Inflammatory myopathies

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]

“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]

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]

A team of Italian clinicians report the observation of a 77-year-old woman presenting with a classic picture of dermatomyositis (DM) with positive anti-Mi-2 autoantibodies: study of a biopsy muscle fragment nevertheless revealed very marked abnormalities in the mitochondria, in addition to the characteristic lesions usually found in DM (extensive perifascicular atrophy), the patient did not … [Read more]

An international consortium of clinicians coordinated by the Strasbourg Reference Centre looked at patients with inflammatory myopathy with regard to two uncommon clinical signs: camptocormia and/or head droop syndrome in 49 patients with inflammatory myopathy, their clinical and biological data were compared with those of 98 patients with inflammatory myopathy but without these two signs, … [Read more]

A Canadian team conducted a single-center study of 41 people with refractory myositis treated with tofacitinib after failure of four to five immunosuppressants: 23 had classical dermatomyositis, 12 an amyopathic form and 6 polymyositis; tofacitinib produced statistically and clinically significant skin improvement in the dermatomyositis group, but had no significant effect on muscle in all … [Read more]

Texas clinicians report the highly atypical case of a 65-year-old patient initially diagnosed with dermatomyositis on the basis of classic disease criteria: the initial picture consisted of a muscle deficit that appeared a few weeks after a rash on the face, the work-up revealed hyperCKemia at 4,000 IU/l, positive autoantibodies to Mi2, inflammatory infiltrates and … [Read more]

Chinese researchers analyzed a series of 79 patients with idiopathic inflammatory myopathy, distinguishing between those with prolonged fever at the initial stage of their disease and those who remained apyretic: in the febrile group, there was a higher frequency of cutaneous signs (mechanic’s hand) and respiratory complications (acute interstitial lung disease), all occurring in patients … [Read more]

Dermatomyositis is accompanied, in a significant number of cases, and most often in the medium to long term, by para-articular calcifications (calcinosis). The pathophysiology of calcinosis remains poorly understood. American researchers at the NIH studied it quantitatively and qualitatively using whole-body CT scans: 31 patients were included in this prospective study, including 14 adults with … [Read more]

Two German teams report on their successful CAR-T cell treatment of refractory anti-Jo1-associated antisynthetase syndrome in two patients. A first case reported by a team from Erlangen After an initial transient worsening (myalgia, CPK 13,600 U/L, fever for 3 days), the 41-year-old man’s condition quickly improved. Three months after the injection of CAR-T cells: lesions … [Read more]