Pathogenic role of anti-cN1A autoantibodies in sporadic inclusion body myositis
Conclusions
Active cN1A peptide immunisation partially reproduced the clinical and histological aspects of sIBM in wild-type mice. The murine model demonstrates the pathogenic properties of anti-cN1A autoantibodies to cause sIBM-like histological changes. (Source: Journal of Neurology, Neurosurgery and Psychiatry)
Source: Journal of Neurology, Neurosurgery and Psychiatry - November 15, 2023 Category: Neurosurgery Authors: Yamashita, S., Tawara, N., Zhang, Z., Nakane, S., Sugie, K., Suzuki, N., Nishino, I., Aoki, M. Tags: Neuromuscular Source Type: research
Prominent mitochondrial pathology in a case of refractory dermatomyositis: coincidence or concause?
We report on clinical, immunological, radiological, and myopathological findings of a case of severe, treatment-refractory anti-Mi-2-positive DM.ResultsA 77-year-old woman developed anti-Mi-2 DM with severe diffuse muscle weakness associated with abundant mitochondrial abnormalities at muscle biopsy, beside the typical features of inflammatory myopathy. The patient was poorly responsive to multiple-line therapies and finally anti-JAK (anti-Janus activated kinase) was administered, leading to partial clinical improvement.DiscussionGiven the usual satisfactory treatment response and favorable outcome of anti-Mi-2 DM, we supp...
Source: Neurological Sciences - November 13, 2023 Category: Neurology Source Type: research
The Personal Financial Burden Associated with Idiopathic Inflammatory Myopathies
Idiopathic inflammatory myopathies (IIMs) are a group of rare systemic autoimmune diseases. IIMs include dermatomyositis, polymyositis, immune-mediated necrotizing myopathy, overlap syndrome, and inclusion body myositis (IBM). Although IIMs are rare with an annual incidence ranging from 14.0 to 17.4 per 100,000, the cost burden of IIMs on United States (US) healthcare spending is high. [13,18,19] The estimated total costs associated with IIMs, specifically with dermatomyositis and polymyositis, range from $533 to $702 million a year. (Source: Neuromuscular Disorders)
Source: Neuromuscular Disorders - November 3, 2023 Category: Neurology Authors: Catherine Hua, Abhiram R. Bhashyam, Manuel Lubinus, Lynn Wilson, Salman Bhai Source Type: research
Idiopathic Inflammatory Myopathies
AbstractIdiopathic inflammatory myopathies (IIMs) are a diverse group of diseases characterized by proximal muscle weakness and inflammation in skeletal muscle. Phenotypically, the subtypes include dermatomyositis, polymyositis, inclusion body myositis, and amyopathic dermatomyositis. The most common IIM in children is juvenile dermatomyositis (JDM). In contrast to adult dermatomyositis (DM), children are likely to have frequent relapses, vasculopathy, and long-term metabolic and other complications like lipodystrophy, insulin resistance, and calcinosis. Significant advances in our understanding of pathogenesis, disease co...
