Inflammatory myopathies

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Specific autoantibodies can predict response to treatment in dermatomyositis

French researchers have identified specific autoantibodies associated with myositis that may predict the response to treatment of dermatomyositis with Janus kinase (JAK) inhibitors (JAKi): 39 children and adolescents with juvenile dermatomyositis were included in the study, all of whom were followed at the Necker-Enfants Malades Reference Center in Paris. A positive, complete, or partial response … [Read more]

The particularities of dermatomyositis on black skin

The prevalence of dermatomyositis is higher in people with dark skin. After analysing 100 cases published since 1951, Canadian authors note that for this population: the initial diagnosis is incorrect in 10% of cases, with myositis being confused with hypertrophic lichen planus, an allergic reaction, a skin infection, etc. dyschromia (hypo- or hyperpigmentation), associated in … [Read more]

New developments in the treatment of certain refractory inflammatory myositis

German clinicians report a case of antisynthetase syndrome (ASyS) treated with several biotherapies: the patient, aged 45, had ASyS with signs of joint, muscle (myositis) and respiratory (interstitial lung disease) disease, with the presence of autoantibodies directed against the Jo-1 antigen, an initial treatment with CAR-T cells directed against CD19 lymphocytes resulted in remission for … [Read more]

Towards a better understanding of the side effects associated with the use of CAR-T cells

German clinicians have studied adverse side effects in patients with autoimmune diseases who have received CAR-T cells targeting the CD19 antigen: 39 adult patients, including six with inflammatory myopathy of autoimmune origin, were selected for this retrospective study. 30 of them developed a cutaneous and/or renal intolerance syndrome within 10 days of CAR-T cell injection. … [Read more]

Clinical presentations of immune-mediated necrotizing myopathy need to be better recognised

American clinicians, including Andrew Mammen, the researcher behind the first descriptions of immune-mediated necrotizing myopathy (IMNM), take stock of this pathology and highlight its atypical forms: after recalling the usual framework of this myopathy characterised by muscle weakness and the positivity of autoantibodies against the SRP and/or HMGCR proteins, the authors have compiled the atypical … [Read more]

The association of dermatomyositis and encephalopathy is exceptional but should be noted

Mexican clinicians report the clinical and immunological data of three patients with concomitant encephalopathy and dermatomyositis (DM): the three patients were adults in their fifties with no previous history of the disease, all met the criteria for dermatomyositis, including the presence of myositis-specific antibodies (in particular MDA5, Mi2 and TIF1g), the clinical picture included sub-acute … [Read more]

The benefit of reusing data from placebo groups in inclusion myositis

American researchers associated with the Institute of Myology Research Centre have compiled and re-analysed the data accumulated in the placebo arms of several clinical trials in inclusion myositis (IMM) : 11 therapeutic trials were selected, they involved 257 participants who had received only a placebo, the main endpoints were changes in muscle strength and, in … [Read more]

Immune-mediated necrotising myopathy in children, a Chinese picture

A study conducted by a hospital in Beijing (China) has refined our knowledge of the phenotype of immune-mediated necrotising myopathy in paediatrics: 55 of the 116 children and adolescents followed up for myositis by this centre between 2012 and 2024 had immune-mediated necrotising myopathy, a much higher proportion (47.4%) than expected, but one that would … [Read more]

Idiopathic myositis: an increased and early risk of cardiovascular events in cases of cardiac involvement at diagnosis

The Hôpital Saint-Antoine (Paris) conducted a retrospective study between 1997 and 2020 of 78 patients with idiopathic inflammatory myopathy (myositis). Of these, 42% had dermatomyositis, 23% had antisynthetase syndrome, 15% had overlapping myositis, 14% had necrotising myopathy and 5% had inclusion myositis. Cardiac involvement was present in 15% of patients at the time of myositis … [Read more]

FSHD combined with genuine myositis: an intriguing association

A French study involving clinicians from the Institute of Myology reports several new and disturbing cases of patients with two co-existing neuromuscular pathologies: firstly, facioscapulohumeral muscular dystrophy (FSHD), proven by molecular biology, and secondly, myositis authenticated by the presence of specific autoantibodies, of the 5 cases of this type identified in the myositis database of … [Read more]