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

Complement-induced procoagulant platelets in patients with antiphospholipid antibodies (ASH 2025) - "On certain occasions sera were heat inactivated at 56º C for30 min or pre-treated with IdeS (IgG cleaving enzyme) (10000 U/ml at 37º C for 30 min), eculizumab (1mg/ml), sutimlimab (C1s inhibitor) (1.5 mg/ml) and/or danicopan (Factor D inhibitor) (100 µM). Also, onother occasions platelets were pre-incubated with antagonists of C3aR (SB290157), FcgRIIa (IV.3) or C5aR1(Avacopan) (10 µM) before serum.ResultsWe found that although activation and responsiveness to agonists of platelets from aPL+/APS patientswere like those from healthy individuals, unstimulated platelets from APS patients exhibited higherphosphatidylserine (PS) exposure (19.6±3.9% vs 4.0±1.0% controls) and P-selectin expression (15.9±2.3%vs 8.6±1.8% controls) than controls suggesting induction of procoagulant activity...Pre-incubating platelets with FcgRIIa, C3aR or C5aR1 antagonistsdid not reverse the procoagulant activity induced by APS serum.ConclusionWe demonstrate..."

Clinical • Cardiovascular • Genetic Disorders • Hematological Disorders • Immunology • Thrombosis • ANXA5 • CASP3 • CASP7 • SELP

Granulomatosis With Polyangiitis (GPA) Presenting With Painless Scleritis and Ocular Hypertension: Case Report. (PubMed, Case Reports Immunol) - "He was subsequently managed with rituximab and avacopan as per standard GPA therapy. This case highlights the importance of considering vasculitis in patients presenting with unexplained elevation of EVP, and painless scleritis, particularly when there are also extraocular complaints."

Journal • Cardiovascular • Glaucoma • Ocular Inflammation • Ophthalmology • Pain • Rare Diseases • Rheumatology • Scleritis • Vasculitis

Avacopan or Glucocorticoids for Severe Antineutrophil Cytoplasmic Autoantibody-Associated Rapidly Progressive Glomerulonephritis. (PubMed, Kidney Int Rep) - "Fifty patients received a GC regimen combined with cyclophosphamide (CYC) (30%), rituximab (RTX, 54%) or a combination of both (16%), whereas 20 patients received avacopan combined with RTX (90%) or RTX plus CYC (10%). No new safety signal was identified in the avacopan group. At months 6 and 12, patients with severe forms of ANCA-RPGN and receiving avacopan plus a short course of GCs have similar kidney recovery rates to patients receiving GC regimen."

Journal • ANCA Vasculitis • Glomerulonephritis • Inflammation • Lupus Nephritis • Nephrology • Renal Disease • Vasculitis

Mohs Surgery as a Precipitant of Bullous Disease: A Review of Reported Cases and Translational Implications (ISDS 2025) - "Five received systemic corticosteroids, often with azathioprine, doxycycline, or niacinamide; one improved with topical and intralesional therapy...Agents such as IL-1β inhibitors (e.g., anakinra), complement antagonists (e.g., avacopan), and FcRn blockers (e.g., efgartigimod) target relevant immune pathways and warrant exploration in refractory or rapidly progressive cases. While uncommon, PV, PF, and BP may emerge after MMS and mimic typical postoperative complications. Prompt biopsy and immunofluorescence facilitate early diagnosis and immunosuppressive therapy, minimizing progression and promoting wound healing."

Clinical • Review • Surgery • Bullous Pemphigoid • Contact Dermatitis • Dermatitis • Dermatology • Dermatopathology • Immunology • Infectious Disease • Pemphigus Vulgaris • IL1B

Novel Treatment Paradigms: Complement Inhibition in Antineutrophil Cytoplasmic Autoantibody Vasculitis. (PubMed, Kidney Int Rep) - "Avacopan is an oral small molecule C5a receptor antagonist approved as adjunctive therapy to standard treatments for severe active ANCA vasculitis...Although early results suggest that complement inhibitors may offer more effective and safer alternatives to established therapies, there are limitations and barriers that prevent their more widespread use. Further research is needed to better understand their efficacy and long-term safety and to inform how to optimize their integration into treatment paradigms for ANCA vasculitis."

Journal • Review • ANCA Vasculitis • Nephrology • Renal Disease • Vasculitis

Antineutrophil Cytoplasmic Antibody (ANCA) Vasculitis, Fatigue, and Treatment With Avacopan: A Case Series. (PubMed, Cureus) - "This report describes three patients who developed marked somnolence and fatigue during treatment with avacopan for antineutrophil cytoplasmic antibody (ANCA) vasculitis. Though fatigue is a common symptom in ANCA vasculitis, each of these patients reported noticeable worsening of fatigue after starting avacopan, with improvement shortly after stopping the medication. All of them had a good response to treatment of their underlying vasculitis, and none of them had other clear causes of worsening fatigue."

Journal • ANCA Vasculitis • Fatigue • Glomerulonephritis • Lupus Nephritis • Nephrology • Vasculitis

A Real-World Disproportionality Analysis of Avacopan in Anti-Neutrophil Cytoplasmic Antibodies Associated Vasculitis: Insights From FDA Adverse Event Reporting System. (PubMed, Pharmacol Res Perspect) - "This study provides the first FAERS-based pharmacovigilance assessment of Avacopan, confirming known risks and identifying novel post-approval safety signals, underscoring the need for close early-treatment monitoring and personalized strategies. Further research is warranted to confirm emerging signals and explore their mechanisms."

