Inflammatory myopathies

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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]

Histological and molecular confirmation of the tolerability of atorvastatin in dermatomyositis and antisynthetase syndrome

A Brazilian team conducted a pilot study of the effects of atorvastatin 20mg/day versus placebo for 12 weeks in 20 people with stable dermatomyositis (DM) or antisynthetase syndrome (SAS) and hyperlipidaemia. The results, published in 2021, showed good clinical muscle tolerance and an improvement in the lipid profile on atorvastatin. The same team studied muscle … [Read more]

Two new biomarkers useful for diagnosing inclusion myositis and autoimmune necrotizing myopathy

Three Belgian researchers tested the diagnostic value of two serum markers in muscle pathology: the chemokine CXCL10 and the cytokine GDF5. These biomarkers were measured in 21 patients with autoimmune necrotising myopathy (ANIM), 18 with sporadic inclusion myositis (SIM), three with overlapping myositis, two with dermatomyositis and one with an anti-synthetase syndrome, all were compared … [Read more]

guide to systematic screening for cancers associated with myositis

At the initiative of the international consortium IMACS (International Myositis Assessment and Clinical Studies Group), experts in adult idiopathic inflammatory myopathies have drawn up recommendations designed to improve cancer screening in this type of disease: online surveys combined with a Delphi-type methodology served as the basis for the experts’ deliberations, cancer risk stratification and screening … [Read more]

Facial involvement is common in inclusion myositis

Following on from previous work on facioscapulohumeral muscular dystrophy (FSHD), researchers in Marseilles have studied the characteristics of facial muscle deficit in people with inclusion myositis (IBM), an inflammatory pathology preferentially affecting a middle-aged population: the study cohort comprised 32 IBM sufferers, the protocol consisted of video analysis of facial movements during seven specific tasks, … [Read more]

IV immunoglobulins are particularly effective against the cutaneous signs of dermatomyositis

The good results of the international ProDERM trial, evaluating Octagam® intravenous immunoglobulin versus placebo in 95 adults with dermatomyositis, led to an extension of the indication in 2022. Post-hoc analysis of these data shows that monthly infusions of Octagam® : a significant improvement versus placebo in the cutaneous manifestations of the disease (CDASI-A and CDASI-D … [Read more]

The development of arimoclomol stops in inclusion myositis

With a prevalence of 1 in 200,000 in Europe, sporadic inclusion myositis is the most common idiopathic myositis after the age of 50. It is characterised by muscle damage, often asymmetric with amyotrophy, and begins in the quadriceps and/or finger flexors. Muscle biopsy shows two phenomena: inflammation and degeneration with accumulation of various proteins (beta-amyloid, … [Read more]

Myositis patients prefer subcutaneous Ig at home to intravenous infusions in hospital

“I have regained control of my life”. This verbatim summarises the very consensual view of a group of seven adults with polymyositis or dermatomyositis on the home administration of polyvalent immunoglobulins (Ig) subcutaneously, using an infusion pump or syringe pump. Followed at the Pitié-Salpêtrière Hospital (Paris), these patients took part in a qualitative study, which … [Read more]

A comparative study of the extra-muscular manifestations of anti-synthetase syndrome and dermatomyositis

As part of the MYONET patient registry, a consortium of clinicians compared the clinical and biological data of adult patients with either antisynthetase syndrome (ASys) or dermatomyositis (DM): 1054 patients were included in the study (405 with ASys and 649 with DM), One third of patients in the ASys cohort had skin manifestations suggestive of … [Read more]