Inflammatory myopathies

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The type of muscle damage has no influence on the prognosis of scleroderma

A multicentre retrospective study of 71 scleroderma patients who had undergone muscle biopsy found 46.5% with fibrosing myopathy, 25.5% with inflammatory myopathy and 28% with autoimmune necrotising myopathy. During follow-up, which averaged 6.4 years, 21 patients died, mainly from cardiovascular disease (39%) or infections (29%). The survival rate 10 years after the appearance of the … [Read more]

Belgian family confirms possible link between HLA-DRB1*11:01 and statin-induced myositis

In Belgium, a father and daughter : developed autoimmune necrotising myopathy with HMGCR autoantibodies a few years apart, following treatment with statins; are both carriers of HLA-DRB1*11:01, a variant already identified as being more frequent in this disease. The case of this Belgian family strengthens the hypothesis of genetic susceptibility to autoimmune myositis. Investigation of … [Read more]

Beware of phytotherapies in dermatomyositis

American clinicians have studied the prevalence and risk-taking among patients suffering from cutaneous autoimmune diseases who use herbal treatments: These included dermatomyositis and systemic lupus erythematosus (SLE), 673 adult patients consulting the University of Pennsylvania between 2007 and 2024 were included in a retrospective study, a third of the patients, particularly younger patients and Hispanics, … [Read more]

Positive feedback on multidisciplinary management of interstitial lung disease in France

Patients with certain inflammatory myopathies are at risk of developing severe interstitial lung disease. French clinicians working in the FAI2R and RespiFil healthcare networks report on their experience after setting up a multidisciplinary unit to help practitioners in the field confronted with this problem: this unit is made up of lung specialists, internists, rheumatologists, radiologists, … [Read more]

Granulomatous 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]

‘Universal’ 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]