A patient affected by a "limb-girdl syndrome" type of polymyositis shows clinical symptoms and signs of progressively worsening dilatative myocardiopathy. The diagnosis was based on well established criteria, such as the following: EF < 45-55%, left ventricular telediastolic diameter higher than 2.7 cm/mq, and normal wall thickness (L. Mestroni 1994, W.H. Abelmann 1989, et al.). The above-mentioned clinical picture was further complicated by amiodarone induced thyrotoxicosis. After the case presentation, and having exhaustively and critically revised the existing literature on polymyositis associated dilatative myocardiopathy and on eventual joint pathogenetic mechanisms, the essay in question focuses on the discussion of the clinical data brought forth in the first part.
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RMD Open
January 2025
TYKS laboratories, Clinical Microbiology, Turku University Hospital, Turku, Finland.
Objectives: We assessed the positive predictive value (PPV) of 17 myositis antibodies for having a diagnosis of myositis and other myositis-spectrum conditions (interstitial lung disease (ILD), connective tissue diseases (CTD), malignancy) and evaluated the impact of semiquantitative classification and antibody overlap on the PPVs.
Materials And Methods: We retrospectively identified 1068 individuals ≥18 years who tested positive for ≥1 antibody in the EUROLINE myositis line blot assay or positive for anti-3-hydroxy-3-methylglutaryl-CoA reductase (HMGCR) in an ELISA-based test between 2015 and 2020 in 15 out of the 20 hospital districts in Finland. We extracted clinical diagnoses from the Care Register for Health Care between January 2013 and June 2022.
Cureus
December 2024
Cardiology, King Khalid University Hospital, Riyadh, SAU.
Troponin is a highly specific biomarker for myocardial injury. It plays a critical role in the diagnosis of acute coronary syndrome (ACS). However, elevated troponin levels are not exclusively due to cardiac ischemia and may be observed in many non-cardiac conditions, including inflammatory myopathies.
View Article and Find Full Text PDFActa Dermatovenerol Croat
November 2024
Prof. Marija Jelušić, MD, PhD, Department of Paediatrics, University of Zagreb, School of Medicine, Division of Clinical Immunology, Rheumatology and Allergology, Centre of Reference for Paediatric and Adolescent Rheumatology of Ministry of Health of the Republic Croatia, University Hospital Centre Zagreb, Kispaticeva 12, 10 000 Zagreb, Croatia;
Juvenile dermatomyositis with emphasized vasculopathy is rare, but the most severe form of the disease, with a poor prognosis with relapsing and chronic course or, in some cases, lethal outcome. We present a case of a 19-year-old Caucasian female, who developed severe acute juvenile dermatomyositis with emphasized multisystem vasculopathy, including retinal vasculopathy and maculopathy (cotton-wool spots, retinal hemorrhages, macular edema) at the age of 8. Due to no response to standard treatment protocols and rapid worsening of clinical symptoms and laboratory findings, a TNF inhibitor (infliximab) was introduced after the third week of treatment resulting in complete normalisation of muscle enzyme levels and complete resolution of eye changes within the next 2 weeks with a gradual general recovery.
View Article and Find Full Text PDFLancet
January 2025
Department of Dermatology, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, USA. Electronic address:
Background: Dermatomyositis is a chronic autoimmune disease with distinctive cutaneous eruptions and muscle weakness, and the pathophysiology is characterised by type I interferon (IFN) dysregulation. This study aims to assess the efficacy, safety, and target engagement of dazukibart, a potent, selective, humanised IgG1 neutralising monoclonal antibody directed against IFNβ, in adults with moderate-to-severe dermatomyositis.
Methods: This multicentre, double-blind, randomised, placebo-controlled, phase 2 trial was conducted at 25 university-based hospitals and outpatient sites in Germany, Hungary, Poland, Spain, and the USA.
Arthritis Res Ther
January 2025
Rheumatology Department, Hospital Universitario Reina Sofía, Universidad de Córdoba, Instituto Maimónides de Investigación Biomédica de Córdoba (IMIBIC), Córdoba, Spain.
Objective: To evaluate the main outcomes of disease activity and their association with other measures of activity, damage, and quality of life in patients with idiopathic inflammatory myopathy (IIM) according to time since diagnosis and positivity to antisynthetase autoantibodies (ASAs).
Methods: Cross-sectional multicenter study within the Spanish Myo-Spain registry. Cases were classified as incident (≤ 12 months since diagnosis) and prevalent.
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