Article
Rheumatology
Pitcha Chompoopong, Michael P. Skolka, Floranne C. Ernste, Margherita Milone, Teerin Liewluck
Summary: This study investigated the clinical and pathological spectrum of myopathy in sarcoidosis patients and found that sarcoid myopathy is not the only cause of symptomatic myopathy in these patients. IBM is the second most common cause of myopathies in sarcoidosis, and the frequency of IBM in sarcoidosis is higher than in the general population. The study also identified features associated with non-sarcoid myopathies and found that immunosuppressive therapy has limited efficacy in improving symptoms in non-sarcoid myopathy patients. Abnormal MxA expression was observed in some patients, which warrants further research.
Article
Geriatrics & Gerontology
Judith Canto-Santos, Laura Valls-Roca, Ester Tobias, Francesc Josep Garcia-Garcia, Mariona Guitart-Mampel, Anna Esteve-Codina, Beatriz Martin-Mur, Mercedes Casado, Rafael Artuch, Estel Solsona-Vilarrasa, Jose Carlos Fernandez-Checa, Carmen Garcia-Ruiz, Carles Rentero, Carlos Enrich, Pedro J. J. Moreno-Lozano, Jose Cesar Milisenda, Francesc Cardellach, Josep M. M. Grau-Junyent, Gloria Garrabou
Summary: In this study, molecular disturbances were found in fibroblast samples from IBM patients, including abnormal gene expression related to inflammation, mitochondria, and cell cycle regulation, as well as functional changes in inflammatory, autophagy, mitochondrial, and metabolic processes. These findings contribute to a better understanding of the pathogenesis of IBM and provide insights for the identification of new biomarkers and therapeutic strategies.
JOURNAL OF CACHEXIA SARCOPENIA AND MUSCLE
(2023)
Article
Rheumatology
Sangmee Sharon Bae, Ani Shahbazian, Jennifer Wang, Ilana Golub, Buzand Oganesian, Tyler Dowd, Beata Vayngortin, Ryan Wang, David Elashoff, Srinivasa T. Reddy, Christina Charles-Schoeman
Summary: This study found that the activity of PON1 is significantly reduced in patients with IIM compared to healthy controls, and it is inversely associated with disease activity and the presence of severe ILD. The PON1 Q192R polymorphism is strongly linked to the paraoxonase activity of PON1 in IIM, and patients with the PON1 QQ genotype may have better disease outcomes in IIM.
Article
Rehabilitation
Gabrielle Brokamp, Lauren Hurst, Leigh Hartog, Ferdinand Vilson, Jerold Reynolds, Bakri H. Elsheikh, W. David Arnold
Summary: This study retrospectively investigates the frequency and progression of ventilatory muscle dysfunction in patients with inclusion body myositis. It shows a high frequency of ventilatory pump muscle weakness, which is associated with more severe respiratory symptoms. Baseline muscle strength may indicate the risk of respiratory decline, highlighting the importance of pulmonary function surveillance as ventilatory and limb muscle decline may not progress correspondingly.
AMERICAN JOURNAL OF PHYSICAL MEDICINE & REHABILITATION
(2023)
Article
Biochemistry & Molecular Biology
Brunetta Porcelli, Miriana d'Alessandro, Latika Gupta, Silvia Grazzini, Nila Volpi, Maria Romana Bacarelli, Federica Ginanneschi, Giovanni Biasi, Francesca Bellisai, Marta Fabbroni, David Bennett, Claudia Fabiani, Luca Cantarini, Elena Bargagli, Bruno Frediani, Edoardo Conticini
Summary: This study assessed the diagnostic accuracy of anti-cN1A antibodies in patients with suspected IIM and evaluated the agreement between line immunoassay (LIA) and enzyme-linked immunoassay (ELISA). The results showed excellent concordance between LIA and ELISA for detecting anti-cN1A antibodies, suggesting that LIA could be a rapid and useful adjunct, and it could even replace ELISA for cN1A assay.
Article
Biochemistry & Molecular Biology
Karsten Kummer, Imke Bertram, Sabrina Zechel, Daniel B. B. Hoffmann, Jens Schmidt
Summary: This study identified the NLRP3 inflammasome as a key factor in the development of inclusion body myositis (IBM), a chronic and treatment-resistant muscle disease. The overexpression of NLRP3 was observed both in cell culture models and muscle biopsies from IBM patients, indicating its potential role in driving inflammation and protein accumulation in IBM.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Clinical Neurology
Ulrika Lindgren, Carola Hedberg-Oldfors, Rille Pullerits, Christopher Lindberg, Anders Oldfors
Summary: Inclusion body myositis (IBM) is an inflammatory myopathy with no effective treatment. A population-based study in western Sweden identified 142 patients with IBM, among which 6 patients were defined as early-onset IBM due to young age at symptom onset. These patients showed severe disease progression, high muscle mitochondrial DNA mutation load, and reduced survival in young and middle-aged individuals.
JOURNAL OF NEUROLOGY
(2023)
Article
Medicine, General & Internal
Valerie Leclair, Angeles S. Galindo-Feria, Simon Rothwell, Olga Krystufkova, Sepehr Sarrafzadeh Zargar, Herman Mann, Louise Pyndt Diederichsen, Helena Andersson, Martin Klein, Sarah Tansley, Lars Ronnblom, Kerstin Lindblad-Toh, Ann-Christine Syvanen, Marie Wahren-Herlenius, Johanna K. Sandling, Neil McHugh, Janine A. Lamb, Jiri Vencovsky, Hector Chinoy, Marie Holmqvist, Matteo Bianchi, Leonid Padyukov, Ingrid E. Lundberg, Lina-Marcela Diaz-Galloc
Summary: In patients with idiopathic inflammatory myopathies (IIM), autoantibodies are associated with specific HLA genetic variants and clinical manifestations. Distinct HLA class II and I associations were observed for almost all autoantibody-defined subgroups, supporting the use of autoantibody profiles for classifying IIM.
