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

PR3-ANCA–ASSOCIATED VASCULITIS WITH ELEVATED IGG4 AND RETROPERITONEAL FIBROSIS: A DIAGNOSIS AND THERAPIUTIC CHALLENGE (ISN-WCN 2026) - "Treatment included half-pulse methylprednisolone, oral prednisolone (60 mg/day), and two doses of rituximab...A second steroid pulse and avacopan were added, leading to improvement in neurological symptoms, scleritis, skin lesions, urinary findings, and imaging, including RPF and hydronephrosis.Conclusion This case illustrates the rare coexistence of PR3-ANCA–positive AAV and retroperitoneal fibrosis (RPF) with elevated IgG4...This case highlights the importance of integrating histopathology, imaging, and clinical context to guide diagnosis and treatment in atypical presentations of AAV. Open renal biopsy was essential for accurate diagnosis and therapeutic decision-making."

ANCA Vasculitis • Fibrosis • Immunology • Inflammation • Nephrology • Ocular Inflammation • Ophthalmology • Renal Disease • Scleritis • Vasculitis • CXCL8

REAL-WORLD USE AND EXTENDED TREATMENT OUTCOMES OF AVACOPAN IN ANCA-ASSOCIATED VASCULITIS: INSIGHTS FROM THE MULTICENTER EUROPEAN AVAC-EUR STUDY (ISN-WCN 2026) - "Avacopan-based treatment strategies included predominantly rituximab (84.3%), and cyclophosphamide (20.4%). EXT-avacopan did not increase infections or hospitalizations and showed fewer relapses (0.13 vs 0.35 per patient-year, p=0.08).Conclusion In European real-world practice, avacopan during EAP was predominantly combined with rituximab and steroids. Extended treatment beyond 1 year may lower relapse risk without increased infections or hospitalizations.The content presented in this abstract was also submitted for the 22nd International Vasculitis Workshop 2026 in Melbourne, Australia."

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

CLINICAL IMPACT OF IMMUNOSUPPRESSIVE COMBINATION THERAPY IN MPO-ANCA–ASSOCIATED RAPIDLY PROGRESSIVE GLOMERULONEPHRITIS: A RETROSPECTIVE COMPARISON WITH STEROID MONOTHERAPY (ISN-WCN 2026) - "This study aimed to compare the clinical course, renal outcomes, and safety between the two treatment strategies in MPO-ANCA–associated RPGN.Methods We retrospectively analyzed patients diagnosed with AAV-related RPGN at our hospital between April 2012 and March 2025.Inclusion criteria:(1) Age ≥18 years; (2) positive for myeloperoxidase (MPO)-ANCA; (3) diagnosis of microscopic polyangiitis (MPA); and(4) ≥6 months of follow-up after initiation of therapy.Exclusion criteria:(1) proteinase-3 (PR3)-ANCA positivity,(2) anti-glomerular basement membrane (GBM) antibody positivity,(3) drug-induced vasculitis, and(4) patients who required maintenance dialysis from the time of diagnosis.A total of 70 patients were included: 44 in the steroid monotherapy group and 26 in the immunosuppressive combination group.Immunosuppressive agents included rituximab (RTX), azathioprine (AZA), and avacopan, and treatment choice was at the discretion of the attending physician.Clinical..."

Combination therapy • Monotherapy • Retrospective data • ANCA Vasculitis • Diabetes • Diabetic Nephropathy • Glomerulonephritis • Infectious Disease • Lupus Nephritis • Metabolic Disorders • Nephrology • Vasculitis • MPO

INFLUENCE OF EXPANDING THERAPEUTIC OPTIONS ON REMISSION INDUCTION STRATEGIES AND OUTCOMES IN ANCA-ASSOCIATED VASCULITIS: A SINGLE-CENTER RETROSPECTIVE STUDY (ISN-WCN 2026) - "Introduction The therapeutic landscape of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) has evolved considerably following the approval of rituximab (RTX) and, more recently, the introduction of avacopan...The use of steroid pulse therapy became significantly more frequent, whereas the initial and 2-month doses of oral prednisolone did not differ significantly between groups...However, no significant improvement was observed in short-term renal or survival outcomes or in infection risk. These findings suggest that further optimization of treatment regimens, particularly with respect to minimizing glucocorticoid exposure, remains warranted."

