Inflammatory myopathies

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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]

Contrasting trends in anti-MDA5 dermatomyositis

Japanese researchers have studied data from 154 patients diagnosed with dermatomyositis (DM), with the presence of autoantibodies directed against the MDA5 (menaloma differentiation-associated gene) antigen: in these forms, muscular involvement is generally modest, unlike interstitial lung disease, which often takes center stage, mortality is particularly high in the medium term (26% of cases studied), although … [Read more]

The serological signature of certain types of myositis could be useful in establishing a pulmonary prognosis

British and Spanish researchers retrospectively analysed the profile of myositis-specific autoantibodies in a group of patients with inflammatory myopathy with associated pneumonitis (ILD-IMM) and in another where the patients had interstitial pneumonitis with autoimmune features (IPAF): 430 patients recruited in the UK and belonging mainly (80%) to the first group were included in the study, … [Read more]

The favourable results of topical application of sodium thiosulphate in dermatomyositis

The CATSS-O retrospective study, for Calcifications Treated by Sodium Thiosulphate – Observational, included data acquired between 2014 and 2020 from 35 patients, including 28 with dermatomyositis or scleromyositis with ectopic calcifications that were often multiple (calcinosis). After six months of daily applications of a sodium thiosulphate-based ointment (25%) prepared by the Limoges University Hospital pharmacy, … [Read more]