Review
Medicine, General & Internal
Catherine Ashton, Shereen Paramalingam, Brittany Stevenson, Anna Brusch, Merrilee Needham
Summary: Idiopathic inflammatory myopathy is an umbrella term for various subtypes including dermatomyositis, polymyositis, and necrotising autoimmune myopathy, each presenting with similar clinical features but distinguishable by serum creatine kinase levels and specific muscle involvement. Treatment options are evolving, with focus on diagnosis and therapy based on clinical assessment, serology, and imaging.
INTERNAL MEDICINE JOURNAL
(2021)
Review
Clinical Neurology
Adeel S. Zubair, Sharfaraz Salam, Mazen M. Dimachkie, Pedro M. Machado, Bhaskar Roy
Summary: Idiopathic inflammatory myopathies (IIMs) are acquired muscle diseases characterized by muscle inflammation, weakness, and extra-muscular manifestations. Imaging biomarkers such as MRI, muscle ultrasound, EIM, and PET play a crucial role in the diagnosis, assessment, and management of IIMs. MRI is the most commonly used imaging biomarker, but its availability and cost are limitations. Muscle ultrasound and EIM are convenient and can be performed in clinical settings, but further validation is needed. These technologies provide objective assessment of muscle health in IIMs and contribute to improving patient care.
FRONTIERS IN NEUROLOGY
(2023)
Review
Cell Biology
Ramy Abdelnaby, Khaled Ashraf Mohamed, Anas Elgenidy, Yousef Tarek Sonbol, Mahmoud Mostafa Bedewy, Aya Moustafa Aboutaleb, Mohamed Ayman Ebrahim, Imene Maallem, Khaled Tarek Dardeer, Hamed Amr Heikal, Hazem Maher Gawish, Jana Zschuentzsch
Summary: Inclusion body myositis (IBM) is a progressive muscle weakness disease diagnosed by clinical and histopathological criteria. Muscle ultrasound shows potential diagnostic value for IBM, but there is currently no international standardized criteria. Existing studies have demonstrated significant differences in echogenicity of the flexor digitorum profundus (FDP) and gastrocnemius (GC) muscles between IBM patients and healthy controls. Further high-quality research is needed to implement muscle ultrasound in the diagnostic criteria for IBM.
Article
Cardiac & Cardiovascular Systems
Aleksandra Halina Opinc, Marcin Adam Makowski, Zuzanna Malgorzata Lukasik, Joanna Samanta Makowska
Summary: This review provides a detailed study of cardiovascular involvement in idiopathic inflammatory myopathies (IIM), indicating that while such involvement is frequent in IIM, it typically remains subclinical with congestive heart failure being the most common symptomatic form. Further research is needed to investigate the development of cardiovascular diseases and the impact of different treatment options.
HEART FAILURE REVIEWS
(2021)
Article
Clinical Neurology
Mridul Johari, Anna Vihola, Johanna Palmio, Manu Jokela, Per Harald Jonson, Jaakko Sarparanta, Sanna Huovinen, Marco Savarese, Peter Hackman, Bjarne Udd
Summary: The study investigates the molecular mechanisms in inclusion body myositis (IBM) through gene expression and splicing analysis. It reveals dysregulation of genes involved in calcium homeostasis and their impact on T-cell functioning in IBM muscles, suggesting that loss of apoptotic control of cytotoxic T cells may contribute to the abnormal cytolytic activity in IBM.
JOURNAL OF NEUROLOGY
(2022)
Article
Agriculture, Dairy & Animal Science
Yvet Opmeer, Guy C. M. Grinwis, G. Diane Shelton, Marco Rosati, Vanessa Alf, Hille Fieten, Peter A. J. Leegwater, Kaspar Matiasek, Paul J. J. Mandigers
Summary: The Dutch Kooiker dog is one of nine Dutch dog breeds and has a heritable inflammatory myopathy. This paper aims to describe the clinical signs, laboratory results, electromyography, and histopathology of the affected dogs. The study reveals a likely heritable inflammatory myopathy in this breed, and further research is needed to understand its classification, treatment, and genetic cause to eliminate the disease.
Review
Immunology
Edoardo Conticini, Miriana D'Alessandro, Suhel Gabriele Al Khayyat, Roberto D'Alessandro, Emilio D'Ignazio, Anna Paola Pata, Giulia Vallifuoco, Paolo Falsetti, Caterina Baldi, Marco Bardelli, Stefano Gentileschi, Claudia Fabiani, Maria Antonietta Mazzei, Andrea Guarnieri, Elena Bargagli, Luca Cantarini, Bruno Frediani
Summary: Vasculitis can affect various organs and systems, but muscle involvement is rare. This review collected evidence on muscle involvement in systemic vasculitis patients and found that most of them had medium and small vessels vasculitis or vasculitis secondary to rheumatoid arthritis. Muscle vasculitis is usually associated with necrotizing vasculitis, while granulomatous vasculitis is more common in ANCA-associated vasculitis patients. The disease presentation can be severe, but conventional immunosuppressants and/or glucocorticoids have shown to be effective treatments.
AUTOIMMUNITY REVIEWS
(2022)