Serum muscle enzymes and standard inflammatory markers at time of diagnosis in idiopathic inflammatory myopathies – a retrospective analysis

2010 ◽  
Vol 41 (01) ◽  
Author(s):  
F Hanisch ◽  
T Müller ◽  
S Zierz
Keyword(s):  
Retrospective Analysis ◽  
Inflammatory Markers ◽  
Idiopathic Inflammatory Myopathies ◽  
Inflammatory Myopathies ◽  
Muscle Enzymes

scholarly journals FRI0248 PROGNOSTIC FACTORS FOR STEROID-FREE REMISSION IN PATIENTS WITH IDIOPATHIC INFLAMMATORY MYOPATHIES: IMPORTANCE OF ANTHROPOMETRIC MEASUREMENTS

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 708.1-708
Author(s):  
J. S. Lee ◽  
S. H. Nam ◽  
S. J. Choi ◽  
W. J. Seo ◽  
S. Hong ◽  
...  
Keyword(s):  
Prognostic Factors ◽  
Cox Regression ◽  
Idiopathic Inflammatory Myopathies ◽  
Inflammatory Myopathies ◽  
Immunosuppressant Therapy ◽  
Muscle Enzymes ◽  
Cox Regression Analysis ◽  
Factors Associated ◽  
Early Use

Background:Several studies have been conducted on factors associated with mortality in idiopathic inflammatory myopathies (IIM), but few studies have assessed prognostic factors for steroid-free remission in IIM.Objectives:We investigated the various clinical factors, including body measurements, that affect IIM treatment outcomes.Methods:Patients who were newly diagnosed with IIM between 2000 and 2018 were included. Steroid-free remission was defined as at least three months of normalisation of muscle enzymes and no detectable clinical disease activity. The factors associated with steroid-free remission were evaluated by a Cox regression analysis.Results:Of the 106 IIM patients, 35 displayed steroid-free remission during follow-up periods. In the multivariable Cox regression analyses, immunosuppressants’ early use within one month after diagnosis [hazard ratio (HR) 6.21, 95% confidence interval (CI) 2.61–14.74, p < 0.001] and sex-specific height quartiles (second and third quartiles versus first quartile, HR 3.65, 95% CI 1.40–9.51, p = 0.008 and HR 2.88, 95% CI 1.13–7.32, p = 0.027, respectively) were positively associated with steroid-free remission. Polymyositis versus dermatomyositis (HR 0.21, 95% CI 0.09–0.53, p = 0.001), presence of dysphagia (HR 0.15, CI 0.05–0.50, p = 0.002) and highest versus lowest quartile of waist circumference (WC) (HR 0.24, 95% CI 0.07–0.85, p = 0.027) were negatively associated with steroid-free remission.Conclusion:The early initiation of immunosuppressant therapy, type of myositis and presence of dysphagia are strong predictors of steroid-free remission in IIM; moreover, height and WC measurements at baseline may provide additional important prognostic value.Disclosure of Interests:None declared

Myositis (Oxford Rheumatology Library)

2018 ◽  
Keyword(s):  
Risk Factors ◽  
Skeletal Muscle ◽  
Environmental Risk ◽  
Inclusion Body Myositis ◽  
Idiopathic Inflammatory Myopathies ◽  
Inflammatory Myopathies ◽  
Histopathological Changes ◽  
Muscle Enzymes ◽  
Health Significance ◽  
Laboratory Investigations

Myositis, or the idiopathic inflammatory myopathies (IMM) are a group of rare autoimmune diseases of considerable health significance. Features include muscle weakness, raised skeletal muscle enzymes, and characteristic histopathological changes. IIM can be split into polymyositis (PM), dermatomyositis (DM), and inclusion body myositis (IBM). If diagnosed promptly PM and DM can be treated with immunosuppression, but IBM currently cannot be treated. Summarizing the current understanding of the epidemiology, genetic and environmental risk factors, and clinical features, this handbook gives practical strategies for laboratory investigations and treatment paradigms. Future strategies and ongoing trials are also discussed.

scholarly journals A study on demographic pattern of idiopathic inflammatory myopathies in a tertiary care hospital

2019 ◽  
Vol 6 (2) ◽  
pp. 236
Author(s):  
Srujith C. H. ◽  
Kavitha Mohanasundaram ◽  
Jagadeesan M. ◽  
Halleys Kumar E. ◽  
Kannan R. ◽  
...  
Keyword(s):  
Tertiary Care ◽  
Lactic Dehydrogenase ◽  
Idiopathic Inflammatory Myopathies ◽  
Care Hospital ◽  
Inflammatory Myopathies ◽  
Muscle Enzymes ◽  
Cross Sectional ◽  
Inflammatory Myositis ◽  
Idiopathic Inflammatory Myositis

