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

Infection Risk Associated with Steroid-Sparing Therapies in GCA, PMR, and ANCA-Associated Vasculitis: A Systematic Review. (PubMed, Joint Bone Spine) - "Steroid-sparing therapies reduced glucocorticoid exposure with varying magnitudes but were not consistently associated with lower infection risk. These findings underscore the heightened risk of infection when these novel therapies are used in combination with glucocorticoids when the glucocorticoid dose cannot be adequately tapered, particularly in older adults."

Journal • ANCA Vasculitis • Giant Cell Arteritis • Immunology • Infectious Disease • Musculoskeletal Pain • Pain • Polymyalgia Rheumatica • Rheumatology • Vasculitis

Rituximab With or Without Avacopan in ANCA-Associated Vasculitis With Severe Renal Involvement. (PubMed, Kidney Int Rep) - "Avacopan significantly reduced cumulative prednisone exposure at 12 months (1.8 vs 4.3 g; P < 0.001) and shortened time to prednisone discontinuation (3.3 vs 7.3 months; log-rank P < 0.001). One patient (3.3%) discontinued avacopan because of drug-induced liver injury, with transaminase normalization after discontinuation. In our cohort of patients with AAV and severe renal involvement treated with rituximab-based induction, avacopan did not improve renal remission rates or longitudinal eGFR trajectory in comparison to glucocorticoids but achieved significant and faster glucocorticoid reduction with an acceptable safety profile."

Journal • ANCA Vasculitis • Glomerulonephritis • Hepatology • Liver Failure • Nephrology • Vasculitis

Pyoderma gangrenosum lesions (EADV 2026) - "Rational treatment options could be escalation through 1st line therapies (topical steroids, tacrolimus, IL steroids, tetracyclines) to 2nd line therapies (prednisolone 0.5-1mg/kg/day, IV methylprednisolone, ciclosporin 4-5mg/kg/day, mycophenolate 1-3gm/day, methotrexate 15-25mg weekly, azathioprine 2-2.5mg/kg/day, dapsone, colchicine and then to 3rd line therapies (TNF inhibitors, IL-12/23 and IL-23 inhibitors, IL-17, IL-1, IL-36 and JAK inhibitors, IVIg, cyclophosphamide). C5a binding to its ligand complement component 5a receptor (C5aR) (also known as C5aR1) is not only a potent inducer of NETosis but is also a powerful chemoattractant, recruits and activates neutrophils. Avacopan (C5aR inhibitor), Vilobelimab (C5a neutralising antibody) as well as JAK inhibitors (NETosis pathway involves JAK-STAT signalling) would appear to be logical upstream inhibitors to prevent NETosis and PG and IL-36 inhibition for downstream inhibition of the cytokine storm in PG may well..."

Gastroenterology • Gastrointestinal Disorder • Hematological Disorders • Immunology • Inflammation • Inflammatory Arthritis • Inflammatory Bowel Disease • Pyoderma Gangrenosum • CTSG • ELANE • IL12A • IL17A • IL23A • MPO • PRSS3

Reduced severe infection risk with avacopan in ANCA-associated vasculitis: a multicentre REVEAL cohort with time-varying exposure modelling. (PubMed, Rheumatology (Oxford)) - "In this multicentre real-world cohort, AVA exposure was associated with a lower estimated risk of severe infection and reduced glucocorticoid exposure, whereas no statistically significant reduction in recurrent relapse risk was observed. These findings suggest that an AVA-containing treatment strategy may be associated with improved infection-related outcomes in routine practice, although the observational design precludes causal inference."

Journal • ANCA Vasculitis • Infectious Disease • Vasculitis

RECURRENT PULMONARY GRANULOMATOSIS WITH POLYANGIITIS PRESENTING WITH NEW CAVITARY LESIONS IN A PEDIATRIC PATIENT DESPITE PRIOR RITUXIMAB AND AVACOPAN MAINTENANCE (CHEST 2026) - No abstract available

Clinical • Pediatrics • Rare Diseases • Vasculitis

Non-suppurative Destructive Cholangitis After Avacopan Therapy in Myeloperoxidase-Antineutrophil Cytoplasmic Antibody (MPO-ANCA)-Associated Glomerulonephritis: A Case Report and Review of the Literature. (PubMed, Cureus) - "A 55-year-old obese female with myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA)-associated crescentic glomerulonephritis achieved remission via steroid pulse therapy, oral prednisolone (PSL), and rituximab. Seven weeks before admission, avacopan and ursodeoxycholic acid (UDCA) were initiated...To the best of our knowledge, this is the first report describing a comprehensive immunohistochemical evaluation of the periportal microenvironment in avacopan-induced biliary injury. This case highlights that severe cholangitis can occur regardless of baseline risk profiles or disease activity, underscoring the need for vigilant, long-term monitoring of liver enzymes and bilirubin levels during avacopan therapy."

