A 78-year-old woman with a history of intractable otitis media presented with a fever, hearing impairment, thigh pain, and a skin rash. She had renal dysfunction, positive myeloperoxidase-antineutrophil cytoplasmic autoantibody, otitis media, and multiple nodules in both lungs. She was diagnosed with granulomatosis with polyangiitis, crescentic glomerulonephritis, and interstitial nephritis, which was confirmed in a kidney biopsy specimen. Induction therapy with rituximab and avacopan without glucocorticoids promptly resolved her fever and thigh pain and improved her auditory acuity and nodule in the right lung. The patient experienced no adverse effects with rituximab or avacopan.
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http://dx.doi.org/10.2169/internalmedicine.3072-23 | DOI Listing |
Expert Rev Clin Pharmacol
November 2024
Department of Nephrology, 1st Faculty of Medicine, Charles University and General University Hospital, Prague, Czech Republic.
Introduction: ANCA-associated vasculitis (AAV) is a rare, life-threatening disease which may result in serious pulmonary and kidney damage. Cyclophosphamide or rituximab and high-dose glucocorticoids significantly improved patient outcomes, but at the expense of severe complications. Moreover, many patients still relapse and bear a significant burden of both disease- and treatment-related complications.
View Article and Find Full Text PDFEur J Ophthalmol
October 2024
Uveitis and Scleritis Service, Moorfields Eye Hospital NHS Foundation Trust, London, UK.
Purpose: Avacopan is a novel C5a receptor inhibitor which was recently licensed for treatment of severe granulomatosis with polyangiitis (GPA) in the European Union and the United Kingdom. To the best of our knowledge, this is the first described case on initial ophthalmic outcomes in a patient with severe GPA and concurrent refractory scleritis treated with avacopan.
Case Description: We present a case of de novo scleritis in a 77-year-old male with a background of retinitis pigmentosa with Argus II implant in situ.
Intern Med
October 2024
Department of Immunology and Rheumatology, Division of Advanced Preventive Medical Sciences, Nagasaki University Graduate School of Biomedical Sciences, Japan.
We herein report three cases of microscopic polyangiitis (MPA). Two patients were administered avacopan in combination with glucocorticoid (GC), whereas one patient was treated with avacopan monotherapy; none of the patients were co-administered either rituximab or cyclophosphamide. The doses of GC were successfully reduced after the introduction of avacopan in the two patients, and the serum C-reactive protein levels decreased in the patient treated with avacopan monotherapy.
View Article and Find Full Text PDFJACC Case Rep
September 2024
Department of Cardiology, Trinity Health Ann Arbor, Ann Arbor, Michigan, USA.
CEN Case Rep
September 2024
Department of Nephrology, Kameda Medical Center, 929 Higashi-cho, Kamogawa, Chiba, 296-8602, Japan.
The prognosis of anti-glomerular basement membrane (anti-GBM) nephritis, often accompanied by the presence of antineutrophil cytoplasmic antibodies (ANCA), is poor, and even with aggressive therapeutic approaches, kidney replacement therapy (KRT) is typically required. Here, we present a case of necrotizing crescentic glomerulonephritis in a patient double-seropositive for anti-GBM antibodies and ANCA who successfully achieved dialysis independence following aggressive treatment, including avacopan. The patient was a 77-year-old woman with rapidly progressive glomerulonephritis and double seropositivity for myeloperoxidase-ANCA and anti-GBM antibodies.
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