Background: Idiopathic inflammatory myopathies (IIMs) are a group of chronic systemic autoimmune diseases characterized by proximal muscle weakness and elevated muscle enzymes. Aim and Objective was to analyze the demographic profile of patients with idiopathic inflammatory myopathies (IIM).Methods: This was a cross sectional observational study conducted over a period of two years (2016-2018). After obtaining institutional ethical committee clearance, informed consent from patients. 16 patients who fulfilled the criteria were included in the study. The demographic and the clinical data were analysed.Results: The mean age was 47.3±11.2 years. The study showed female predominance. ANA was positive in 11(68.7%) patients. Among the 16 patients, 5 (31.25%) had polymyositis and 11 (68.7%) had dermatomyositis. The median enzymes levels were creatinine kinase 1134 U/L, lactic dehydrogenase 477U/L, ALT (alanine aminotransferase) 154 IU/L, AST (aspartate aminotransferase) 236IU/L. Raynaud's phenomenon was seen in 37.5%. In our study, 31.25% had hypothyroidism and 6.25% had diabetic mellitus. On follow up 37.5% developed interstitial lung disease (ILD) and 18.75% were found to have malignancy.Conclusions: Steroids and immunomodulators are the mainstay of treatment in patients with idiopathic inflammatory myositis. All our patients improved with steroids. It is important to evaluate these patients during early stages and follow up to prevent complications.

Polymyositis and Dermatomyositis

2019 ◽  
pp. 187-189
Author(s):  
Josephina A. Vossen
Keyword(s):  
Muscle Biopsy ◽  
Clinical Examination ◽  
Immune Suppression ◽  
Imaging Modality ◽  
Idiopathic Inflammatory Myopathies ◽  
Inflammatory Myopathies ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Muscle Enzymes ◽  
Muscle Enzyme

Chapter 42 discusses polymyositis and dermatomyositis, which are idiopathic inflammatory myopathies characterized by muscle inflammation, proximal muscle weakness, and elevated muscle enzymes. Idiopathic inflammatory myopathies represent a heterogeneous group of muscle diseases. Diagnosis is based on clinical examination, muscle enzyme laboratory values, electromyography (EMG), and muscle biopsy. MRI is the most important imaging modality used in diagnosis and management. Radiographs and CT may detect calcinosis. Treatment is primarily by immune suppression.

scholarly journals Prognostic factors for steroid-free remission in patients with idiopathic inflammatory myopathies: importance of anthropometric measurements

2020 ◽  
Vol 12 ◽  
pp. 1759720X2093682
Author(s):  
Jung Sun Lee ◽  
Jung Eun Lee ◽  
Seokchan Hong ◽  
Chang-Keun Lee ◽  
Bin Yoo ◽  
...  
Keyword(s):  
Prognostic Factors ◽  
Cox Regression ◽  
Idiopathic Inflammatory Myopathies ◽  
Inflammatory Myopathies ◽  
Immunosuppressant Therapy ◽  
Muscle Enzymes ◽  
Cox Regression Analysis ◽  
Factors Associated ◽  
Early Use

Background: Several studies have been conducted on factors associated with mortality in idiopathic inflammatory myopathies (IIMs), but few studies have assessed prognostic factors for steroid-free remission in IIM. We investigated the various clinical factors, including body measurements, that affect IIM treatment outcomes. Methods: Patients who were newly diagnosed with IIM between 2000 and 2018 were included. Steroid-free remission was defined as at least 3 months of normalisation of muscle enzymes and no detectable clinical disease activity. The factors associated with steroid-free remission were evaluated by a Cox regression analysis. Results: Of the 106 IIM patients, 35 displayed steroid-free remission during follow-up periods. In the multivariable Cox regression analyses, immunosuppressants’ early use within 1 month after diagnosis [hazard ratio (HR) 6.21, 95% confidence interval (CI) 2.61–14.74, p < 0.001] and sex-specific height quartiles (second and third quartiles versus first quartile, HR 3.65, 95% CI 1.40–9.51, p = 0.008 and HR 2.88, 95% CI 1.13–7.32, p = 0.027, respectively) were positively associated with steroid-free remission. Polymyositis versus dermatomyositis (HR 0.21, 95% CI 0.09–0.53, p = 0.001), presence of dysphagia (HR 0.15, CI 0.05–0.50, p = 0.002) and highest versus lowest quartile of waist circumference (WC; HR 0.24, 95% CI 0.07–0.85, p = 0.027) were negatively associated with steroid-free remission. Conclusion: The early initiation of immunosuppressant therapy, type of myositis and presence of dysphagia are strong predictors of steroid-free remission in IIM; moreover, height and WC measurements at baseline may provide additional important prognostic value.

Biological therapy in idiopathic inflammatory myopathies

2014 ◽  
Vol 155 (1) ◽  
pp. 3-10
Author(s):  
Levente Bodoki ◽  
Melinda Nagy-Vincze ◽  
Zoltán Griger ◽  
Andrea Péter ◽  
Csilla András ◽  
...  
Keyword(s):  
T Cells ◽  
Muscle Weakness ◽  
Biological Therapy ◽  
Therapeutic Options ◽  
Idiopathic Inflammatory Myopathies ◽  
Inflammatory Myopathies ◽  
Progressive Muscle Weakness ◽  
Immune Mediated ◽  
Novel Therapeutic

Idiopathic inflammatory myopathies are systemic, immune-mediated diseases characterized by proximal, symmetrical, progressive muscle weakness. The aim of this work is to give an overview of the biological therapy used in the treatment of idiopathic inflammatory myopathies. The authors also focus on novel results in the therapy directed against the B- and T-cells. They emphasize the importance of new trials in these diseases which may lead to the introduction of novel therapeutic options in these disorders. Orv. Hetil., 2014, 155(1), 3–10.

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.

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