Retrospective data • ANCA Vasculitis • Eosinophilic Granulomatosis With Polyangiitis • Infectious Disease • Langerhans Cell Histiocytosis • Rare Diseases • Vasculitis

A CASE OF LIVER INJURY IN AN ELDERLY PATIENT WITH DIABETES AND ANCA-ASSOCIATED VASCULITIS DURING AVACOPAN USE (ISN-WCN 2026) - "Renal involvement was considered an organ manifestation of vasculitis, and prednisone (PSL) 0.5 mg/kg/day was initiated...Although avacopan was discontinued during amenamevir administration, prior avacopan use before starting amenamebvir may have led to increased amenamevir blood levels.Conclusion ANCA-associated vasculitis is a systemic disease causing multi-organ dysfunction, necessitating therapeutic intervention. Recent advances in molecularly targeted therapies, such as rituximab and avacopan, have expanded treatment options. Clinicians involved in future management must select optimal treatment strategies, considering both therapeutic timing, dosage, and administration methods, while also weighing healthcare economics."

Clinical • ANCA Vasculitis • Anorexia • Cardiovascular • Diabetes • Hepatology • Herpes Zoster • Hypertension • Inflammation • Liver Failure • Metabolic Disorders • Nephrology • Varicella Zoster • Vasculitis • CYP3A4

EXPERIENCE WITH AVACOPAN FOR THE TREATMENT OF ANTI-NEUTROPHIL CYTOPLASMIC ANTIBODY (ANCA)-ASSOCIATED VASCULITIS (AAV) IN THE NEPHROLOGY DEPARTMENT OF NISHI-KOBE MEDICAL CENTER. (ISN-WCN 2026) - "Concomitant immunosuppressive agents included cyclophosphamide in 6 patients, rituximab in 2, azathioprine in 5, and mizoribine in 6; 2 patients underwent plasma exchange.Clinical remission was achieved in 85% of patients at 26 weeks...Avacopan was restarted at a lower dose in all cases—one with concomitant ursodeoxycholic acid and another after alcohol abstinence. No infections requiring hospitalization were observed during the observation periodConclusion Avacopan appeared to be a safe and effective glucocorticoid-sparing therapeutic option for AAV. However, regular monitoring of liver function is warranted.I have potential conflict of interest to disclose.I have received lecture fees from KISSEI PHARMACEUTICAL CO.,LTD.I did not use generative AI and AI-assisted technologies in the writing process."

ANCA Vasculitis • Atherosclerosis • Cardiovascular • Infectious Disease • Nephrology • Rare Diseases • Vasculitis

A CASE OF VANISHING BILE DUCT SYNDROME (VBDS) CAUSED BY AVACOPAN (AVA) ADMINISTRAION FOR TREATMENT OF MICROSCOPIC POLYANGIITIS (MPA) (ISN-WCN 2026) - "A week after PSL administration, AVA and rituximab (RTX) were added...She was treated with ursodeoxycholic acid and glucocorticoid (including 1,000mg/day mPSL). For liver injury due to AVA, mycophenolate mofetil (MMF) was added...Therefore, we referred her to university hospital for consideration of liver transplantation.Conclusion We experienced a case of VBDS caused by AVA administration for treatment of MPA. When we are planning to dose the patients to AVA during remission induction therapy for MPA, we should pay attention to risks of liver injury and consider frequent laboratory follow-ups."

Clinical • ANCA Vasculitis • Glomerulonephritis • Hepatology • Liver Failure • Lupus Nephritis • Vasculitis • MPO

CASE-BASED INFERENCE OF AVACOPAN'S IMPACT ON RENAL INFLAMMATION AND TISSUE DAMAGE IN ANCA-ASSOCIATED VASCULITIS PATIENTS (ISN-WCN 2026) - "The standard induction therapy for remission has been oral glucocorticoids (GC) combined with either cyclophosphamide (CY) or rituximab (RTX). In Case 2, however, a combination of PSL 30 mg and avacopan did not sufficiently suppress highly active lesions that had already progressed to necrosis. This suggests that avacopan alone may have relatively limited anti-inflammatory potency compared to GC."

Clinical • ANCA Vasculitis • B Cell Lymphoma • Hematological Malignancies • Infectious Disease • Inflammation • Large B Cell Lymphoma • Lymphoma • Non-Hodgkin’s Lymphoma • Pneumonia • Respiratory Diseases • Vasculitis

A CASE OF SUCCESSFUL TREATMENT OF SEVERE ANCA-ASSOCIATED VASCULITIS WITH DIVERSE NEUROLOGICAL SYMPTOMS USING A STEROID REDUCTION REGIMEN COMBINED WITH AVACOPAN AND RITUXIMAB. (ISN-WCN 2026) - "The therapeutic effect of Avacopan for neurological disorders due to AVA has not been clearly established until now. This case suggests that Avacopan may be an effective treatment option for ANCA-associated vasculitis with neuropathy.Conclusion This case suggests that a steroid reduction regimen combined with Avacopan may be effective for neurological disorders caused by AVA."

