scholarly journals Adult Dermatomyositis with Bleeding Ulcer in the Pharynx

2014 ◽  
Vol 2014 ◽  
pp. 1-4
Author(s):  
Junko Kusano ◽  
Yuka Takahashi ◽  
Yoshikata Misaki ◽  
Norihiko Murai
Keyword(s):  
Respiratory Tract ◽  
Inflammatory Cell ◽  
Immunosuppressive Agents ◽  
Preventive Measure ◽  
Low Grade ◽  
Idiopathic Inflammatory Myopathies ◽  
Inflammatory Myopathies ◽  
Rare Manifestation ◽  
Electric Cautery ◽  
Gi Mucosa

Dermatomyositis (DM) is one of the idiopathic inflammatory myopathies caused by complement-mediated vasculopathy or vasculitis in the muscle. Although the gastrointestinal (GI) mucosa has been reported to be involved as a result of vasculitis or vasculopathy, ulceration in the pharynx is a rare manifestation of DM. A 54-year-old woman complaining of muscle weakness in the extremities, low-grade fever, and dysphagia was diagnosed as having DM. Despite medical treatment with corticosteroids and immunosuppressive agents, her DM progressed rapidly, leading to exacerbation of the dysphagia. About 3 weeks after undergoing tracheostomy as a preventive measure against aspiration, the patient developed intractable respiratory tract hemorrhage. Repeated laryngoendoscopy revealed a bleeding ulceration in the pharynx that required hemostasis with electric cautery under general anesthesia. No bleeding recurred thereafter. Histopathologically, the pharynx exhibited nonspecific inflammatory cell infiltration in the muscle tissue. This rare manifestation may be considered in cases of DM with unexplainable airway bleeding.

In the idiopathic inflammatory myopathies (IIM), do reactive oxygen species (ROS) contribute to muscle weakness?

2015 ◽  
Vol 74 (7) ◽  
pp. 1340-1346 ◽  
Author(s):  
Adam P Lightfoot ◽  
Anne McArdle ◽  
Malcolm J Jackson ◽  
Robert G Cooper
Keyword(s):  
Reactive Oxygen Species ◽  
Muscle Weakness ◽  
Inflammatory Cell ◽  
Immune Cell ◽  
Ros Generation ◽  
Muscle Dysfunction ◽  
Idiopathic Inflammatory Myopathies ◽  
Oxygen Species ◽  
Inflammatory Myopathies

The idiopathic inflammatory myopathies (IIMs) are a group of rare autoimmune disorders, collectively known as myositis. Affected patients present with proximal muscle weakness, which usually improves following treatment with immunosuppressants, but often incompletely so, thus many patients remain weak. IIMs are characterised histologically by inflammatory cell infiltrates into skeletal muscle and overexpression of major histocompatibility complex I on muscle cell surfaces. Although inflammatory cell infiltrates represent a major feature of myositis there is growing evidence that muscle weakness correlates only poorly with the degree of cellular infiltration, while weakness may in fact precede such infiltrations. The mechanisms underpinning such non-immune cell mediated weakness in IIM are poorly understood. Activation of the endoplasmic reticulum stress pathways appears to be a potential contributor. Data from non-muscle cells indicate that endoplasmic reticulum stress results in altered redox homeostasis capable of causing oxidative damage. In myopathological situations other than IIM, as seen in ageing and sepsis, evidence supports an important role for reactive oxygen species (ROS). Modified ROS generation is associated with mitochondrial dysfunction, depressed force generation and activation of muscle catabolic and autophagy pathways. Despite the growing evidence demonstrating a key role for ROS in skeletal muscle dysfunction in myopathologies other than IIM, no research has yet investigated the role of modified generation of ROS in inducing the weakness characteristic of IIM. This article reviews current knowledge regarding muscle weakness in the absence of immune cells in IIM, and provides a background to the potential role of modified ROS generation as a mechanism of muscle dysfunction. The authors suggest that ROS-mediated mechanisms are potentially involved in non-immune cell mediated weakness seen in IIM and outline how these mechanisms might be investigated in this context. This appears a timely strategy, given recent developments in targeted therapies which specifically modify ROS generation.

