Tavneos (avacopan) / Kissei, Amgen, Otsuka, CSL Behring - LARVOL DELTA

A Presentation of Strawberry Gingivitis as the Initial Presenting Symptom of Recurrence of Granulomatosis With Polyangiitis. (PubMed, Cureus) - "The patient's symptoms had been under control since initial induction therapy with IV prednisone, cyclophosphamide, and two doses of rituximab, and he was then continued on a maintenance therapy regimen of azathioprine for two years symptom-free. His new maintenance therapy regimen included avacopan, azathioprine, and prednisone. Since initiating this therapy, he has remained symptom-free with no new manifestations or signs of relapse."

Journal • Dental Disorders • Rare Diseases • Rheumatology • Vasculitis

Avacopan-induced liver injury: an uncommon but emerging concern? (PubMed, Clin Exp Rheumatol) - No abstract available

Journal • Hepatology • Liver Failure

3-months short course of avacopan in ANCA vasculitis with severe clinical involvement. A real-life experience. (ERA 2025) - No abstract available

Clinical • ANCA Vasculitis • Vasculitis

AvacoStar, a real-world evidence study of avacopan in ANCA-associated vasculitis – baseline characteristics of initial patients (ERA 2025) - No abstract available

Clinical • HEOR • Real-world • Real-world evidence • ANCA Vasculitis • Vasculitis

Three years of avacopan treatment in patients with ANCA-associated vasculitis (ERA 2025) - No abstract available

Clinical • ANCA Vasculitis • Vasculitis

Use of avacopan-based therapy among adults with severe active ANCA-associated vasculitis (AAV): a cross-sectional survey of physicians in Germany and the UK (ERA 2025) - No abstract available

Clinical • ANCA Vasculitis • Vasculitis

General, nervous system, eye, and skin involvement in the phase 3 trial of avacopan for the treatment of antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) (UKKW 2025) - No abstract available

P3 data • ANCA Vasculitis • Vasculitis

Drug-induced liver injury due to avacopan improved by mycophenolate mofetil: A case report. (PubMed, Medicine (Baltimore)) - "MMF is effective in treating DILI caused by avacopan."

Journal • ANCA Vasculitis • Anorexia • Fatigue • Hepatology • Liver Failure • Rare Diseases • Vasculitis

Successful treatment of MPO-ANCA positive crescentic IgA nephropathy/IgA vasculitis with nephritis potentially triggered by a COVID-19 vaccine in a young adult female using corticosteroids, rituximab, and avacopan. (PubMed, CEN Case Rep) - "This case emphasizes the need for a reliable laboratory method to detect pathogenic ANCA to guide both induction and maintenance therapy. Further investigation into the effectiveness of the ANCA-associated glomerulonephritis treatment protocol including corticosteroids, RTX, and avacopan in managing crescentic IgAN/IgAVN could offer valuable insights into improving patient care."

Journal • ANCA Vasculitis • Anorexia • Glomerulonephritis • IgA Nephropathy • Infectious Disease • Lupus Nephritis • Nephrology • Novel Coronavirus Disease • Pain • Renal Disease • Vasculitis • MPO

Safety of Avacopan for the Treatment of Antineutrophil Cytoplasmic Antibody-Associated Vasculitis: Combined Data From Three Clinical Trials. (PubMed, ACR Open Rheumatol) - P2, P3 | "In clinical trials of GPA or MPA, use of avacopan was associated with fewer AEs, SAEs, and WBC count reductions and fewer infections than non-avacopan treatment. Safety data support the use of avacopan in patients with GPA or MPA."

Journal • ANCA Vasculitis • Infectious Disease • Rare Diseases • Vasculitis

CARDIAC CONDUCTION ABNORMALITIES IN MICROSCOPIC POLYANGIITIS: A REVERSIBLE YET CRITICAL MANIFESTATION - Minh Tran (ACC 2025) - "The patient was discharged on avacopan, rituximab, and prednisone. This case highlights the complex nature of MPA, including multi-systemic involvement with notable cardiac conduction abnormalities. This case highlights the complex nature of MPA, including multi-systemic involvement with notable cardiac conduction abnormalities. The AV block, likely secondary to granulomatous inflammation at the AV node, resolved with early immunosuppressive treatment. Awareness of cardiac involvement in MPA is critical, as timely intervention can prevent permanent pacing and improve outcomes."

Anemia • Bronchiectasis • Cardiomyopathy • Cardiovascular • Coronary Artery Disease • Diabetes • Glomerulonephritis • Hematological Disorders • Hypertension • Hypotension • Immunology • Inflammation • Interstitial Lung Disease • Lupus Nephritis • Metabolic Disorders • Nephrology • Pulmonary Disease • Renal Disease • Respiratory Diseases • Vasculitis

CORONARY ARTERY VASOSPASM AS AN INITIAL PRESENTATION OF GRANULOMATOSIS WITH POLYANGIITIS - Eshaan Parikh (ACC 2025) - "He received 2 doses of rituximab and was started on avacopan.Decision-making: Although the patient had an ECG on initial admission with ST-segment elevations in inferior leads, he had a negative troponin I level and an unremarkable cardiac catheterization. Even without classic symptoms, it is crucial to consider GPA high on the differential diagnosis when there is multi-organ involvement coupled with ANCA-positivity. Timely diagnostic workup and treatment mitigate disease complications for patients."

