Inflammatory myopathies
RSS feedENMC workshop rethinks diagnosis and clinical trial design in inclusion myositis
Ten years after the publication of the diagnostic criteria for inclusion myositis, an international group of around thirty experts (including two from France) took part in the 272nd workshop of the European Neuromuscular Centre (ENMC) in June 2023 in the Netherlands. Their work resulted in: the inclusion of new diagnostic criteria, such as the finding … [Read more]
Inclusion myositis and sirolimus: the final results of the Rapami trial pave the way for a phase III
Sporadic inclusion myositis combines inflammatory processes and degeneration. It is the most common myositis after the age of 50, but also the only one that is refractory to the usual treatments for these autoimmune diseases (corticoids, immunosuppressants). Between 2015 and 2017, with financial support from the AFM-Téléthon, Prof. Olivier Benveniste’s team conducted a randomised, double-blind, … [Read more]
Effect of sirolimus on muscle in inclusion myositis observed by MRI and spectroscopy – Interview with Harmen Reyngoudt
Harmen Reyngoudt is co-director of the Institute’s NMR imaging and spectroscopy laboratory. He has just published an article* in J Cachexia Sarcopenia Muscle on the effect of sirolimus on muscle assessed by magnetic resonance imaging and spectroscopy. Interview with Harmen Reyngoudt. What is the background to this work? In order to make progress in clinical … [Read more]
Characterisation of Ku-positive myositis reveals a degree of heterogeneity
An international consortium, including clinicians from the Institute of Myology, pooled clinical and biological data from patients suffering from a rare form of inflammatory myopathy, Ku-positive myositis, and compared them with existing data for autoimmune necrotising myopathy (AINM): 26 muscle biopsies (anti-Ku-positive) from a cohort of 50 Japanese patients and 10 German patients investigated for … [Read more]
Dermatomyositis, polymyositis and the inner ear
In Taiwan, an analysis of reimbursement data for 1,622 patients suffering from polymyositis or dermatomyositis and 8,109 people free of these diseases showed : a significant difference in the prevalence of tinnitus (16.1% versus 12.7%) and sensorineural hearing loss (9.2% versus 6.8%) in the myositis group, there was also a significant difference in the prevalence … [Read more]
A Canadian survey highlights the very varied levels of knowledge of many adult neurologists regarding corticosteroid therapy
Adult neurologists specialising in neuromuscular diseases often prescribe long-term corticosteroids, particularly for autoimmune diseases (myositis, myasthenia gravis, etc.): an online survey of 99 Canadian neurologists practising in their country of origin or elsewhere was carried out, analysis of the responses from the 71 actual participants revealed shortcomings both in the screening of patients before starting … [Read more]
Overview of neuromuscular diseases
After a brief reminder of the structure of the motor unit and the various modes of inheritance, this document provides short descriptions of the neuromuscular diseases that are part of our scope at AFM-Téléthon, as well as how to manage and treat them. For each group of diseases, as well as in the motor unit … [Read more]
Transcranial direct current stimulation tested in myositis
A Brazilian single-center, randomized crossover clinical trial evaluated: in 17 adults with autoimmune necrotizing myopathy or dermatomyositis in remission or minimal activity, chronic algic and asthenic ; the combined effects over 10 days of a daily 30-minute session of physical activity (treadmill walking) and real (2 mA for 20 minutes) or mimed (30 seconds only) … [Read more]
Remission of refractory necrotizing myopathy after 18 months of CAR-T cell therapy
After good results in lupus, myasthenia and anti-synthetase syndrome, a new publication reports the successful treatment of another autoimmune disease in China with CAR-T cells directed against the B lymphocyte maturation antigen (or BCMA) developed by Iaso biotherapeutics, CT103A : the 25-year-old patient had lost his ability to walk due to refractory autoimmune necrotizing myopathy, … [Read more]
Some of the autoantibodies associated with myositis have prognostic value in juvenile forms
British researchers studied the serum levels and distribution of myositis-associated autoantibodies (MAAs) in 551 patients previously diagnosed with juvenile myositis: 36% of patients had at least one positive MAA, and 13% had at least two, among the positives, there was a higher prevalence of overlapping myositis, as well as Raynaud’s syndrome and interstitial lung disease, … [Read more]
