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Eur Respir J
January 2025
Department of Rheumatology, Renji Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China
Objective: To compare the effectiveness and safety of tofacitinib (TOF) calcineurin inhibitor (CNI) as initial immunosuppressive regimen for anti-melanoma differentiation-associated gene 5-positive dermatomyositis with interstitial lung disease (MDA5+DM-ILD).
Methods: Adult Chinese patients with newly-diagnosed MDA5+DM-ILD (ILD course<3 months) from five tertiary referral centres between April 2014 and January 2023 were included for this retrospective cohort study. The primary effectiveness endpoint was lung transplantation-free survival within 1 year.
BMC Pulm Med
January 2025
Department of Key Laboratory of Ningxia Stem Cell and Regenerative Medicine, Institute of Medical Sciences, Department of Pulmonary and Critical Care Medicine, General Hospital of Ningxia Medical University, Yinchuan, Ningxia, 750004, China.
Background: In this study, we aimed to explore the association between baseline and early changes in the neutrophil-to-lymphocyte ratio (NLR) and the 30-day mortality rate in patients having anti-melanoma differentiation-associated gene 5 (MDA5)-positive dermatomyositis with interstitial lung disease (DM-ILD).
Methods: Overall, 263 patients with anti-MDA5 DM-ILD from four centers in China were analyzed. Multivariate logistic regression analysis was used to evaluate the impact of baseline NLR on the 30-day mortality rate in patients with anti-MDA5-positive DM-ILD.
Int J Rheum Dis
January 2025
Department of Medicine, McGill University, Montreal, Canada.
Acta 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.
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