Clinical profile of anti-NXP-2 antibody-positive inflammatory myositis and outcome in an Indian population
ConclusionThe current study shows that adult DM patients with anti-NXP-2 autoantibodies have a unique clinical phenotype. Its presentation differs between adult and JDM, even in different parts of the world. Muscle weakness is mild and responds to treatment. Dysphagia needs more time and aggressive IS for improvement as compared to other muscle involvement.Key Points• Anti-NXP-2 antibody presentation varied from adult to child, as in different parts of the world.• In Indian adult patients, non-specific skin manifestations were more common, whereas in JDM, specific skin features were common.• There was less likely inv...
Source: Clinical Rheumatology - October 6, 2023 Category: Rheumatology Source Type: research

Acthar ® Gel Treatment for Patients with Autoimmune and Inflammatory Diseases: An Historical Perspective and Characterization of Clinical Evidence
AbstractActhar® Gel (repository corticotropin injection) is a naturally sourced complex mixture of adrenocorticotropic hormone analogs and other pituitary peptides that is believed to have both steroidogenic and nonsteroidogenic immunomodulatory effects via activation of melanocortin receptors in various cells throughout the body. Since 1952, Acthar has been approved by the US Food and Drug Administration to treat a variety of autoimmune and inflammatory diseases. Since 2014, Mallinckrodt Pharmaceuticals has conducted a large number of preclinical, clinical, and real-world-evidence studies of Acthar for the treatment of r...
Source: Clinical Drug Investigation - October 4, 2023 Category: Drugs & Pharmacology Source Type: research

P332 Mitochondrial pathology associated with refractory dermatomyositis after COVID-19 vaccination
We report on clinical, immunological, radiological and myopathological characterization of a case of refractory anti-Mi-2 DM which began after SARS-CoV-2 vaccine administration. (Source: Neuromuscular Disorders)
Source: Neuromuscular Disorders - October 1, 2023 Category: Neurology Authors: A. Lauletta, G. Merlonghi, L. Fionda, M. Garibaldi Source Type: research

P330 Profile of adult idiopathic inflammatory myopathy in Dr Cipto Mangunkusumo Hospital Indonesia as tertiary health care
Idiopathic inflammatory myopathy (IIM) is a heterogeneous group of autoimmune disorders, frequently underdiagnosed, and characterized by clinical, autoantibody (Ab), and pathology findings, encompassing dermatomyositis (DM), immune-mediated necrotizing myopathy (IMNM), antisynthetase syndrome (ASS), overlap myositis (OM), polymyositis (PM), and inclusion body myositis. The study aims to characterize the organ manifestations and Ab profile of IIM in Cipto Mangunkusumo Hospital. This is the first reported IIM profile in Indonesia. (Source: Neuromuscular Disorders)
Source: Neuromuscular Disorders - October 1, 2023 Category: Neurology Authors: L. Indrawati, S. Wibowo, A. Widhani, E. Novianto, C. Nagpal, D. Paveta, E. Susanto, Y. Bilianti, N. Fadli, A. Budikayanti, A. Safri, W. Wiratman, F. Octaviana, M. Hakim Source Type: research

P329 The role of autoantibodies in diagnosis of Idiopathic inflammatory myopathies
Idiopathic inflammatory myopathies (IIM) are heterogenous disease group with common characteristics such as muscle weakness, muscle fatigue, and inflammation of muscle tissue. In recent years, four main types of IIM are widely recognized: Polymyositis (PM), Dermatomyositis (DM), Inclusion body myositis (IBM) and immune-mediated necrotizing myopathy (IMNM). We investigated clinical, pathological and serological features in subgroups of IIM patients who have visited Gangnam Severance hospital. We retrospectively reviewed the medical records of the myopathy database from August 2002 to March 2023. (Source: Neuromuscular Disorders)
Source: Neuromuscular Disorders - October 1, 2023 Category: Neurology Authors: S. Kim, S. Lee, H. Park, Y. Choi Source Type: research

Single cell RNA sequencing sheds light on infiltrating T cells in idiopathic inflammatory myopathies
EMBO Mol Med. 2023 Sep 28:e18190. doi: 10.15252/emmm.202318190. Online ahead of print.ABSTRACTIdiopathic inflammatory myopathies (IIM), also referred to as "myositis," are a group of heterogeneous autoimmune disorders characterised by muscle weakness, atrophy and progressive reduced mobility (Lundberg et al, 2021). IIM represent a significant health burden in adult populations, affecting individuals at a mean age of 50 with an estimated prevalence of 2.9-34 per 100,000 (Dobloug et al, 2015; Svensson et al, 2017). IIM encompass several subtypes including dermatomyositis, immune-mediated necrotising myopathy, inclusion-body ...
Source: Molecular Medicine - September 28, 2023 Category: Molecular Biology Authors: Momina Yazdani Lukas Mach Michela Noseda Source Type: research

Correction to: Polymyositis/dermatomyositis readmissions: analysis of the nationwide readmission database
(Source: Clinical Rheumatology)
Source: Clinical Rheumatology - September 14, 2023 Category: Rheumatology Source Type: research

Immunopathological features of myopathy associated with small-to-medium-sized vessel vasculitis and differences from autoimmune myositis
CONCLUSIONS: Patients with vasculitis demonstrated mild myofiber damage based on the lower involvement of CD56/NCAM-expressing myofibers compared to those with AIM. Complement component deposits on the vessel walls and hypervascularity in the endomysium areas may be immunopathological features of vasculitic myopathy.PMID:37706291 | DOI:10.55563/clinexprheumatol/hpoapl (Source: Clinical and Experimental Rheumatology)
Source: Clinical and Experimental Rheumatology - September 14, 2023 Category: Rheumatology Authors: Shun Nomura Yasuhiro Shimojima Takanori Ichikawa Daigo Miyazaki Akinori Uruha Dai Kishida Yoshiki Sekijima Source Type: research