Inflammatory myopathies
RSS feedA 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]
Thrombotic microangiopathy observed in the course of SRP autoimmune necrotising myopathy
A Japanese team of neurologists and nephrologists report the observation of a 61-year-old patient diagnosed with autoimmune necrotising myopathy: the clinical history and the positivity of anti-SRP autoantibodies were consistent with this diagnosis, treatment consisted of corticosteroids and immunoglobulins, followed by tacrolimus, eight weeks after initiation of this treatment, thrombotic microangiopathy was observed and confirmed … [Read more]
A West Indian cohort of patients with juvenile dermatomyositis
Specialists in rheumatology and neuromuscular pathology in the French West Indies report the clinical and biological data of 21 adolescents aged between 2.5 and 14 diagnosed with juvenile dermatomyositis (JDM) between 2000 and 2023 in hospitals in the French West Indies-Guyana region: in addition to motor deficits, these patients had dysphagia (two-thirds of cases) and … [Read more]