Source: Indian Journal of Pediatrics - November 3, 2023 Category: Pediatrics Source Type: research
Paraneoplastic dermatomyositis associated with urothelial cancer: report of a case and systematic review of the literature
ConclusionIn conclusion, our study provides knowledge and understanding for identifying specific risk factors in patients with the coexistence of bladder cancer and DM/PM and their management. During the initial and follow-up screening, age, gender, and the clinicopathological subgroup of myositis should be considered to ensure proper management of the condition. (Source: Frontiers in Oncology)
Source: Frontiers in Oncology - October 31, 2023 Category: Cancer & Oncology Source Type: research
A longitudinal study using B mode ultrasound and power Doppler as monitoring imaging tools in inclusion body myositis
CONCLUSIONS: Our study highlights previously unreported sex differences in US findings in IBM. Certain US domains, such as FT, showed measurable changes over time and correlated with disease progression. However, further studies with longer follow-up periods and larger patient cohorts will need to be performed to determine whether B mode US could be a useful disease outcome measure for therapeutic trials.PMID:37877419 | DOI:10.55563/clinexprheumatol/qkjmy8 (Source: Clinical and Experimental Rheumatology)
Source: Clinical and Experimental Rheumatology - October 25, 2023 Category: Rheumatology Authors: Shereen Paramalingam Merrilee Needham Frank L Mastaglia Helen I Keen Source Type: research
Cost of illness in inclusion body myositis: results from a cross-sectional study in Germany
Inclusion body myositis (IBM) is the most frequent type of myositis in elder patients with a slow chronic progression and refractory to treatment. Previous cost of illness (COI) studies in IBM used claims data... (Source: Orphanet Journal of Rare Diseases)
Source: Orphanet Journal of Rare Diseases - October 25, 2023 Category: Internal Medicine Authors: Katja C. Senn, Simone Thiele, Karsten Kummer, Maggie C. Walter and Klaus H. Nagels Tags: Research Source Type: research
Senescent fibro-adipogenic progenitors are potential drivers of pathology in inclusion body myositis
In this study, we focused on senescence of tissue-resident cells as potential driver of disease. For this purpose, we compared IBM patients to non-diseased controls and immune-mediated necrotizing myopathy patients. Histopathological analysis suggested that cellular senescence is a prominent feature of IBM, primarily affecting non-myogenic cells. In-depth analysis by single nuclei RNA sequencing allowed for the deconvolution and study of muscle-resident cell populations. Among these, we identified a specific cluster of fibro-adipogenic progenitors (FAPs) that demonstrated key hallmarks of senescence, including a pro-inflam...
Source: Acta Neuropathologica - October 15, 2023 Category: Neurology Source Type: research
Inclusion body myositis —health-related quality of life and care situation during phases of the “patience journey” in Germany: results from a qualitative study
To understand the health-related quality of life (HRQoL) in inclusion body myositis (IBM) from a holistic perspective on the background of a complex care situation. The focus was on how the patient journey may... (Source: Health and Quality of Life Outcomes)
Source: Health and Quality of Life Outcomes - October 10, 2023 Category: International Medicine & Public Health Authors: Katja C. Senn, Simone Thiele, Laura Gumbert, Sabine Krause, Maggie C. Walter and Klaus H. Nagels Tags: Research Source Type: research
P340 The MikroIBioM study - Comparison of gut microbiome of sporadic Inclusion Body Myositis (sIBM) patients and unaffected spouses
Sporadic inclusion body myositis (sIBM) is a disorder with features of both inflammation and degeneration but yet without effective treatment. Influences of the gut microbiome on degenerative as well as inflammatory disorders and immune treatments are known. Curious whether the gut microbiome might influence the development or recalcitrance of sIBM, we appealed to sIBM patients and their unaffected spouses for stool samples and data on stool, gastrointestinal symptoms and nutrition. We included 22 patients (n=2 clinically, n=20 clinico-pathologically defined; m:f 18:4; 51 – 83 years) and 21 controls (m:f 8:13; 53 – 82 ...
Source: Neuromuscular Disorders - October 1, 2023 Category: Neurology Authors: M. Winkler, W. Seel, C. Kornblum, M. Simon, J. Reimann Source Type: research
P335 Inhibition of KDM5A reverses pathological features in sporadic Inclusion Body Myositis-like cell models
Sporadic Inclusion Body Myositis (sIBM) is the most common acquired myopathy in adults over the age of 50 and characterised by inflammatory and degenerative processes leading to progressive muscle weakness and physical disability. In an in-house proteomics study, KDM5A, a histone demethylase, was found as key upstream regulator in sIBM patients whole muscle lysates, interconnecting with hallmark processes in sIBM, and hypothesised as disease master regulator. KDM5A overactivity and inhibition was studied in sIBM-like cell models, incubating human myoblasts with inflammatory cytokines (IL-1 β and TNFα) or through APP over...
Source: Neuromuscular Disorders - October 1, 2023 Category: Neurology Authors: G. De Vries, W. de Ridder, J. Baets 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
