Background: Granulomatosis with polyangiitis is a systemic anti-neutrophil cytoplasmic antibody-associated vasculitides (AAVs), mainly involving the respiratory tract and renal system. Treatment by Rituximab as a next-generation therapy in ANCA-associated vasculitis is associated with promising outcomes in GPA patients. Despite symptoms improvements, disease recurrence and drug reaction are a challenging topic nowadays.
Objectives: In this study, we examined six GPA patients who were confirmed to have paradoxical reactions to rituximab and then described how to control their symptoms.
Methods: In this study, all the systemic GPA patients (diagnosed based on ACR/EULAR criteria) who received RTX in Amir-Allam hospital were monitored for any sign of disease exacerbation up to 3 months after RTX exposure.
Results: From 78 GPA-diagnosed patients, six, including one man and five women with the mean age of 37.3 ± 13.8, were identified for exacerbation after RTX administration.
Conclusion: According to our observation, it could be recommended not to deprive the patient of the benefits of RTX treatment due to the early patient's possible complications.
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Highlights from the breakout session: optimising new treatments.
Rheumatology (Oxford)
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Vasculitis and Lupus Clinic, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK.
This session presented emerging realworld evidence on novel glucocorticoid (GC) sparing therapies for ANCA-associated vasculitis (AAV). It covered the first-in-class oral C5a receptor antagonist avacopan for severe granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA), and antiinterleukin-5 therapies in the management of eosinophilic granulomatosis with polyangiitis (EGPA). Avacopan was approved by the US Food and Drug Administration (FDA) and European Medicines Agency (EMA) for the treatment of AAV in 2021, following the phase 3 ADVOCAT E trial comparing oral avacopan to an oral GC taper for GPA and MPA.
View Article and Find Full Text PDFCharacterizing the Cellular Constituents of Proximal Airway Disease in Granulomatosis With Polyangiitis.
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March 2025
Department of Otolaryngology-Head and Neck Surgery, Vanderbilt University Medical Center, Nashville, Tennessee, USA.
Objective: Granulomatosis with polyangiitis (GPA) is a rare multisystem autoimmune vasculitis. 10-20% of patients suffer life-threatening obstruction of their proximal airways. Although progress has been made in the treatment of systemic disease, ameliorating airway disease in GPA remains an unmet need arising from limited understanding of disease pathogenesis.
View Article and Find Full Text PDFGranulomatosis With Polyangiitis Mimicking Giant Cell Arteritis.
Cureus
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Internal Medicine, Royal Blackburn Hospital, Blackburn, GBR.
Granulomatosis with polyangiitis (GPA) is a rare auto-immune ANCA-associated small-vessel vasculitis characterized by necrotizing granulomatous inflammation, primarily affecting the sinuses, respiratory tract, and kidneys. Early diagnosis and treatment are crucial for improving patient outcomes and preventing rapidly progressive renal failure. We present a case of a 66-year-old woman presenting to the emergency department (ED) with a two-week history of productive cough, nausea, fevers, and headache.
View Article and Find Full Text PDFAssociation of C4d with disease activity in anti-neutrophil cytoplasmic antibody-associated vasculitis: evidence for classical/lectin complement pathway activation.
Arthritis Res Ther
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Front Med (Lausanne)
February 2025
Department of Neurology, Seoul National University College of Medicine, Seoul, Republic of Korea.
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