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Anti-SRP myositis: a diagnostic and therapeutic challenge.
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.
Profiling of Anti-Signal-Recognition Particle Antibodies and Clinical Characteristics in South Korean Patients With Immune-Mediated Necrotizing Myopathy.
J Clin Neurol
January 2025
Department of Neurology, Gangnam Severance Hospital, Yonsei University College of Medicine, Seoul, Korea.
Background And Purpose: This study evaluated the diagnostic utility of an anti-signal-recognition particle 54 (anti-SRP54) antibody-based enzyme-linked immunosorbent assay (ELISA) as well as the clinical, serological, and pathological characteristics of patients with SRP immune-mediated necrotizing myopathy (IMNM).
Methods: We evaluated 87 patients with idiopathic inflammatory myopathy and 107 healthy participants between January 2002 and December 2023. The sensitivity and specificity of the ELISA for anti-SRP54 antibodies were assessed, and the clinical profiles of patients with anti-SRP54 antibodies were determined.
Successful treatment of anti-signal recognition particle antibody-positive myositis with intravenous cyclophosphamide: A case report.
Fukushima J Med Sci
December 2024
Department of Rheumatology, Fukushima Medical University School of Medicine.
Myositis-specific autoantibodies play an important role on the disease phenotype of idiopathic inflammatory myopathies (IIMs). Anti-signal recognition particle (SRP) antibody-positive patients with IIMs may present with severe myopathy and highly elevated serum creatine kinase levels. These patients are often resistant to immunosuppressive therapy, but there is no established treatment strategy.
View Article and Find Full Text PDFAnti-SRP Antibodies and Myocarditis in Systemic Sclerosis Overlap Syndrome with Immune-Mediated Necrotizing Myositis (IMNM).
Medicina (Kaunas)
October 2024
Faculty of Medicine, "Carol Davila" University of Medicine and Pharmacy, 020021 Bucharest, Romania.
Article Synopsis
- - Overlap syndrome of systemic sclerosis and idiopathic inflammatory myopathies is becoming more common, but its link with immune-mediated necrotizing myositis is not well-documented, requiring careful differentiation for proper treatment and prognosis.
- - Anti-signal recognition particle (anti-SRP) antibodies are crucial in diagnosing immune-mediated necrotizing myopathy, causing muscle damage and weakness, while their relationship with cardiac issues remains inconclusive, complicating potential management.
- - Diagnosing heart complications like myocarditis in these patients is challenging, often relying on cardiac imaging rather than biopsies, and this paper explores the issues of accurately diagnosing and treating diseases related to collagen and anti-SRP antibodies.
Comprehensive Enteroviral Serology Links Infection and Anti-Melanoma Differentiation-Associated Protein 5 Dermatomyositis.
ACR Open Rheumatol
January 2025
Johns Hopkins University School of Medicine, Baltimore, Maryland.
Objective: Idiopathic inflammatory myopathies (IIMs) are a group of heterogeneous, systemic autoimmune diseases characterized by specific clinical features and, frequently, skeletal muscle inflammation. Specific subtypes of IIMs can be characterized by myositis-specific autoantibodies and are associated with distinct clinical phenotypes. Here, we focus on anti-melanoma differentiation-associated protein 5 (MDA5)-positive myositis and anti-signal recognition particle (SRP)-positive myositis, both of which exhibit seasonality but lack known environmental triggers.
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