Review: Myositis-specific autoantibodies, a cornerstone in immune-mediated necrotizing myopathy

Publication date: Available online 11 January 2019Source: Autoimmunity ReviewsAuthor(s): Céline Anquetil, Olivier Boyer, Nadège Wesner, Olivier Benveniste, Yves AllenbachAbstractOver the past few years, myositis-specific autoantibodies played an increasing role in the inflammatory idiopathic myositis definition. They became the critical immunological marker for immune-mediated necrotizing myopathy diagnosis (IMNM) since the paradigm switch from histological to serological criteria.This review is focused on the key role of the anti-signal recognition particle (anti-SRP) and the anti-3-Hydroxy-3-MethylGlutaryl-Coenzyme A Reductase (anti-HMGCR) antibodies in immune-mediated necrotizing myopathy.Anti-SRP and anti-HMGCR antibodies are robust diagnostic tools in case of both the classical subacute form and the slowly progressive form of IMNM that may mimic muscular dystrophy. Anti-SRP and anti-HMGCR patients share clinical, biological and histological features with some antibody-associated specificity. Anti-SRP patients harbour more severe muscle weakness and atrophy with severe muscle damage on magnetic resonance imaging study. Approximately 10–20% of anti-SRP patients develop extramuscular symptoms, especially lung interstitial disease. Conversely, anti-HMGCR patients are often associated with statin exposure. In both cases, patients have a poor outcome with frequent relapse and the use of combined immunotherapy. Of note, various data suggest a direct pathogen...
Source: Autoimmunity Reviews - Category: Allergy & Immunology Source Type: research

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Our objective is to describe two genetically confirmed Oculopharyngeal Muscular Dystrophy (OPMD) patients with HMGCoARdase-antibody positive autoimmune necrotic myopathy (AINM) responsive to immunosuppressive treatment. Although clinicians have suspected muscular dystrophy patients are at increased risk of statin side-effects, to date no patients with genetically confirmed muscular dystrophy have been reported with HMGCoRdase-antibody positive AINM. Here we describe the clinical presentation, serology, muscle pathology and response to treatment in two patients with genetically confirmed OPMD who developed statin-associated AINM.
Source: Neuromuscular Disorders - Category: Neurology Authors: Source Type: research
We report a 9-year-old boy with familial hyperlipidemia who presented with creatinine kinase elevation.
Source: Neuromuscular Disorders - Category: Neurology Authors: Source Type: research
In conclusion, our study demonstrated that elevated cumulative SBP or DBP was independently associated with increased risk of CVD in the Chinese population. Among participants with 15-year cumulative BP levels higher than the median, that is, 1970.8/1239.9 mmHg-year for cumulative SBP/DBP, which was equivalent to maintaining SBP/DBP level higher than 131/83 mmHg in 15 years, the CVD risk would increase significantly irrespective of whether or not the BP measurements at one examination was high. Our findings emphasize the importance of cumulative BP level in identifying individuals with high risk of CVD in the future. ...
Source: Fight Aging! - Category: Research Authors: Tags: Newsletters Source Type: blogs
This article expresses sentiments regarding medical technology and human longevity that we'd all like to see more of in the mainstream media. At some point, it will come to be seen by the average person as basically sensible to work towards minimizing the tide of suffering and death caused aging and age-related disease. It has been, in hindsight, a strange thing to live in a world in which most people were reflexively opposed to that goal. Death and aging constitute a mystery. Some of us die more quickly. We often ask about it as children, deny it in youth, and reluctantly come to accept it as adults. Aging is uni...
Source: Fight Aging! - Category: Research Authors: Tags: Newsletters Source Type: blogs
Today's materials are a helpful overview of the brace of biotech companies working to slow or reverse aspects of mitochondrial aging. Mitochondria play a central role in core cellular processes and are important in degenerative aging. Every cell contains a herd of hundreds of mitochondria, the descendants of an ancient symbiosis between the first cells and bacteria that could help them survive. Each mitochondrion contains one or more copies of mitochondrial DNA, a small remnant of the original bacterial genome, just a few genes that evolution has yet to move to the cell nucleus. The mitochondrial population of a cell is dy...
Source: Fight Aging! - Category: Research Authors: Tags: Longevity Industry Source Type: blogs
This update begins with muscle manifestations of coronavirus 2019. They may include myalgias and elevations in serum creatine kinase. It is unknown whether there is direct muscle invasion and how often the critically ill have muscle sequelae. Regarding autoimmune myopathies, a retrospective study of statin-induced necrotizing myopathy is covered. A relatively large proportion of patients had normal strength at presentation. Examples of dermatomyositis associated with immune checkpoint inhibitors are provided including one with cytokine storm. A report of juvenile dermatomyositis with severe abdominal complications is notew...
Source: Journal of Clinical Neuromuscular Disease - Category: Neurology Tags: Review Article Source Type: research
This study examines the function of the α1 and α2 Na,K-ATPase isozymes in diaphragm and soleus muscles from mdx and Bla/J mice compared to control С57Bl/6 mice. Conventional electrophysiology, quantitative PCR and Western blotting, co-immunoprecipitation as well as confocal microscopy with cytochemistry were used. In diaphragm muscle fibers from mdx and Bla/J mice plasma membrane was depolarized due to specific loss of the α2 Na,K-ATPase electrogenic activity, which was more pronounced in the junctional (motor endplate) region; the a2 Na,K-ATPase abundance decrease and membrane cholesterol re-distributio...
Source: Am J Physiol Cell Ph... - Category: Cytology Authors: Tags: Am J Physiol Cell Physiol Source Type: research
ász Csanádi Vígh Bácskay BGP-15 is a new insulin sensitizer drug candidate, which was developed by Hungarian researchers. In recent years, numerous research groups have studied its beneficial effects. It is effective in the treatment of insulin resistance and it has protective effects in Duchenne muscular dystrophy, diastolic dysfunction, tachycardia, heart failure, and atrial fibrillation, and it can alleviate cardiotoxicity. BGP-15 exhibits chemoprotective properties in different cytostatic therapies, and has also proven to be photoprotective. It can additionally have advantageou...
Source: Molecules - Category: Chemistry Authors: Tags: Review Source Type: research
This study demonstrates for the first time that senescent cells secrete functional LTs, significantly contributing to the LTs pool known to cause or exacerbate idiopathic pulmonary fibrosis. Against Senolytics https://www.fightaging.org/archives/2019/11/against-senolytics/ There is no consensus in science that is so strong as to have no heretics. So here we have an interview with a naysayer on the matter of senolytic treatments, who argues that the loss of senescent cells in aged tissues will cause more harm to long-term health than the damage they will do by remaining. To be clear, I think this to be a ...
Source: Fight Aging! - Category: Research Authors: Tags: Newsletters Source Type: blogs
Our world has never witnessed a time of greater promise for improving human health. Many of today’s health advances have stemmed from a long arc of discovery that begins with strong, steady support for basic science. In large part because of fundamental research funded by the National Institutes of Health (NIH), which traces its roots to 1887, Americans are living longer, healthier lives. Life expectancy for a baby born in the U.S. has risen from 47 years in 1900 to more than 78 years today. Among the advances that have helped to make this possible are a 70% decline in the U.S. death rate from cardiovascular disease ...
Source: TIME: Science - Category: Science Authors: Tags: Uncategorized Healthcare medicine Source Type: news
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