myopathies (IIM). Muscle contraction during exercise is a major stimulus for the release of myokines that are supposed to take part in the beneficial adaption to exercise.Objectives:The aim of this study was to find out how a six-month physiotherapy andin vitroexercise-mimicking treatment affect myokine and cytokine production in myotubes of IIM patients.Methods:Seven patients with chronic IIM took part in a six-month physiotherapy (stretching and strengthening), which significantly improved their muscle strength and endurance. IIM patients (n=7) before and after the six months exercise and their respective healthy counterparts (HC, n=9) underwent amusculus vastus lateralisbiopsy. Isolated skeletal muscle cells were grown, differentiated into myotubes, which were treated with a pharmacological cocktail: palmitate, forskolin and ionomycin (PFI) to mimic exercise-stimulated contractions in vitro. Myokine and cytokine concentrations produced by myotubes to the culture medium were analyzed with ELISA and the multiplex immunoassay, respectively. RT-PCR was used for the evaluation of myokine gene expression in the cultured myotubes.Results:Compared to myotubes of healthy controls, myotubes of IIM patient released more myostatin and activin A into the medium. The myostatin gene was expressed significantly more in muscle cells of patients than in healthy controls’ cells (p<0.05). After a six-month rehabilitation program, activin A secretion was four-fold reduced in myotubes of patients with IIM, while myostatin release and gene expression remained unchanged. In myotubes of IIM patients, less follistatin and more follistatin like 3 were detected in the culture medium compared to HC myotubes. Myotubes derived from IIM patients after six months of rehabilitation secreted twice as much follistatin and half the amount of follistatin like 3 into the medium than myotubes derived from IIM patients prior to rehabilitation (p<0.05). There was no difference in secretion of interleukin (IL) 6, IL-17, tumor necrosis factor (TNF) and vascular endothelial growth factor (VEGF) between myotubes of IIM patients and myotubes of HC. However, six-month exercise significantly (p<0.05) reduced release of IL-6, TNF and VEGF in myotubes of IIM patients. Contrary to our expectation, stimulation of PFI had no effect on the release of myostatin, activin A, follistatin and follistatin like 3, or the expression of their genes. PFI treatment significantly (p<0.05) increased IL-6 secretion in myotubes from HC and IIM patients prior to six months of rehabilitation. On the other hand, it was observed that myotubes of HC and IIM patients exposed to the PFI cocktail secreted significantly less inflammatory cytokines IL-17, TNF and VEGF into the medium compared to unstimulated myotubes (p<0.05).Conclusion:In conclusion, long-term exercise influenced the production of myokines and decreased release of inflammatory cytokines in myotubes of IIM patients.In vitroexercise-mimicking treatment increased the secretion of IL-6 and decreased the release of inflammatory cytokines as IL-17, TNF-α and VEGF in myotubes of patients with IIM and healthy individuals.Acknowledgments:This work was supported by the Ministry of Health of the Czech Republic grants nr. 16-33746A and donation 140.0000008.Disclosure of Interests:None declared

Long‐term follow‐up of 76 Iranian patients with idiopathic inflammatory myopathies

2018 ◽  
Vol 21 (8) ◽  
pp. 1627-1633 ◽  
Author(s):  
Mehrzad Hajialilo ◽  
Amir Ghorbanihaghjo ◽  
Alireza Khabbazi ◽  
Sousan Kolahi ◽  
Mohammad Reza Jafari Nakhjavani ◽  
...  
Keyword(s):  
Idiopathic Inflammatory Myopathies ◽  
Inflammatory Myopathies ◽  
Long Term Follow Up ◽  
Iranian Patients

scholarly journals Long-Term Survival of Patients With Idiopathic Inflammatory Myopathies According to Clinical Features

Medicine ◽  
2004 ◽  
Vol 83 (1) ◽  
pp. 35-42 ◽  
Author(s):  
Katalin Dankó ◽  
Andrea Ponyi ◽  
Tamás Constantin ◽  
Gábor Borgulya ◽  
Gyula Szegedi
Keyword(s):  
Clinical Features ◽  
Idiopathic Inflammatory Myopathies ◽  
Inflammatory Myopathies ◽  
Term Survival ◽  
Long Term Survival
Sign in / Sign up

Export Citation Format

Share Document