Clinical • ANCA Vasculitis • CNS Disorders • Cough • Glomerulonephritis • Infectious Disease • Interstitial Lung Disease • Lupus Nephritis • Nephrology • Otorhinolaryngology • Pneumonia • Renal Disease • Respiratory Diseases • Sinusitis • Vasculitis

A CASE OF GRANULOMATOSIS WITH POLYANGIITIS PRESENTING WITH ABDOMINAL PAIN AND IGA DEPOSITION IN CUTANEOUS VESSEL WALLS, MIMICKING IGA VASCULITIS (ISN-WCN 2026) - "Oral prednisolone was initiated, which improved abdominal pain and arthralgia...The patient was treated with high-dose intravenous steroids, avacopan, rituximab, and plasma exchange therapy...The mechanism of IgA deposition in the skin remains unclear; however, chronic mucosal inflammation, as seen in patients with chronic sinusitis or allergic rhinitis, may lead to alterations in IgA structure or metabolism, which could in turn contribute to its deposition within small dermal vessels.Conclusion This case highlights that GPA can present with abdominal pain and IgA deposition in the skin, findings that are atypical for GPA and mimic IgA vasculitis. In such cases, kidney biopsy is crucial for accurate diagnosis and timely initiation of appropriate immunosuppressive therapy."

Clinical • Allergic Rhinitis • Glomerulonephritis • Inflammation • Lupus Nephritis • Mucositis • Musculoskeletal Pain • Nephrology • Otorhinolaryngology • Pain • Rare Diseases • Renal Disease • Respiratory Diseases • Sinusitis • Vasculitis

VANISHING BILE DUCT SYNDROME RELATED TO AVACOPAN THERAPY IN A PATIENT WITH ANCA-ASSOCIATED VASCULITIS. (ISN-WCN 2026) - "Following steroid pulse therapy, she started oral prednisolone 40 mg daily, combined with avacopan. At a post-discharge outpatient visit (day 32 of avacopan administration), marked elevation of liver enzymes was noted, requiring readmission.Results The patient was diagnosed with liver injury due to abacavir and abacavir was discontinued...Treatment with plasma exchange resulted in improvement of the liver injury, and the patient was discharged.Conclusion To avoid progression to severe liver dysfunction, strict monitoring of liver function is required during treatment. Plasma exchange may be useful for improving liver dysfunction caused by avacopan.We presented atThe 55th Western Regianal Meeting of the Japanese Society of Nephrology."

Clinical • ANCA Vasculitis • Glomerulonephritis • Hepatology • Liver Failure • Lupus Nephritis • Nephrology • Renal Disease • Vasculitis

TWO CASES OF VANISHING BILE DUCT SYNDROME FOLLOWING INITIATION OF AVACOPAN THERAPY FOR ANCA-ASSOCIATED VASCULITIS. (ISN-WCN 2026) - "After remission induction therapy with glucocorticoids, rituximab, and plasma exchange, avacopan (60 mg daily) was initiatedto facilitate rapid tapering of steroids...Despite discontinuation of avacopan and initiation of ursodeoxycholic acid (UDCA), liver dysfunction persisted, his general condition consequently worsened, and he ultimately died...VBDS associated with avacopan is a very rare but potentially severe form of DILI. Among our two cases of avacopan-related VBDS, one patient recovered after discontinuation of avacopan, whereas the other progressed to severe liver injury leading to death.Conclusion 【Conclusion】Because avacopan-related VBDS can progrese to severe DILI, careful monitoring of liver function is essential after initiation of avacopan."

Clinical • ANCA Vasculitis • Glomerulonephritis • Hepatology • Liver Failure • Lupus Nephritis • Nephrology • Vasculitis

Evaluation of the Long-Term Safety of Avacopan in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis in the Real World (AvacoStar): Protocol for a Noninterventional Prospective Cohort Study. (PubMed, JMIR Res Protoc) - "Avacopan, an oral selective C5a receptor antagonist, was approved by the European Commission in January 2022 for the treatment of adult patients with severe, active GPA or MPA in combination with rituximab (RTX) or cyclophosphamide (CYC). In the pivotal phase 3 ADVOCATE (Avacopan Development in Vasculitis to Obtain Corticosteroid Elimination and Therapeutic Efficacy) study, avacopan was noninferior to prednisone taper in achieving remission at week 26 and superior in sustaining remission at week 52; furthermore, a greater improvement in estimated glomerular filtration rate with avacopan was also observed at week 52...The AvacoStar study will be the largest European prospective real-world evidence comparative study conducted to date that evaluates the long-term safety of avacopan in severe, active GPA or MPA. This study is expected to yield important insights on the use of avacopan in severe, active GPA or MPA in a real-world setting."