Journal • Glomerulonephritis • Hepatology • Liver Failure • Lupus Nephritis • Nephrology • Obesity • CD4 • CD68 • CK19 • KRT19 • MPO

Effectiveness and Safety of Avacopan Added to Standard of Care (SOC) Versus SOC Alone in ANCA-associated Vasculitis (ACR Convergence 2026) - No abstract available

Clinical • ANCA Vasculitis • 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 (ACR Convergence 2026) - No abstract available

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

Real-World Effectiveness and Safety of Avacopan in Granulomatosis with Polyangiitis and Microscopic Polyangiitis Over Two Years in a Large US Healthcare System (ACR Convergence 2026) - No abstract available

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

Real-World Effectiveness, Safety, and Discontinuation of Avacopan in ANCA-Associated Vasculitis: A Systematic Review and Meta-Analysis (ACR Convergence 2026) - No abstract available

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

Multi-database pharmacovigilance identifies disproportionate reporting of hepatobiliary events with avacopan: an integrative study with network pharmacology and interpretable machine learning. (PubMed, Naunyn Schmiedebergs Arch Pharmacol) - "This real-world study expands the safety profile of avacopan and highlights hepatobiliary disorders as important signals requiring clinical attention. These findings support strengthened post-marketing surveillance and careful liver monitoring during avacopan therapy."

Adverse events • Journal • Cholestasis • Gastroenterology • Gastrointestinal Disorder • Hepatology • Infectious Disease • Liver Failure • Metabolic Disorders • Rare Diseases • Vasculitis

Complement targeting in autoimmune diseases. (PubMed, Curr Opin Hematol) - "Recognizing complement's role in the pathophysiology of hematological and autoimmune diseases has driven effective new therapies. Comprehensive profiling complement biomarkers, now feasible through complementomics in fluids and tissues, may reveal responsive endotypes and optimal cascade steps to target in diseases where complement is implicated but trials have not yet matched the right drug to the right patients."

Journal • Autoimmune Hemolytic Anemia • Complement-mediated Rare Disorders • Glomerulonephritis • Hematological Disorders • IgA Nephropathy • Immunology • Inflammatory Arthritis • Lupus • Lupus Nephritis • Nephrology • Paroxysmal Nocturnal Hemoglobinuria • Rare Diseases • Renal Disease • Systemic Lupus Erythematosus

Kidney Disease: Improving Global Outcomes (KDIGO) responds to the retraction of the ADVOCATE trial of avacopan for the treatment of ANCA-associated vasculitis. (PubMed, Kidney Int) - No abstract available

Journal • ANCA Vasculitis • Nephrology • Renal Disease • Vasculitis

Hepatotoxicity of Avacopan: Real-World Incidence and Confirmed Cases of Severe Drug-Induced Liver Injury in a US National Rheumatology Registry (ACR Convergence 2026) - No abstract available

Clinical • Real-world • Real-world evidence • Hepatology • Liver Failure • Rheumatology

Real-World Experience with Avacopan in ANCA-Associated Vasculitis: Renal Outcomes (ACR Convergence 2026) - No abstract available

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

Hepatotoxicity Associated with Avacopan in ANCA-Associated Vasculitis: A Single-Center Real-World Experience (ACR Convergence 2026) - No abstract available

Adverse events • Clinical • Real-world • Real-world evidence • ANCA Vasculitis • Hepatology • Vasculitis

Clinical Outcomes of Real-World Avacopan Use in ANCA-associated Vasculitis: A Multicenter, Retrospective, Comparative-Effectiveness Study (ACR Convergence 2026) - No abstract available

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

AvacoStar: First interim results from a prospective real-world safety study of avacopan in adults with ANCA-associated vasculitis (AAV) (ACR Convergence 2026) - No abstract available

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

Real-World Effectiveness and Safety of Avacopan in ANCA-Associated Vasculitis: A Target Trial Emulation (ACR Convergence 2026) - No abstract available

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

Real-World Evaluation of Hepatotoxicity in Avacopan-Treated Patients with ANCA-Associated Vasculitis: A Post-Marketing Safety Study (ACR Convergence 2026) - No abstract available

Clinical • HEOR • P4 data • Real-world • Real-world evidence • ANCA Vasculitis • Hepatology • Vasculitis

The Canadian Registry of Avacopan for ANCA Vasculitis (CARAvAN) study: Outcomes at 6-Month Follow-up (ACR Convergence 2026) - No abstract available

ANCA Vasculitis • Vasculitis

Safety and efficacy of avacopan in ANCA-associated vasculitis: a single-center experience (ACR Convergence 2026) - No abstract available

Clinical • ANCA Vasculitis • Vasculitis

Beyond the Guidelines: Updated Management of Sytemic Vasculitis (ACR Convergence 2026) - "Moreover, we now have access to multiple agents that were not available at the time the ACR/VF guidelines were created (such as avacopan, benralizumab, and upadacitinib), but how to incorporate these new agents into the guidelines is not clear. In this session, we will briefly highlight specific recommendations made by the ACR/VF guidelines, and demonstrate how to integrate recent advances into an updated therapeutic approach. This session will also address knowledge gaps by describing the approach to clinical scenarios not considered by the 2021 ACR/VF guidelines Learning Objectives: Propose an updated approach to the management of granulomatosis with polyangiitis and microscopic polyangiitis Examine the role of IL-5 blockade in the management of eosinophilic granulomatosis with polyangiitis Describe the use of newly approved agents for the management of giant cell arteritis"

Eosinophilic Granulomatosis With Polyangiitis • Giant Cell Arteritis • Immunology • Langerhans Cell Histiocytosis • Rare Diseases • Vasculitis • IL5

TAVNEOS for Otolaryngologic Manifestations of Granulomatosis With Polyangiitis (clinicaltrials.gov) - P2/3 | N=30 | Recruiting | Sponsor: Robert Spiera, MD | Not yet recruiting ➔ Recruiting

Enrollment open • Otorhinolaryngology • Rare Diseases • Vasculitis

UK regulator plans to withdraw controversial vasculitis drug Tavneos over unreliable data. (PubMed, BMJ) - No abstract available

Journal • Vasculitis