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An ambulatory abortion service in a municipal hospital is described. Objectives were to provide safe, ambulatory, and easily accessible service to patients. The role of family planning in the service is discussed and the success of the operation is evaluated. [ABSTRACT FROM AUTHOR]

Les myopathies inflammatoires idiopathiques sont un groupe de maladies musculaires rares dont le diagnostic repose sur l'évaluation clinique et les biomarqueurs sérologiques et histologiques. Alors que l'immunomarquage HLA-I (MHC-I) est utilisé en routine comme marqueur diagnostic de myosite, le rôle de l'immunomarquage HLA-DR (MHC-II) reste à préciser. Dans cette étude, nous avons examiné les profils d'expression d'HLA-DR par les myofibres et les capillaires, sur les biopsies musculaires de 103 patients, appartenant à cinq sous-groupes de myopathies inflammatoires idiopathiques : dermatomyosite (DM, n = 31), myosite à inclusions (IBM, n = 24), syndrome des anti-synthétase (ASyS, n = 10), myopathie nécrosante auto-immune (MNAI, n = 18) et myosite de chevauchement (OM, n = 20). Une expression anormale de l'HLA-DR par les myofibres a été observée chez 63 des 103 patients (61 %), avec des profils distincts dans les différents sous-groupes : diffuse dans les IBM (96 %), négative dans les MNAI (83 %), périfasciculaire dans les ASyS (70 %), à la fois négative (61 %) et périfasciculaire (32 %) dans les DM, et groupée (40 %), périfasciculaire (30 %) ou hétérogène diffuse (15 %) dans les OM. En outre, le marquage capillaire HLA-DR a révélé des anomalies capillaires quantitatives, comme une perte en capillaires (47 sur 88, 53 %), et des anomalies qualitatives comme les capillaires dilatés et les marquages capillaires « baveux » (79 sur 98, 81 %). Ces résultats indiquent que le marquage HLA-DR représente un outil complémentaire intéressant pour le diagnostic des sous-groupes de myopathies inflammatoires idiopathiques. Nos résultats suggèrent que l'ajout de l'immunomarquage HLA-DR dans les évaluations histologiques de routine pourrait faciliter le diagnostic entre les différents sous-types de myopathies inflammatoires idiopathiques. [ABSTRACT FROM AUTHOR]

Among the idiopathic inflammatory myopathies, patients harbouring an Antisynthetase syndrome exhibit a unique clinical picture, with characteristic signs such as myositis, interstitial lung disease, arthritis, rash, and/or fever. Characteristic morphological features on skeletal muscle biopsies differentiate Antisynthetase syndrome from other forms of myositis. Autoantibodies typically recognizing one of the members of the aminoacyl-tRNA synthetase family of proteins can be detected in the serum of such patients, with anti-Jo1 being most frequent. Until now, an international consensus definition of the Antisynthetase syndrome is lacking, hence this workshop has undertaken the task to inform about the clinical, morphological and autoantibody profiles of Antisynthetase syndrome. The authors also expand their aims by giving management and therapeutic strategies, and finally provide precise classification criteria for Antisynthetase syndrome., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024.)

Idiopathic inflammatory myopathies (IIM) are rare, acquired muscle diseases; their diagnosis of is based on clinical, serological, and histological criteria. MHC-I-positive immunostaining, although non-specific, is used as a marker for IIM diagnosis; however, the significance of major histocompatibility complex (MHC)-II immunostaining in IIM remains debated. We investigated patterns of MHC-II immunostaining in myofibers and capillaries in muscle biopsies from 103 patients with dermatomyositis ([DM], n = 31), inclusion body myositis ([IBM], n = 24), anti-synthetase syndrome ([ASyS], n = 10), immune-mediated necrotizing myopathy ([IMNM], n = 18), or overlap myositis ([OM], n = 20). MHC-II immunostaining of myofibers was abnormal in 63/103 of patients (61%) but the patterns differed according to the IIM subgroup. They were diffuse in IBM (96%), negative in IMNM (83%), perifascicular in ASyS (70%), negative (61%) or perifascicular (32%) in DM, and either clustered (40%), perifascicular (30%), or diffuse heterogeneous (15%) in OM. Capillary MHC-II immunostaining also identified quantitative (capillary dropout, n = 47/88, 53%) and qualitative abnormalities, that is, architectural abnormalities, including dilated and leaky capillaries, (n = 79/98, 81%) in all IIM subgroups. Thus, MHC-II myofiber expression patterns allow distinguishing among IIM subgroups. We suggest the addition of MHC-II immunostaining to routine histological panels for IIM diagnosis., (© The Author(s) 2024. Published by Oxford University Press on behalf of American Association of Neuropathologists, Inc.)