Clinical protocol • Journal • Real-world evidence • ANCA Vasculitis • Hepatology • Infectious Disease • Liver Failure • Oncology • Rare Diseases • Vasculitis

Evolving Therapeutic Strategies in ANCA-Associated Vasculitis: Current Standards and Emerging Targets for GPA and MPA. (PubMed, Clin Rev Allergy Immunol) - "Standard induction therapy combines glucocorticoids (GCs) with rituximab (RTX) or cyclophosphamide (CYC), with growing emphasis on GC minimization and selective use of avacopan in patients at high risk of GC toxicity. These include agents targeting B cells and plasma cells (anti-CD38, anti-CD19, proteasome inhibition, CAR-T cells), complement components, and T-cell co-stimulation or cytokine networks (abatacept, IL-6 and JAK-inhibitors). Collectively, these advances are shifting AAV care from broad immunosuppression toward precision immunotherapy aimed at durable remission with reduced GC exposure and minimized long-term toxicity."

IO biomarker • Journal • Review • ANCA Vasculitis • Immunology • Inflammation • Rare Diseases • Vasculitis • IL6

Should steroids take a back seat in 2025: real world use of avacopan (ERA 2026) - No abstract available

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

Cost effectiveness of avacopan for ANCA-associated vasculitis in China. (PubMed, Clin Exp Rheumatol) - "Avacopan-based regimens are cost-effective versus GC-based therapy for GPA/MPA in China and support value-based pricing of $11.12-$12.70 per 10 mg."

HEOR • Journal • ANCA Vasculitis • Chronic Kidney Disease • Nephrology • Rare Diseases • Renal Disease • Vasculitis

Real-world efficacy and safety of avacopan in ANCA-associated vasculitis: a retrospective comparative study (EULAR 2026) - No abstract available

Real-world • Real-world effectiveness • Real-world evidence • Retrospective data • ANCA Vasculitis • Vasculitis

Beyond drug toxicity: acute hepatitis E in a patient with granulomatosis with polyangiitis during treatment with Rituximab and Avacopan (EULAR 2026) - No abstract available

Clinical • Hepatology • Inflammation • Rare Diseases • Vasculitis

One Year Real-World Effectiveness and Safety with Avacopan in Granulomatosis with Polyangiitis and Microscopic Polyangiitis in Two Large Healthcare Systems (EULAR 2026) - No abstract available

Clinical • Real-world • Real-world effectiveness • Real-world evidence • Rare Diseases • Vasculitis

Clinical Outcomes by Glucocorticoid Duration in Individuals with Granulomatosis with Polyangiitis and Microscopic Polyangiitis Treated with Avacopan in a Real-World Setting in Two Large Healthcare Systems (EULAR 2026) - No abstract available

Clinical • Clinical data • Real-world • Real-world evidence • Rare Diseases • Vasculitis

Relaps free survival in AAV patients on GC-free maintenance treatment after discontinuation of avacopan – a 2-year prospective uni-center observational study stratified into renal and non-renal manifestations (EULAR 2026) - No abstract available

Clinical • Observational data

Avacopan-target C5AR1 is increased in crescentic glomeruli in ANCA vasculitis kidney biopsies (EULAR 2026) - No abstract available

Biopsy • ANCA Vasculitis • Vasculitis

Clinical Remission Despite Persistent MPO-ANCA Positivity During Avacopan-Based Therapy in Microscopic Polyangiitis and Granulomatosis with Polyangiitis: A Retrospective Observational Cohort Study (EULAR 2026) - No abstract available

Observational data • Retrospective data • Rare Diseases • Vasculitis

Modern Management of Necrotizing ANCA-Associated Vasculitis Beyond Cyclophosphamide: Clinical Outcomes with Rituximab plus Avacopan (EULAR 2026) - No abstract available

Clinical • Clinical data • ANCA Vasculitis • Vasculitis

Anti CD19 targeting CAR T cell therapy in ANCA-associated vasculitis. (PubMed, Rheumatology (Oxford)) - "CD19-targeted CAR T cell therapy was safe and efficacious in a patient with relapsed and refractory GPA. CAR T cell therapy has the potential to transform disease management and improve long-term outcomes for affected patients."

Journal • ANCA Vasculitis • Immunology • Inflammation • Rare Diseases • Vasculitis • CRP