Acute Kidney Injury • Chronic Kidney Disease • Glomerulonephritis • Lupus Nephritis • Musculoskeletal Pain • Nephrology • Rare Diseases • Renal Disease • Vasculitis

Glucocorticoid free remission in maintenance treatment of anca associated vasculitis (AAV) after discontinuation of avacopan - a unicentre non-interventional prospective observational study of 34 patients over 18 months (EULAR 2025) - No abstract available

Clinical • Observational data • ANCA Vasculitis • Vasculitis

Efficacy and Safety of Avacopan for Treatment of Patients with ANCA-Associated Vasculitis Receiving Cyclophosphamide (EULAR 2025) - No abstract available

Clinical • ANCA Vasculitis • Vasculitis

Interim Report on Post-Marketing Surveillance of avacopan, a C5a Receptor antagonist, for Microscopic Polyangiitis and granulomatosis with polyangiitis-1st Report- (EULAR 2025) - No abstract available

P4 data • Rare Diseases • Vasculitis

Early Access Program (EAP) of Avacopan for the Treatment of ANCA-Associated Vasculitis (AAV): The Italian Multicenter Prospective Real-World Experience Beyond 52 Weeks (EULAR 2025) - No abstract available

Clinical • Real-world • Real-world evidence • ANCA Vasculitis • Vasculitis

Comparing avacopan use in patients with GPA during induction phase with Rituximab and prednisone: A retrospective study using the TriNetx database (EULAR 2025) - No abstract available

Retrospective data

AvacoStar, a real-world evidence study of avacopan in ANCA-associated vasculitis – baseline characteristics of initial patients (EULAR 2025) - No abstract available

Clinical • HEOR • Real-world • Real-world evidence • ANCA Vasculitis • Vasculitis

Three years of avacopan treatment in patients with ANCA-associated vasculitis (EULAR 2025) - No abstract available

Clinical • ANCA Vasculitis • Vasculitis

Pediatric ANCA-Associated Vasculitis Study of Avacopan Efficacy, Pharmacokinetics, and Safety: A Phase 3 Trial in Progress (EULAR 2025) - No abstract available

Clinical • P3 data • PK/PD data • ANCA Vasculitis • Pediatrics • Vasculitis

The Crosstalk Between NETs and the Complement Cascade: An Overview in Nephrological Autoimmune Disease. (PubMed, Int J Mol Sci) - "We here present the interaction between complement components and NETs in specific autoimmune diseases that mostly affect the kidney, such as systemic lupus erythematosus, Antineutrophilic Cytoplasmic Antibody (ANCA)-associated vasculitis and antiphospholipid syndrome. The mechanisms reported here may represent specific targets for the development of possible therapeutic strategies."

Journal • Review • ANCA Vasculitis • Genetic Disorders • Hematological Disorders • Immunology • Infectious Disease • Inflammation • Inflammatory Arthritis • Lupus • Nephrology • Systemic Lupus Erythematosus • Vasculitis

Assessing the Effect of Avacopan on Increasing ANCA Titres During Remission Maintenance in ANCA-Associated Vasculitis. (PubMed, Int J Rheum Dis) - No abstract available

Journal • ANCA Vasculitis • Vasculitis

Emerging targeted therapies in ANCA-associated vasculitis. (PubMed, Rheumatology (Oxford)) - "Drug development in ANCA-associated vasculitis has aimed to improve on the success of the B cell depleting monoclonal antibody rituximab and exploit better understanding of inflammatory pathways. The involvement of the complement system in pathogenesis is more complicated than previously thought and extends beyond C5a dysregulation and its inhibition with avacopan, broader complement inhibitors and complement regulatory agonists are potential newer therapies. Other approaches have aimed to directly control neutrophil activation and to try to modulate tissue repair and fibrosis that occurs following vasculitis inflammation."

Journal • Review • ANCA Vasculitis • Fibrosis • Immunology • Inflammation • Vasculitis

Diagnostic and Prognostic Value of Proteinuria and Hematuria in a GPA nephritis (NKF-SCM 2025) - "Initially, she was treated with steroids, cyclophosphamide, rituximab, and IVIG, but these were discontinued following a hospitalization for a suspected lower lung infection. Infection was ruled out, and she was discharged on prednisone 60 mg daily. Rheumatology managed her care, and after an ANCA vasculitis panel, she was started on mycophenolate mofetil (MMF)...Due to treatment failure, she was started on cyclophosphamide 500 mg every two weeks and evaluated for the C5aR antagonist avacopan. A kidney biopsy was performed, revealing crescents in Bowman's space, capillary proliferation, minimal inflammation, and minimal immunofluorescence.Conclusion Proteinuria and hematuria are critical markers of GPA renal involvement, providing insights into disease progression and guiding treatment intensity. Regular urinalysis is essential for early detection and treatment evaluation."

ANCA Vasculitis • Chronic Kidney Disease • Infectious Disease • Inflammation • Nephrology • Ocular Inflammation • Ophthalmology • Pain • Rare Diseases • Renal Disease • Respiratory Diseases • Rheumatology • Scleritis • Vasculitis

The Great Impersonator: ANCA vasculitis presenting as mastoiditis (NKF-SCM 2025) - "Patient was discharged on a 4-dose series of weekly rituximab infusions, a prednisone taper, and eventual transition to avacopan.Conclusion ANCA are associated with primary systemic vasculitis, but they have also been described in other conditions such as malignancies, drug-induced vasculitis, and infection. In conclusion, patients presenting with atypical mastoiditis, neuropathy, and MRI with leptomenigeal enhancement, particularly in cases where anti-infection treatment has been proven ineffective, it is imperative to test ANCA titers to enhance diagnostic precision and avoid misdiagnosis."

ANCA Vasculitis • Infectious Disease • Oncology • Pain • Rare Diseases • Vasculitis