Myotonic dystrophy type I (DM1) is a genetic disease characterized by a multisystemic RNA metabolism dysregulation and splicing toxicity. Numerous signaling pathways are deregulated, and especially AMPK, a key sensor and regulator of cellular metabolism. To restore AMPK signaling activity in DM1 muscle tissue and cells could improve mitochondrial biogenesis and dynamics, mitophagy and oxidative stress, energy production and, in fine, skeletal muscle tissue homeostasis., (© 2024 médecine/sciences – Inserm.)

Granulomatous myositis is a clinical-pathological entity, which has been rarely reported, mostly described in sarcoidosis. Currently, no clear and simple prognostic factor has been identified to predict granulomatous myositis evolution. The clinical, anatomopathological, imaging, and biological characteristics of 26 patients with granulomatous myositis were retrospectively collected to describe clinical presentation and outcomes of this condition. Twenty-six patients with granulomatous myositis were included (14 males) with a median age of symptom onset of 65 years. 54 % of patients presented a severe form of the disease defined as a Rankin score ≥2 at last follow-up visit or a progressive form of the disease (no improvement under treatment). Etiology were sarcoidosis (n = 14), inclusion body myositis (n = 4), autoimmune disease (n = 1), hematological malignancy (n = 1), and idiopathic (n = 6). Distal deficit and amyotrophy were more frequent among those with a severe disease. Corticosteroids led to improvement in 75 % of cases, but 66 % of responders relapsed. Methotrexate appeared as a promising second line therapy with clinical improvement in 50 % of patients, and no relapse in responders. Granulomatous myositis is often a severe and difficult-to-treat disease in which patients frequently progress towards severe disability. The presence of muscle atrophy and distal weakness appears to be frequently associated with a severe form of the disease., Competing Interests: Declaration of competing interest The authors have no conflicts of interest to declare., (Copyright © 2024. Published by Elsevier B.V.)

Myositis with anti-Ku-autoantibodies is a rare inflammatory myopathy associated with various connective tissue diseases. Histopathological studies have identified inflammatory and necrotizing aspects, but a precise morphological analysis and pathomechanistic disease model are lacking. We therefore aimed to carry out an in-depth morpho-molecular analysis to uncover possible pathomechanisms. Muscle biopsy specimens from 26 patients with anti-Ku-antibodies and unequivocal myositis were analyzed by immunohistochemistry, immunofluorescence, transcriptomics, and proteomics and compared to biopsy specimens of non-disease controls, immune-mediated necrotizing myopathy (IMNM), and inclusion body myositis (IBM). Clinical findings and laboratory parameters were evaluated retrospectively and correlated with morphological and molecular features. Patients were mainly female (92%) with a median age of 56.5 years. Isolated myositis and overlap with systemic sclerosis were reported in 31%, respectively. Isolated myositis presented with higher creatine kinase levels and cardiac involvement (83%), whereas systemic sclerosis-overlap patients often had interstitial lung disease (57%). Histopathology showed a wide spectrum from mild to pronounced myositis with diffuse sarcolemmal MHC-class I (100%) and -II (69%) immunoreactivity, myofiber necrosis (88%), endomysial inflammation (85%), thickened capillaries (84%), and vacuoles (60%). Conspicuous sarcoplasmic protein aggregates were p62, BAG3, myotilin, or immunoproteasomal beta5i-positive. Proteomic and transcriptomic analysis identified prominent up-regulation of autophagy, proteasome, and hnRNP-related cell stress. To conclude, Ku + myositis is morphologically characterized by myofiber necrosis, MHC-class I and II positivity, variable endomysial inflammation, and distinct protein aggregation varying from IBM and IMNM, and it can be placed in the spectrum of scleromyositis and overlap myositis. It features characteristic sarcoplasmic protein aggregation on an acquired basis being functionally associated with altered chaperone, proteasome, and autophagy function indicating that Ku + myositis exhibit aspects of an acquired inflammatory protein-aggregate myopathy., (© 2024. The Author(s).)