Evaluation of a New Skeletal Troponin I Assay in Patients with Idiopathic Inflammatory Myopathies

2020 ◽  
Vol 5 (2) ◽  
pp. 320-331
Author(s):  
Katriina Bamberg ◽  
Laura Mehtälä ◽  
Olli Arola ◽  
Seppo Laitinen ◽  
Pauliina Nordling ◽  
...  
Keyword(s):  
Diagnostic Accuracy ◽  
Troponin I ◽  
Limit Of Detection ◽  
Clinical Performance ◽  
Low Grade ◽  
Idiopathic Inflammatory Myopathies ◽  
Strong Positive Correlation ◽  
Inflammatory Myopathies ◽  
Reference Limit ◽  
Upper Reference Limit

Abstract Background The current biomarkers for diagnosis and monitoring of injured and diseased skeletal muscles, such as creatine kinase (CK), have limited tissue specificity and incapability to differentiate between pathological and physiological changes. Thus, new biomarkers with improved diagnostic accuracy are needed. Our aim was to develop and validate a novel assay for skeletal troponin I (skTnI), and to assess its clinical performance in patients with idiopathic inflammatory myopathies (IIM). Methods A two-step fluoroimmunoassay was used to analyze samples from healthy reference individuals (n = 140), patients with trauma (n = 151), and patients with IIM (n = 61). Results The limit of detection was 1.2 ng/mL, and the upper reference limit (90th percentile) was 5.2 ng/mL. The median skTnI concentrations were <limit of detection (LoD), 2.7 ng/mL, and 8.6 ng/mL in reference, trauma, and IIM cohorts, respectively. Differences in measured skTnI levels were statistically significant between all three study cohorts (Kruskal–Wallis P < 0.001; Mann–Whitney P < 0.001 for all). skTnI and CK had a strong positive correlation (Spearman’s r = 0.771, P < 0.001), and the longitudinal changes in skTnI mirrored those observed with CK. Conclusions With the skTnI assay, patients with IIM were identified from healthy individuals and from patients with traumatic muscular injuries. When compared to CK, skTnI appeared to be more accurate in managing patients with low-grade IIM disease activities. The developed assay serves as a reliable analytical tool for the assessment of diagnostic accuracy of skTnI in the diagnosis and monitoring of myopathies.

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.

scholarly journals Myositis autoantibodies in a racially diverse population of children with idiopathic inflammatory myopathies

2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Megan Mariko Perron ◽  
Natalia Vasquez-Canizares ◽  
Gabriel Tarshish ◽  
Dawn M. Wahezi
Keyword(s):  
Clinical Phenotype ◽  
Laboratory Data ◽  
Small Sample ◽  
Organ Involvement ◽  
Idiopathic Inflammatory Myopathies ◽  
Diverse Population ◽  
Inflammatory Myopathies ◽  
Racially Diverse ◽  
Parametric Testing ◽  
Overlap Myositis

Abstract Background Juvenile idiopathic inflammatory myopathies (JIIMs) is a group of autoimmune disorders, including juvenile dermatomyositis (JDM), juvenile polymyositis (JPM) and overlap myositis, that are characterized by proximal muscle weakness, elevated levels of serum muscle enzymes, and pathognomonic skin findings. While the exact etiology of JIIMs is unclear, the presence of myositis specific autoantibodies (MSAs) have been associated with certain clinical phenotypes, organ involvement and disease prognosis. To date, there have been few studies of the associations between MSA presence and patient ethnicity. It is important to understand the extent to which ethnicity impacts disease manifestations, organ involvement and clinical outcomes. The goal of our study is to determine MSA and myositis associated autoantibody (MAA) presence, clinical phenotype, and disease course in a racially diverse population of pediatric patients with JIIMs. Methods Patients age 2–21 years with a prior diagnosis of JDM, JPM or overlap myositis, who had been tested for MSA/MAA, were eligible for study inclusion. Clinical and laboratory data were collected retrospectively via manual chart review in this single-center study. Descriptive statistics were performed to summarize each variable. Given the small sample size, non-parametric testing was performed using Fischer’s exact test, Wilcoxon rank sum test and Kruskal-Wallis test. Results Thirty one patients were included in the analysis. Race and ethnicity were self-reported as Hispanic (48.4%), white (25.8%), and Black (25.8%). The most prevalent MSAs were anti-MDA5 (25.8%), anti-p155/140 (22.6%) and anti-MJ (19.4%). Presence of autoantibodies (p = 0.04) and pulmonary disease (p = 0.03) were significantly higher in patients of Black or Hispanic descent compared with white descent. Anti-MDA5 antibodies, cutaneous ulceration, cardiopulmonary involvement, hospitalizations and one death were only reported in patients with Black or Hispanic descent. Patients with anti-MDA5 antibodies were more likely to be male (p = 0.04) and to have cutaneous ulceration (p = 0.02). Conclusions This study describes the prevalence of MSA/MAA in a racially diverse group of patients with JIIM and further delineates clinical phenotype and disease complications in these groups. We found a relatively high proportion of children with anti-MDA5 antibodies and described potentially worse clinical courses in children of Black or Hispanic descent. Further investigation is warranted to examine these findings.

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