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Objectives: The clinicopathological features of immune-mediated necrotizing myopathy (IMNM) sometimes mimic muscular dystrophy, complicating accurate diagnosis. The European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) classification criteria of idiopathic inflammatory myopathies (IIMs) are superior in terms of sensitivity and specificity; however, the sensitivity is reported to be relatively low in IMNM. We examined the clinicopathological characteristics and the prognoses of anti-3-hydroxy-3-methylglutaryl-CoA reductase (HMGCR) antibody-positive and anti-signal recognition particle (SRP) antibody-positive cases that do not satisfy the EULAR/ACR classification criteria.
Methods: Seventy-one anti-HMGCR antibody-positive cases and 94 anti-SRP antibody-positive cases were included. We compared the clinicopathological characteristics of the groups that were not classified as IIMs (
Results: Nineteen anti-HMGCR antibody-positive cases (26%) and 19 anti-SRP antibody-positive cases (20%) were classified as the
Conclusions: Some IMNM cases showing a clinicopathologically mild phenotype and a benign course cannot be classified as IIMs by the EULAR/ACR classification criteria, and antibody testing is crucial for accurate diagnosis.
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http://dx.doi.org/10.1093/rheumatology/keaf183 | DOI Listing |
Front Immunol
August 2025
Department of Neurology, Zhongnan Hospital of Wuhan University, Wuhan, Hubei, China.
Autoimmune encephalitis (AE) is a heterogeneous disorder mediated by autoantibodies targeting neuronal or glial antigens, with anti-NMDAR encephalitis being the most common subtype, while cases with dual antibody positivity remain exceedingly rare. Standard treatment involves stepwise immunotherapy, but refractory cases often require advanced therapies. This study presents the first reported case of dual anti-NMDAR and anti-GFAP antibody-positive refractory AE in a 24-year-old female who failed first-line treatments (steroids, IVIG) and ovarian teratoma resection.
View Article and Find Full Text PDFCureus
July 2025
Division of Rheumatology, Department of Medicine, Jichi Medical University Saitama Medical Center, Saitama, JPN.
We report two cases of anti-melanoma differentiation-associated gene 5 (MDA5) antibody-positive dermatomyositis (DM) that recurred after long-term remission. The first case involved a 41-year-old Japanese woman, and the other involved a 31-year-old Chinese woman. Both patients were initially treated with prednisolone, tacrolimus, and low-dose intravenous cyclophosphamide.
View Article and Find Full Text PDFZhonghua Er Ke Za Zhi
September 2025
Department of Rheumatology and Immunology, Capital Center for Children's Health, Capital Medical University, Capital Institute of Pediatrics, Beijing 100020, China.
To analyze the clinical characteristics of anti-melanoma differentiation associated gene 5 (MDA5) antibody-positive juvenile dermatomyositis (JDM) patients. A retrospective case-control study was conducted. The positive group included 18 children with anti-MDA5 antibody-positive JDM who were admitted to the Department of Rheumatology and Immunology at Capital Center for Children's Health with Capital Medical University between January 2016 and January 2023.
View Article and Find Full Text PDFPediatr Neurol
October 2025
Department of Pediatrics, University of Texas Southwestern Medical Center, Dallas, Texas; Department of Neurology, University of Texas Southwestern Medical Center, Dallas, Texas.
Background: Acute disseminated encephalomyelitis (ADEM) typically presents as a monophasic demyelinating disorder associated with multifocal neurological symptoms and encephalopathy in children. Myelin oligodendrocyte glycoprotein antibody (MOG-IgG) is detected in a subset of patients and may be linked to recurrence risk. This study evaluated the clinical, radiologic, and optic coherence tomographic (OCT) features of children with ADEM based on MOG-IgG serostatus.
View Article and Find Full Text PDFAdv Sci (Weinh)
August 2025
Department of Neurology, the First Affiliated Hospital of Zhengzhou University, Zhengzhou University, Zhengzhou, 450000, China.
The acute-remitting course (the time interval from remission to the next relapse) is a distinct signature of neuromyelitis optica spectrum disorder (NMOSD), which harms patients and perplexes physicians. However, clinically actionable biomarkers to anticipate NMOSD relapse timing are unavailable. Recently, it is found that Cuproptosis-related genes would have an impact on the acute-remitting course in patients with aquaporin-4 (AQP4)-immunoglobulin G antibody positive NMOSD.
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