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How We Treat ANCA-Associated Vasculitis: A Focus on the Maintenance Therapy.
J Clin Med
January 2025
University Center of Excellence on Nephrologic, Rheumatologic and Rare Diseases (ERK-Net, ERN-Reconnet and RITA-ERN Member) with Nephrology and Dialysis Unit and Center of Immuno-Rheumatology and Rare Diseases (CMID), Coordinating Center of the Interregional Network for Rare Diseases of Piedmont and Aosta Valley, San Giovanni Bosco Hub Hospital, ASL Città di Torino and University of Torino, 10154 Turin, Italy.
Recent progress has notably improved outcomes for patients with anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV), namely granulomatosis with polyangiitis and microscopic polyangiitis. Since 2021, several international scientific societies have recommended rituximab (RTX) as the preferred primary treatment for maintaining remission in AAV patients. Decisions regarding retreatment with RTX are based on individual patient risk factors for disease flare-ups and the potential consequences of such flares.
View Article and Find Full Text PDF[Clinical Analysis of 25 Cases of Acquired Hemophilia A in a Single Center].
Zhongguo Shi Yan Xue Ye Xue Za Zhi
December 2024
Department of Hematology, The Second Hospital of Hebei Medical University, Hebei Key Laboratory of Hematology, Shijiazhuang 050000, Hebei Province, China.
Objective: To explore the diagnosis and treatment of acquired hemophilia A (AHA) based on the analysis of clinical data.
Methods: A retrospective analysis was conducted on the clinical manifestations, laboratory characteristics, treatment, and outcomes of 25 patients diagnosed with AHA who were admitted to the Second Hospital of Hebei Medical University.
Results: Among all patients, 11 cases had secondary factors, including 5 cases of autoimmune diseases, 3 cases of pregnancy-related disease, 1 case of pemphigoid, 1 case of Graves' disease, and 1 case of monoclonal gammaglobulinemia of unknown significance (MGUS).
Outcome of extra-nodal follicular lymphoma affected by choice of induction chemoimmunotherapy and maintenance rituximab - real-world retrospective study.
Leuk Lymphoma
December 2024
Department of Medical Oncology, National Cancer Centre Singapore, Singapore, Singapore.
The importance of extra-nodal disease has been well recognized in follicular lymphoma, and is incorporated into various prognostic tools. However, the optimal treatment strategy for this subgroup remains unclear. In this multicenter retrospective study, we analyzed 143 patients who received either R-CHOP or Bendamustine-Rituximab (BR), with a median follow-up of 69.
View Article and Find Full Text PDFANCA-associated vasculitides (AAV) are small-vessel necrotizing vasculitides, with no or few immune deposits. They are usually associated with the presence of ANCA antibodies (AntiNeutrophil Cytoplasmic Antibody), targeted either against proteinase 3 (PR3-ANCA) or myeloperoxidase (MPO-ANCA). ANCA-associated vasculitides include granulomatosis with polyangiitis (formerly Wegener's), microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome).
View Article and Find Full Text PDFSafety, efficacy, and immunogenicity of SARS-CoV-2 mRNA vaccination in children and adult patients with rheumatic diseases: a comprehensive literature review.
Rheumatol Int
December 2024
Department of Medicine, Yong Loo Lin School of Medicine, National University of Singapore, Singapore, Singapore.
Patients with autoimmune inflammatory rheumatic diseases (AIIRD) are potentially at a higher risk of contracting the SARS-CoV-2 virus and have poorer outcomes of the infection as a result of their immunocompromised state due to the nature of the underlying autoimmune conditions and immunosuppressant use. mRNA-based vaccines provide a novel approach to establishing immunity against SARS-CoV-2. However, the implications of toll-like receptors (TLRs), type I interferon (IFN) and pro-inflammatory cytokines raise concerns on disease severity and inefficient immune response following mRNA vaccination.
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