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
Skeletal muscle fibers produce B-cell stimulatory factors in chronic myositis
ConclusionThe results substantiate the hypothesis of an involvement of B cell-associated mechanisms in the pathophysiology of IBM and PM. Muscle fibers themselves seem to contribute to the recruitment of B cells and sustain inflammation. (Source: Frontiers in Immunology)
Source: Frontiers in Immunology - September 5, 2023 Category: Allergy & Immunology Source Type: research
Skeletal muscle cell protein dysregulation highlights the pathogenesis mechanism of myopathy-associated p97/VCP R155H mutations
p97/VCP, a hexametric member of the AAA-ATPase superfamily, has been associated with a wide range of cellular protein pathways, such as proteasomal degradation, the unfolding of polyubiquitinated proteins, and autophagosome maturation. Autosomal dominant p97/VCP mutations cause a rare hereditary multisystem disorder called IBMPFD/ALS (Inclusion Body Myopathy with Paget’s Disease and Frontotemporal Dementia/Amyotrophic Lateral Sclerosis), characterized by progressive weakness and subsequent atrophy of skeletal muscles, and impacting bones and brains, such as Parkinson’s disease, Lewy body disease, Huntington’s disease...
Source: Frontiers in Neurology - August 3, 2023 Category: Neurology Source Type: research
Magnetic Resonance Imaging Findings of the Lower Limb Muscles in Anti-Mitochondrial M2 Antibody-Positive Myositis
Group of disorders characterized by myositis symptoms, including progressive muscle weakness and muscle inflammation in a setting of autoimmune abnormalities are collectively known as autoimmune myositis (AIM) or idiopathic inflammatory myopathy [1,2], including dermatomyositis (DM), anti-synthetase syndrome (ASS), immune-mediated necrotizing myopathy (IMNM), inclusion body myositis (IBM) [3,4] and other less well-characterized conditions. (Source: Neuromuscular Disorders)
Source: Neuromuscular Disorders - July 25, 2023 Category: Neurology Authors: Hirotaka Nomiya, Tadanori Hamano, Naoko Takaku, Hirohito Sasaki, Kojiro Usui, Sayaka Sanada, Tomohisa Yamaguchi, Yuki Kitazaki, Yoshinori Endo, Tomoko Kamisawa, Soichi Enomoto, Norimichi Shirahuzi, Akiko Matsunaga, Asako Ueno, Masamichi Ikawa, Osamu Yamam Tags: Research paper Source Type: research
Inclusion body myositis. Genetics, biomarkers and muscle biopsy
. (Source: International Journal of Neuroscience)
Source: International Journal of Neuroscience - July 19, 2023 Category: Neuroscience Authors: Ioannis Mavroudis Foivos Petridis Dimitrios Kazis Source Type: research
Long-term safety of COVID vaccination in individuals with idiopathic inflammatory myopathies: results from the COVAD study
AbstractLimited evidence on long-term COVID-19 vaccine safety in patients with idiopathic inflammatory myopathies (IIMs) continues to contribute to vaccine hesitancy. We studied delayed-onset vaccine adverse events (AEs) in patients with IIMs, other systemic autoimmune and inflammatory disorders (SAIDs), and healthy controls (HCs), using data from the second COVID-19 Vaccination in Autoimmune Diseases (COVAD) study. A validated self-reporting e-survey was circulated by the COVAD study group (157 collaborators, 106 countries) from Feb –June 2022. We collected data on demographics, comorbidities, IIM/SAID details, COVID-19...
Source: Rheumatology International - July 16, 2023 Category: Rheumatology Source Type: research
Transcriptional derepression of CHD4/NuRD-regulated genes in the muscle of patients with dermatomyositis and anti-Mi2 autoantibodies
Conclusions
Based on these findings, we hypothesise that anti-Mi2 autoantibodies could exert a pathogenic effect by entering damaged myofibres, inhibiting the CHD4/NuRD complex, and subsequently derepressing the unique set of genes defined in this study. (Source: Annals of the Rheumatic Diseases)
Source: Annals of the Rheumatic Diseases - July 13, 2023 Category: Rheumatology Authors: Pinal-Fernandez, I., Milisenda, J. C., Pak, K., Munoz-Braceras, S., Casal-Dominguez, M., Torres-Ruiz, J., Dell'Orso, S., Naz, F., Gutierrez-Cruz, G., Duque-Jaimez, Y., Matas-Garcia, A., Padrosa, J., Garcia-Garcia, F. J., Guitart-Mampel, M., Garrabou, G., Tags: ARD, Myositis Source Type: research
Exploring Hand and Upper Limb Function in Patients with Inclusion Body Myositis (IBM)
Inclusion Body Myositis (Source: Neuromuscular Disorders)
Source: Neuromuscular Disorders - June 26, 2023 Category: Neurology Authors: SM Hunn, LN Alfano, M Seiffert, CC Weihl Source Type: research
Study to Assess Content Validity and Interrater and Intrarater Reliability of the Inclusion Body Myositis Functional Rating Scale
This study consisted of 5 stages including phone interviews (physicians), face-to-face interviews (patients), face-to-face ratings, phone ratings, and ratings of videos using the IBMFRS.
