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Eosinophilic Granulomatosis With Polyangiitis: A Real-World Study of Biologics and Sinus Surgery Use for Managing Nasal Polyposis.
Int Forum Allergy Rhinol
January 2025
Department of Otorhinolaryngology, Mayo Clinic in Arizona, Scottsdale, Arizona, USA.
Background/aim: Eosinophilic granulomatosis with polyangiitis (EGPA) is a multisystemic disease associated with nasal polyposis. Multiple biologics are used for managing EGPA, including some approved for nasal polyps (NP). This study investigated real-world biologic prescription patterns for EGPA and their impact on NP and endoscopic sinus surgery (ESS) use.
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 PDFDifferences in Phenotypes, Treatments, and Outcomes of ANCA-associated Vasculitis across Europe, Japan, and the United States in 2020.
Rheumatology (Oxford)
January 2025
Inflammation and Immunology, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Kyoto, Japan.
Objectives: To clarify the differences in clinical phenotypes, therapeutic patterns, and outcomes of patients with granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) across geographic regions using a multinational cohort.
Methods: Data were collected from patients with newly diagnosed or relapsing GPA or MPA in Europe, Japan, and the United States (US) from January to July 2020. The composite outcome of kidney failure and/or death within 52 weeks after treatment was evaluated, and the hazard ratios across the regions were estimated using the Cox proportional hazard model.
Granulomatosis With Polyangiitis in a Young Lady: Challenges in Achieving Remission.
Cureus
December 2024
Ophthalmology, Universiti Kebangsaan Malaysia Medical Centre (UKMMC), Kuala Lumpur, MYS.
Granulomatosis with polyangiitis (GPA) is a subtype of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) that commonly requires aggressive immunosuppression to achieve remission. We present a case of a young Malay lady with recurrent episodes of ANCA-positive nodular anterior scleritis who responded poorly to topical and systemic corticosteroids and relapsed while on methotrexate. A year later, she had epistaxis, and a sino-nasal biopsy confirmed granulomatous vasculitis.
View Article and Find Full Text PDFRemission induction therapies and long-term outcomes in granulomatosis with polyangiitis and microscopic polyangiitis: real-world data from a European cohort.
Rheumatol Int
December 2024
Department of Nephrology and Clinical Immunology, RWTH Aachen University Hospital, Pauwelsstraße 30, 52074, Aachen, Germany.
To explore disease characteristics, renal involvement and induction treatment strategies over the last decades and evaluate relapse rates and renal outcomes in ANCA-associated vasculitides (AAV). We retrospectively analyzed remission, relapse rates and the occurrence of the composite endpoint (comprising death and renal failure) in newly diagnosed AAV cases in four tertial referral centers in Germany and Switzerland diagnosed between 1999 and 2022. Hazard ratios were computed by Cox proportional hazard and Kaplan-Meier curves were plotted to compare therapeutic strategies after propensity-matching.
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