We report a 37-year-old woman with a 2 month history of proximal muscle weakness and extremely high creatine kinase (21,808 U/L) due to necrotizing auto-immune myositis (NAM) in association with anti-synthetase syndrome. Myositis-specific auto-immune antibody panel was positive for anti-Signal recognition particle and anti-PL-12. CT scan of the chest confirmed interstitial lung disease. Prednisolone, intravenous immunoglobulin and cyclophosphamide therapy was given with gradual improvement. This patient is notable for the unusual combination of NAM and anti-synthetase syndrome with the rare finding of two myositis-specific autoantibodies, which directed testing for associated extramuscular features and management with more aggressive immunotherapy.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1016/j.jocn.2014.04.031 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Increased Risk of Myositis-Specific and Myositis-Associated Autoantibodies After COVID-19 Pandemic and Vaccination: A Spanish Multicenter Collaborative Study.
Biomedicines
December 2024
Immunology Department and IdISSC, Hospital Clínico San Carlos, 28040 Madrid, Spain.
: Emerging evidence suggests that SARS-CoV-2 infection and vaccines may trigger autoimmune responses in predisposed individuals. Idiopathic inflammatory myopathies (IIMs) are diseases with diverse clinical manifestations, often associated with myositis autoantibodies (MAs). Diagnosing IIM is challenging due to limitations in classification criteria and diagnostic assays.
View Article and Find Full Text PDF[Immunological characteristics of patients with anti-synthetase syndrome overlap with rheumatoid arthritis].
Beijing Da Xue Xue Bao Yi Xue Ban
December 2024
Department of Rheumatology and Immunology, Peking University People' s Hospital, Beijing 100044, China.
Article Synopsis
- - The study aimed to examine the clinical and immune system characteristics of patients with anti-synthetase syndrome (ASS) who also have rheumatoid arthritis (RA) compared to those without RA.
- - Out of 104 ASS patients with arthritis, 23.1% were diagnosed with RA and displayed notable differences such as higher rates of rapidly progressive interstitial lung disease, more tender and swollen joints, and increased bone erosion compared to the non-RA group.
- - The overlap group (ASS with RA) also had significantly altered immune cell profiles, including decreased regulatory T cells and B cells, as well as higher levels of certain inflammatory markers, leading to a lower rate of complete clinical response.
Clinical Significance of Abnormal Serum LGALS3BP Expression in Patients with Idiopathic Inflammatory Myopathies.
J Inflamm Res
November 2024
Department of Clinical Laboratory, the First Affiliated Hospital of Guangxi Medical University, Key Laboratory of Clinical Laboratory Medicine of Guangxi Department of Education, Nanning, Guangxi, 530021, People's Republic of China.
Article Synopsis
- - The study investigates the role of LGALS3BP as a potential biomarker to differentiate between four subtypes of idiopathic inflammatory myopathies (IIM): dermatomyositis (DM), anti-synthetase syndrome (ASS), immune-mediated necrotizing myopathy (IMNM), and sporadic inclusion body myositis (sIBM), alongside healthy controls (HCs).
- - Using bioinformatics and enzyme-linked immunosorbent assay (ELISA), the researchers found that serum LGALS3BP levels varied significantly across the IIM subtypes and were linked to clinical features and inflammatory markers, particularly indicating a higher prevalence of interstitial lung disease (ILD) in patients with elevated levels.
- - The
Correction: Longitudinal assessment of reactivity and affinity profile of anti-Jo1 autoantibodies to distinct HisRS domains and a splice variant in a cohort of patients with myositis and anti-synthetase syndrome.
Arthritis Res Ther
December 2024
Division of Rheumatology, Department of Medicine, Karolinska University Hospital, Karolinska Institutet, 171 64, Solna, Stockholm, Sweden.
Rationale, objectives and design of a national prospective database for idiopathic inflammatory myopathies: the Australian Myositis Registry.
Intern Med J
January 2025
Centre for Molecular Medicine and Innovative Therapeutics, Murdoch University, Perth, Western Australia, Australia.
Article Synopsis
- IIMs are autoimmune diseases that lead to muscle inflammation and have serious health impacts, but due to their rarity and variability, understanding these conditions has been difficult.
- The Australian Myositis Registry (AMR) is a new national database that collects clinical and patient data from IIM patients in Australia to enhance research and improve outcomes.
- Launched in 2023 with 170 participants so far, the AMR aims to expand its reach and is significant as Australia’s first national registry for IIMs, providing crucial data for understanding the diseases' epidemiology and improving patient care.
Want AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!