Inflammatory myopathies
RSS feedGranulomatous myositis: a rare form of inflammatory myopathy
French researchers report the clinical and histological data and immunological profile of 26 patients diagnosed with granulomatous myositis: the age of onset was generally very late (median age 65), with clinical severity observed in half the cases, in 14 out of 26 cases, the etiology was sarcoidosis, and more rarely other causes (inclusion myositis, paraneoplastic … [Read more]
Overview of the main myopathies that can begin in the over-50s
A French review has set out to provide an overview of the most emblematic late-onset myopathies (LOMs), those which may appear after the age of 50, and to identify the pitfalls to be avoided and the important steps in the diagnostic approach to these pathologies. Based on an assessment of the literature and medical data … [Read more]
New hypotheses on the pathophysiology of inclusion myositis
The pathophysiology of inclusion body myositis (IBM) remains poorly understood. Against this background, American researchers have put forward some new hypotheses: their study looked at muscle biopsies from 38 patients with IBM and 22 healthy individuals matched for age and gender, several complementary approaches were used (transcript study, immunological profile, immunocytochemistry and Western blot, etc.), … [Read more]
‘Universalâ CAR-T cells in autoimmune necrotising myopathy (and scleroderma)
In February 2024, an initial Chinese team published the case of a patient suffering from a refractory form of autoimmune necrotising myopathy, who went into remission following an infusion of CAR-T cells directed against B lymphocyte maturation antigen (BCMA). Six months later, a second publication, this time from a clinical trial, reported another success in … [Read more]
A case of myositis following gene therapy for DMD
American clinicians involved in gene therapy designed to deliver a truncated version of the dystrophin gene (microdystrophin) by vein, using an adeno-associated virus, have reported a complication that was already known but rarely encountered: the patient was 9 years old and still able to walk at the time of gene therapy (GT), his genotype corresponded … [Read more]
A higher incidence of AINM in the New Zealand population of Polynesian origin
Clinicians report a large and growing number of cases of autoimmune necrotising myopathy (AINM) in New Zealand: 40 new cases of HMGCR autoantibody-positive AINM were diagnosed over a two-year period (2019-2021), all were in people aged over 40 who had been exposed to cholesterol-lowering statins, the calculated incidence was found to be five times higher … [Read more]
Differential circulating microRNA profiles in several subtypes of inflammatory myopathies
Italian researchers have studied extra-cellular vesicles (EVs) present in the bloodstream and containing several types of very small messenger RNA: in particular, microRNAs (miRNAs) and RNAs that interact with the piwi protein (piRNAs) were measured, the plasma of 47 patients with different types of myositis was analysed and compared with the plasma of 45 healthy … [Read more]
Properties of the functional scale used in inclusion myositis
An Anglo-Saxon consortium studied the psychometric properties of the IBMFRS (Inclusion Body Myositis Functional Rating Scale), the reference scale used in patients with inclusion myositis (IBM): to this end, the investigators retrospectively analysed the IBMFRS data accumulated during a multicentre clinical trial involving 150 patients with IBM, over a total period of 20 months, all … [Read more]
Improved pneumococcal vaccination coverage in inflammatory diseases in the United States
Faced with very low vaccination coverage rates, particularly against pneumococcus, in patients with inflammatory pathologies, American clinicians set up an action plan: the target population was patients with lupus or connective tissue diseases (including inflammatory myopathies such as dermatomyositis), whether or not they were being treated with immunosuppressive drugs, two types of vaccine (PCV13 and … [Read more]
ENMC 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]