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Effectiveness for remission maintenance rate and safety of different rituximab regimens for treating anti-neutrophil cytoplasmic antibody-associated vasculitis in Japan: a J-CANVAS study.
Immunol Med
January 2025
Department of Diabetes, Endocrinology and Clinical Immunology, Hyogo Medical University School of Medicine, Hyogo, Japan.
Rituximab (RTX) has been reported to effectively maintain remission in anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV). In this multicenter study involving 57 patients who achieved remission after 24 weeks, we evaluated the effectiveness of RTX in maintaining remission in patients with AAV. Patients were divided into three groups based on RTX administration: continuous, induction phase-only, and maintenance phase-only groups.
View Article and Find Full Text PDFHow 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 PDFWarm Autoimmune Hemolytic Anemia as a Rare Presentation of Pre-fibrotic Primary Myelofibrosis.
Cureus
December 2024
Hematology and Oncology, Olive View University of California Los Angeles (UCLA) Medical Center, Sylmar, USA.
Primary myelofibrosis (PMF) is an uncommon chronic myeloproliferative disorder that is commonly associated with Janus kinase 2 (JAK-2), calreticulin (CALR), or thrombopoietin receptor (MPL) mutations. Pre-fibrotic PMF (also known as pre-PMF or early PMF) is a subtype of PMF that is defined by a lower grade of fibrosis. In this report, we present a rare case of warm autoimmune hemolytic anemia (wAIHA) associated with pre-PMF.
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).
Successful rituximab treatment in IgG4-related coronary periarteritis: a case-based review.
Rheumatol Int
December 2024
Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo, 160-8582, Japan.
Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated disorder characterized by elevated serum IgG4 levels and the enlargement and fibrosis of organs. As a rare manifestation, coronary arteries can be affected by IgG4-RD as coronary periarteritis, leading to serious complications such as stenosis or aneurysm. Although coronary periarteritis poses a life-threatening condition, optimal treatment strategies remain unclear due to its extreme rarity.
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