Objectives: To validate the correlation between histopathological findings and quantitative magnetic resonance imaging (qMRI) fat fraction (FF) and water T2 mapping in patients with idiopathic inflammatory myopathy (IIM).
Methods: The study included 13 patients with histopathologically confirmed IIM who underwent dedicated thigh qMRI scanning within 1 month before open muscle biopsy. For the biopsied muscles, FF derived from the iterative decomposition of water and fat with echo asymmetry and least-squares estimation quantitation (IDEAL-IQ) and T2 time from T2 mapping with chemical shift selective fat saturation were measured using a machine learning software. Individual histochemical and immunohistochemical slides were evaluated using a 5-point Likert score. Inter-reader agreement and the correlation between qMRI markers and histopathological scores were analyzed.
Results: Readers showed good to perfect agreement in qMRI measurements and most histopathological scores. FF of the biopsied muscles was positively correlated with the amount of fat in histopathological slides (p = 0.031). Prolonged T2 time was associated with the degree of variation in myofiber size, inflammatory cell infiltration, and amount of connective tissues (p ≤ 0.008 for all).
Conclusions: Using the machine learning-based muscle segmentation method, a positive correlation was confirmed between qMRI biomarkers and histopathological findings of patients with IIM. This finding provides a basis for using qMRI as a non-invasive tool in the diagnostic workflow of IIM.
Relevance Statement: By using ML-based muscle segmentation, a correlation between qMRI biomarkers and histopathology was found in patients with IIM: qMRI is a potential non-invasive tool in this clinical setting.
Key Points: • Quantitative magnetic resonance imaging measurements using machine learning-based muscle segmentation have good consistency and reproductivity. • Fat fraction of idiopathic inflammatory myopathy (IIM) correlated with the amount of fat at histopathology. • Prolonged T2 time was associated with muscle inflammation in IIM.
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http://dx.doi.org/10.1186/s41747-023-00350-z | DOI Listing |
Clin Rheumatol
January 2025
Division of Pediatric Rheumatology and Nephrology, Department of Pediatrics, Ankara University Faculty of Medicine, Ankara, Turkey.
Objective: Chronic non-bacterial osteomyelitis (CNO) is a rare autoinflammatory bone disease associated with other chronic inflammatory diseases such as familial Mediterranean fever (FMF), juvenile idiopathic arthritis (JIA), spondylarthropathies, inflammatory bowel disease (IBD), and pyoderma gangrenosum. We aimed to describe the clinical and follow-up characteristics of patients with CNO and to compare findings between patients with and without comorbidities.
Methods: The clinical records of patients with CNO who were followed up in our pediatric rheumatology clinic between 2018 and 2023 were reviewed.
Turk J Pediatr
December 2024
Department of Pediatrics, Nilratan Sircar Medical College and Hospital, Kolkata, West Bengal, India.
Objectives: To evaluate the role of serum procalcitonin (PCT) as a diagnostic tool to differentiate bacterial sepsis from flare-ups during febrile episodes in children with known rheumatic disorders compared to other inflammatory markers like C-reactive protein (CRP), and erythrocyte sedimentation rate (ESR).
Methods: Previously diagnosed patients with known rheumatic disorders presenting in emergency or outpatient departments with febrile episodes were included in the study. Blood samples were collected upon admission to test for signs of infection, including serum PCT levels with routine laboratory and radiological tests.
Turk J Pediatr
December 2024
Division of Pediatric Rheumatology, Department of Pediatrics, University of Health Sciences, Ankara Bilkent City Hospital, Ankara, Türkiye.
Background: Anti-signal recognition protein (anti-SRP) myopathy is a rare idiopathic inflammatory myopathy in children. Herein, a 3-year-old patient with severe anti-SRP myopathy showing a rapidly progressive disease course is presented in order to increase the awareness of pediatricians about idiopathic inflammatory myopathies.
Case Presentation: A previously healthy 3-year-old girl presented with progressive symmetrical proximal muscle weakness that caused difficulty in climbing stairs for two months prior to evaluation, and a marked elevation of the serum creatine kinase levels.
Cureus
December 2024
Department of Orthopaedics, Tokyo Metropolitan Bokutoh Hospital, Tokyo, JPN.
Hypertrophic pachymeningitis (HP) is a rare inflammatory disease that causes the thickening of the dura mater. Its etiology is mainly classified as idiopathic or secondary, and autoimmune disease is one of the main causes of secondary HP. Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis and IgG4-related disease are common among autoimmune diseases.
View Article and Find Full Text PDFAm J Ophthalmol Case Rep
March 2025
Department of Ophthalmology, Stanley M. Truhlsen Eye Institute, University of Nebraska Medical Center, Omaha, NE, USA.
Purpose: To describe a rare case of presumed bilateral acute idiopathic maculopathy (AIM) in a pediatric patient.
Observation: An 11-year-old male was evaluated for a "fuzzy Dorito-shaped" spot in the central vision of his right eye (OD) that started 3 days before presenting to our clinic. On examination, best-corrected visual acuity (BCVA) was counting fingers at 5 feet OD, and 20/25 in the left eye (OS).
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