Article
Clinical Neurology
Ying Hou, Kai Shao, Yaping Yan, Tingjun Dai, Wei Li, Yuying Zhao, Duoling Li, Jian-Qiang Lu, Gary L. Norman, Chuanzhu Yan
Summary: The study characterized the clinical and pathological features of anti-HMGCR myopathy by analyzing the presence of anti-HMGCR antibody in patients with idiopathic inflammatory myopathy. Optimization of antibody assays and confirmation by alternative assays improved sensitivity and specificity in diagnosis. Patients with DM-like skin rashes and lymphocytic infiltrates were found to represent a distinct subgroup of IIM.
JOURNAL OF NEUROLOGY
(2022)
Article
Biochemistry & Molecular Biology
Roberta Zerlotin, Marco Fornaro, Mariella Errede, Patrizia Pignataro, Clelia Suriano, Maddalena Ruggieri, Silvia Colucci, Florenzo Iannone, Maria Grano, Graziana Colaianni
Summary: In this cohort study, 41 patients with DM and IMNM were examined. The results showed lower expression of FNDC5 in the muscle biopsies of these patients compared to the control group, while serum irisin levels were higher. The authors hypothesize that increased expression of ADAM10 in the skeletal muscle of DM and IMNM patients may be responsible for the discrepancy between irisin levels and FNDC5 expression. Further research is needed to understand the mechanisms underlying exacerbated FNDC5 cleavage and muscle irisin resistance in these inflammatory myopathies.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Review
Rheumatology
Thomas Khoo, James B. Lilleker, Bernard Yu-Hor Thong, Valerie Leclair, Janine A. Lamb, Hector Chinoy
Summary: Idiopathic inflammatory myopathies (IIMs) are a group of systemic autoimmune diseases that affect skeletal muscles and other body parts. The incidence and prevalence of IIM are influenced by various factors, including age, gender, and geographical location. Although genetic and environmental factors have been identified, more research is needed to understand the differences in different ethnic groups.
NATURE REVIEWS RHEUMATOLOGY
(2023)
Article
Immunology
Barbara Williams, Michael P. Horn, Yara Banz, Laurence Feldmeyer, Peter M. Villiger
Summary: Knowledge about the cutaneous manifestations of HMGCR positive IMNM and HMGCR antibody positivity in other diseases is limited. Patients with IMNM were older, had more frequent statin exposure, and had higher mean peak CK levels. Cutaneous lesions were found to be more frequent in anti-HMRCR positive IMNM and the data suggest an activation of autoreactive B-lymphocytes in non-IMNM patients.
JOURNAL OF AUTOIMMUNITY
(2021)
Article
Immunology
Miao Ma, Kexia Chai, Rui Deng
Summary: The study suggests the involvement of the noncanonical pathway of pyroptosis in the pathogenesis of IIM, with drugs like glyburide and BBG showing intervention effects. Lower expression levels of P2X7R, NLRP3, and serum IL-1 beta were observed in the drug intervention groups compared to control groups.
INTERNATIONAL IMMUNOPHARMACOLOGY
(2021)
Article
Clinical Neurology
Stefan Nicolau, Margherita Milone, Jennifer A. Tracy, John R. Mills, James D. Triplett, Teerin Liewluck
Summary: IMNM patients can present with atypical clinical and histological features, leading to delays in diagnosis and treatment. Despite these atypical findings, clinicians should consider IMNM as a possible cause of unexplained proximal myopathies. Immunomodulatory therapy has shown to be effective in all patients.
Article
Cell Biology
Xue Ma, Hua-Jie Gao, Qing Zhang, Meng-Ge Yang, Zhua-Jin Bi, Su-Qiong Ji, Yue Li, Li Xu, Bi-Tao Bu
Summary: ER stress is involved in idiopathic inflammatory myopathies and correlates with disease activity in immune-mediated necrotizing myopathy.
FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY
(2022)
Review
Immunology
Gioia Merlonghi, Giovanni Antonini, Matteo Garibaldi
Summary: IMNMs are a subgroup of idiopathic inflammatory myopathies characterized by severe clinical presentation with specific histopathological features in muscle biopsy. Careful analysis is needed to differentiate IMNMs from other types of myopathies.
AUTOIMMUNITY REVIEWS
(2022)
Review
Medical Laboratory Technology
Konstantinos I. Tsamis, Constantinos Boutsoras, Evripidis Kaltsonoudis, Eleftherios Pelechas, Ilias P. Nikas, Yannis V. Simos, Paraskevi V. Voulgari, Ioannis Sarmas
Summary: Idiopathic inflammatory myopathies (IIMs) are rare autoimmune disorders primarily affecting muscles, but other organs can also be involved. Recent updates in diagnostic approach, utilizing circulating autoantibodies, have improved patient management.
CRITICAL REVIEWS IN CLINICAL LABORATORY SCIENCES
(2022)
Article
Clinical Neurology
Shahar Shelly, Michelle M. Mielke, Pritikanta Paul, Margherita Milone, Jennifer A. Tracy, John R. Mills, Christopher J. Klein, Floranne C. Ernste, Jay Mandrekar, Teerin Liewluck
Summary: This study aimed to determine the incidence and prevalence of immune-mediated necrotizing myopathy (IMNM) in adults in Olmsted County, Minnesota. The results showed an incidence rate of 8.3 per million person-years and a prevalence rate of 1.85 per 100,000 people in 2010. IMNM patients did not have a higher risk of developing cancer compared to the general population.
Article
Rheumatology
Prateek C. Gandiga, Daniela Ghetie, Elizabeth Anderson, Rohit Aggrawal
Summary: This review provides a practical guide for using intravenous immunoglobulin (IVIG) and subcutaneous immunoglobulin (SCIG) in the management of IIM. IVIG has been proven safe and effective against IIM in recent studies, and it can be used as a first-line or add-on treatment for various types of IIM. However, accessibility and cost remain limiting factors.
CURRENT RHEUMATOLOGY REPORTS
(2023)
Article
Clinical Neurology
Shahar Shelly, Niaz Talha, Naveen L. Pereira, Andrew G. Engel, Jonathan N. Johnson, Duygu Selcen
Summary: Patients with desmin-related myopathy may present with both neurologic and cardiac symptoms, usually starting in the third decade of life. Heart transplantation can be tolerated with improved cardiac function and quality of life.
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.
Article
Rheumatology
Lu Zhang, Hanbo Yang, Jieping Lei, Qinglin Peng, Hongxia Yang, Guochun Wang, Xin Lu
Summary: The study revealed that in IIM patients with AMAs, IMNM was a major histopathological finding, and AMAs were significantly associated with subclinical cardiac involvement. PBC might act as a protective factor for abnormal echocardiography findings in AMA-positive patients.
SEMINARS IN ARTHRITIS AND RHEUMATISM
(2021)
Article
Medical Laboratory Technology
Meichen Zhou, Xiaoxiao Cheng, Wenhua Zhu, Jianhua Jiang, Sijia Zhu, Xuan Wu, Meirong Liu, Qi Fang
Summary: This study investigated the expression of cGAS-STING pathway-associated proteins in patients with idiopathic inflammatory myopathy (IIM). The results showed that the expression of cGAS and STING was increased in muscle samples of IIM patients, mainly localized in vascular structures, inflammatory infiltrates, and atrophic and necrotic fibers. The activation of the cGAS-STING pathway may be responsible for myofiber atrophy and necrosis in patients with dermatomyositis and immune-mediated necrotizing myopathy.
JOURNAL OF CLINICAL LABORATORY ANALYSIS
(2022)