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| http://dx.doi.org/10.1016/j.reumae.2022.06.001 | DOI Listing |
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Background and Clinical Features of a Unique and Mysterious Autoinflammatory Disease, Schnitzler Syndrome.
Int J Mol Sci
January 2025
Immunology Division, Department of Internal Medicine and Hematology, Semmelweis University, 1088 Budapest, Hungary.
Schnitzler syndrome is a unique autoinflammatory disease, of which 747 cases have been described worldwide to date. The main features of the syndrome are a triad of recurrent urticaria, monoclonal IgM gammopathy, systemic inflammation associated with recurrent fever, joint and bone pain, and atypical bone remodeling (osteosclerosis). The abnormal activation of the NLRP3 inflammasome produces IL-1, which drives the disease pathology, but it also involves IL-6 and IL-18.
View Article and Find Full Text PDFCardiovascular Risk Biomarkers in Women with and Without Polycystic Ovary Syndrome.
Biomolecules
December 2024
Research Department, Royal College of Surgeons of Ireland, Adliya, Busaiteen 15503, Bahrain.
Objective: Polycystic ovary syndrome (PCOS) is a prevalent metabolic disorder with an increased risk for cardiovascular disease (CVD) that is enhanced by obesity. This study sought to determine whether a panel of cardiovascular risk proteins (CVRPs) would be dysregulated in overweight/obese PCOS patients, highlighting potential biomarkers for CVD in PCOS.
Methods: In this exploratory cross-sectional study, plasma levels of 54 CVRPs were analyzed in women with PCOS (n = 147) and controls (n = 97).
Off-label use of biologics in urticarial vasculitis: a European retrospective cohort study.
Rheumatology (Oxford)
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Department of internal medicine, CHU Cochin, AP-HP, Paris Cité University, Paris, F-75006, France.
Objectives: Urticarial vasculitis (UV) is characterized by atypical urticarial lesions and leukocytoclastic vasculitis, sometimes with extracutaneous manifestations. First-line treatment is based on colchicine, hydroxychloroquine, dapsone or low-dose glucocorticoids. In refractory forms, the use of biologics has been anecdotally described as potentially effective.
View Article and Find Full Text PDFManagement of chimeric antigen receptor T-cell-related toxicity of a patient affected by cytokine release syndrome and immune effector cell-associated neurotoxicity syndrome, followed by an intestinal perforation: a case report.
J Med Case Rep
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Director of Hospital Pharmacy, Santa Croce e Carle Hospital, Cuneo, Italy.
Background: Mantle cell lymphoma is a diverse B-cell lymphoma with varying clinical behaviors. Treating relapsed or refractory mantle cell lymphoma is challenging, with Bruton's tyrosine kinase inhibitors proving effective but not curative. Post-Bruton's tyrosine kinase inhibitor failure, the prognosis remains unfavorable.
View Article and Find Full Text PDFBackground: Anakinra is an interleukin-1 receptor antagonist (IL-1Ra). Since IL-1 has been shown to play a key role in the etiology of different autoinflammatory diseases, blocking its pathway has become an important therapeutic target, even in neonates.
Aims: We aimed to report our experience in using anakinra to treat specific neonatal inflammatory conditions.
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