Results
The IBMFRS adequately captures all core functional impacts of IBM, which was corroborated by both patient participants and physicians when debriefing the measure. Physicians and patient participants all thought any change on the measure would be meaningful change for a patient, either improvement or worsening. The quantitative analysis demonstrated good interrater reliability for face-to-face ratings (intraclass correlation coeffi...
Source: Neurology Clinical Practice - June 12, 2023 Category: Neurology Authors: Symonds, T., Randall, J., Lloyd-Price, L., Hudgens, S., Dimachkie, M. M., Guldberg, C., Machado, P. M. Tags: All Neuromuscular Disease, Muscle disease Research Article Source Type: research
Morphological and molecular comparison of HIV-associated and sporadic inclusion body myositis
ConclusionDespite HIV-IBM and sIBM sharing important clinical, histopathological, and transcriptomic signatures, the presence of KLRG1+ cells discriminated sIBM from HIV-IBM. This may be explained by longer disease duration and subsequent T-cell stimulation in sIBM. Thus, the presence of TEMRA cells is characteristic for sIBM, but not a prerequisite for the development of IBM in HIV+ patients. (Source: Journal of Neurology)
Source: Journal of Neurology - June 6, 2023 Category: Neurology Source Type: research
Intravenous Immunoglobulin in Idiopathic Inflammatory Myopathies: a Practical Guide for Clinical Use
AbstractPurpose of ReviewIdiopathic inflammatory myopathies (IIM) are a complex family of autoimmune systemic disorders which often affect muscle and/or skin. IIM cause significant morbidity and mortality, but optimal treatment is uncertain. This review provides a practical guide for using intravenous immunoglobulin (IVIG) and subcutaneous immunoglobulin (SCIG) in the management of IIM, including dermatomyositis (DM), polymyositis (PM), immune-mediated necrotizing myositis (IMNM), and spontaneous inclusion body myositis (IBM), based on relevant recent literature and experience. We summarize pertinent considerations when us...
Source: Current Rheumatology Reports - June 1, 2023 Category: Rheumatology Source Type: research
Molecular signature of neutrophil extracellular trap mediating disease module in idiopathic inflammatory myopathy
J Autoimmun. 2023 May 21;138:103063. doi: 10.1016/j.jaut.2023.103063. Online ahead of print.ABSTRACTThe rarity and heterogeneity of idiopathic inflammatory myopathy (IIM) pose challenges for researching IIM in affected individuals. We analyzed integrated transcriptomic datasets obtained using muscle tissues from patients with five distinct IIM subtypes to investigate the shared and distinctive cellular and molecular characteristics. A transcriptomic dataset of muscle tissues from normal controls (n = 105) and patients with dermatomyositis (n = 89), polymyositis (n = 33), inclusion body myositis (n = 121), immune-mediated n...
Source: Journal of Autoimmunity - May 23, 2023 Category: Allergy & Immunology Authors: Su-Jin Moon Seung Min Jung In-Woon Baek Kyung-Su Park Ki-Jo Kim Source Type: research
Molecular signature of neutrophil extracellular trap mediating disease module in idiopathic inflammatory myopathy
J Autoimmun. 2023 May 21;138:103063. doi: 10.1016/j.jaut.2023.103063. Online ahead of print.ABSTRACTThe rarity and heterogeneity of idiopathic inflammatory myopathy (IIM) pose challenges for researching IIM in affected individuals. We analyzed integrated transcriptomic datasets obtained using muscle tissues from patients with five distinct IIM subtypes to investigate the shared and distinctive cellular and molecular characteristics. A transcriptomic dataset of muscle tissues from normal controls (n = 105) and patients with dermatomyositis (n = 89), polymyositis (n = 33), inclusion body myositis (n = 121), immune-mediated n...
Source: Journal of Autoimmunity - May 23, 2023 Category: Allergy & Immunology Authors: Su-Jin Moon Seung Min Jung In-Woon Baek Kyung-Su Park Ki-Jo Kim Source Type: research
