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Clinical features and factors associated with outcomes of antibody-negative autoimmune encephalitis in patients requiring intensive care.
Crit Care
January 2025
Department of Neuro-Intensive Care Unit, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan, China.
Background And Objectives: Antibody-negative autoimmune encephalitis (AE) is a form of encephalitis characterized by the absence of detectable autoimmune antibodies, despite immunological evidence. However, data on management of patients with antibody-negative AE in the intensive care unit (ICU) are limited. This study aimed to explore the characteristics and subtypes of antibody-negative AE, assess the effects of immunotherapy, and identify factors independently associated with poor functional outcomes in patients requiring intensive care.
View Article and Find Full Text PDFPulmonary Arterial Hypertension Incidence in Scleroderma Patients Treated with Bosentan for Digital Ulcers: Evidence from the Italian SPRING Registry.
J Rheumatol
January 2025
Florenzo Iannone, Rheumatology Unit - Department of Precision and Regenerative Medicine of Jonian Area University of Bari, Bari, Italy.
Objective: Bosentan (BOS) is approved for treating pulmonary arterial hypertension (PAH) and preventing digital ulcers (DU) in systemic sclerosis (SSc). Our study aimed to evaluate whether BOS prescribed for DU could reduce the incidence of PAH in a large SSc cohort from the SPRING registry.
Methods: Patients with SSc from the SPRING registry, meeting ACR/EULAR 2013 classification criteria with data on PAH onset, DU status, BOS exposure, and at least a one-year follow-up between 2015 and 2020, and no known PAH at baseline were included.
Incidence, characteristics, and comorbidities of a complete unselected Danish cohort of patients with thymic epithelial tumors.
Acta Oncol
January 2025
Department of Oncology, Copenhagen University Hospital, Copenhagen, Denmark.
Background And Purpose: We report the incidence, characteristics, and comorbidities of the complete unselected Danish cohort of patients with thymic epitheliums (TETs), which may serve as evidence for guiding treatment, surveillance, and counselling of TET patients.
Patients And Methods: All patients diagnosed with TETs from January 1st, 2015, to December 31st, 2020, were identified using the Danish Pathology Data Registry. Data on patient characteristics, comorbidities, and tumor histology were collected from electronic medical records available for all patients.
Hypomorphic RAG2 Deficiency Promotes Selection of Self-Reactive B Cells.
J Clin Immunol
January 2025
Center for Immunity and Immunotherapies, Seattle Children's Research Institute, Seattle, WA, USA.
Reduced function or hypomorphic variants in recombination-activating genes (RAG) 1 or 2 result in a broad clinical phenotype including common variable immunodeficiency (CVID) and even adult-onset disease. Milder RAG variants are less characterized. Here we describe the longitudinal course of a milder combined RAG deficiency in 3 of 7 siblings sharing the same RAG2 mutations over a 50-year study.
View Article and Find Full Text PDFPatterns of Tau pathology in patients with anti-IgLON5 disease visualized by Florzolotau (18F) PET.
J Neurol
January 2025
Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, 100730, China.
Background: Anti-IgLON5 disease is a rare autoimmune neurological disorder with prominent Tau protein deposits in the brainstem and hypothalamus. The aim of this study was to visualize the in vivo distribution patterns of Tau protein in patients with anti-IgLON5 disease using the second-generation Tau PET tracer, Florzolotau (18F) PET imaging.
Methods: Patients diagnosed with anti-IgLON5 disease were enrolled consecutively.
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