Adverse events • Journal • Real-world evidence • Retrospective data • Alopecia • ANCA Vasculitis • Cardiovascular • Hepatology • Immunology • Infectious Disease • Vasculitis • Venous Thromboembolism

Granulomatosis with Polyangiitis (GPA) in a Polish Tertiary Centre (2010-2025): Sex-Stratified Phenotypes, Serology, and Evolving Treatment Patterns. (PubMed, J Clin Med) - "All patients received glucocorticoids for induction; cyclophosphamide 28/50 (56.0%), rituximab 6/50 (12.0%), and mycophenolate with methotrexate 6/50 (32%). Maintenance therapy included methotrexate (78.0%), mycophenolate (64.0%), rituximab (52.0%), and azathioprine (12.0%). This Polish single-centre cohort shows an ear-nose-throat-lung-kidney (ELK)-dominant, PR3-predominant GPA phenotype and frequent but variable kidney involvement. Over 2010-2025, practice changed toward rituximab-based strategies, steroid minimisation, selective use of plasma exchange, and early avacopan uptake, with tofacitinib for maintenance therapy as a possible new therapeutic option."

Journal • ANCA Vasculitis • Chronic Kidney Disease • Inflammation • Nephrology • Ocular Inflammation • Otorhinolaryngology • Rare Diseases • Renal Disease • Respiratory Diseases • Rheumatology • Sinusitis • Vasculitis

A Rare Case of ANCA-Associated Vasculitis (AAV) with Concurrent Immune Complex (IC) Deposition (KIDNEY WEEK 2025) - "Case Description A 74-year old male with past medical history of prostate cancer (CA), non-small cell lung CA, basal cell CA, rheumatoid arthritis (on abatacept) and hypertension presented to the hospital with complaint of flank pain. He reported consuming 1.6-2g/day of ibuprofen. Home medications included nifedipine and carvedilol...He received induction treatment with rituximab, steroids and avacopan...However, the deposition of both IgG and IgA co-dominant deposits is still rare. In spite of his aggressive RPGN, we elected not to use cyclophosphamide given his recent malignancies."

Clinical • ANCA Vasculitis • Genito-urinary Cancer • Glomerulonephritis • Hypertension • IgA Nephropathy • Immunology • Inflammatory Arthritis • Lung Cancer • Lupus Nephritis • Nephrology • Non Small Cell Lung Cancer • Prostate Cancer • Renal Disease • Rheumatoid Arthritis • Rheumatology • Solid Tumor • Thrombosis • Vasculitis • MPO

ANCA Alert: A Curious Case of Amikacin-Induced Vasculitis (KIDNEY WEEK 2025) - "Introduction Drug induced-antineutrophil cytoplasmic antibody-associated vasculitis (AAV) is commonly caused by medications such as hydralazine, propylthiouracil, methimazole, and minocycline, but to date, amikacin has not been reported in the literature.We present the first case of AAV with renal involvement with amikacin as the unusual culprit...Empiric piperacillin-tazobactam and intravenous fluids were started...The patient was discharged on prednisone with plans for outpatient rituximab and avacopan...Plasma exchange and steroids resulted in significant improvement in renal function shortly after initiation which can be associated with return of renal function and creatinine apheresis. As we learn more about renal involvement in AAV, the list of causative agents expands."

Clinical • ANCA Vasculitis • Benign Prostatic Hyperplasia • Chronic Kidney Disease • Glomerulonephritis • Infectious Disease • Lupus Nephritis • Nephrology • Renal Disease • Vasculitis • MPO

Pharmacokinetics (PK) of Avacopan in Participants with ESKD Requiring Hemodialysis (HD) (KIDNEY WEEK 2025) - "Conclusion Given the PK variability of avacopan (23% to 54%), this study indicates no clinically meaningful effect of ESKD on avacopan, M1 and TAM exposures. No dose adjustment is warranted for avacopan due to ESKD or HD status."

PK/PD data • Rare Diseases • Renal Disease • Vasculitis

Patient Profiles, Treatment Patterns, and Economic Burden Among Users of Avacopan in the United States (KIDNEY WEEK 2025) - "Among those with 12-month follow-up (n=42), mean total oral prednisone-equivalent daily dose decreased from 19.8 mg to 10.6 mg between 90 days pre- and post-avacopan initiation. Conclusion A majority of users of avacopan with background immunosuppressive therapy had kidney involvement at baseline. Most patients have decreased GC exposure post-avacopan initiation."

Clinical • HEOR • Rare Diseases • Rheumatology • Vasculitis

Avacopan in Patients with ANCA-Associated Vasculitis and Severe Kidney Disease (KIDNEY WEEK 2025) - "Eighty percent received rituximab induction, and 33% received plasmapheresis. The mean time to initiation of avacopan was 43 days, with 80% successfully tapered off prednisone...Conclusion Our study demonstrated that avacopan use in AAV patients with severe kidney disease was associated with substantial eGFR recovery in non-RRT patients and had an acceptable safety profile. However, ESKD occurred in 53% of patients, and these observations warrant further study on this cohort."

Clinical • ANCA Vasculitis • Infectious Disease • Nephrology • Renal Disease • Vasculitis

Avacopan in Clinical Practice: Adherence, Steroid Reduction, and Other Claims-Based Real-World Outcomes (KIDNEY WEEK 2025) - "At baseline, 35.8% of patients had kidney disease and most patients used glucocorticoids (GCs, 95.8%) and rituximab (65.3%)...Mean prednisone-equivalent daily dose of oral GCs 3mo before index was 16.3mg and decreased to 9.0, 5.3, 5.4 and 3.3mg by 3, 6, 9 and 12mo post-index, respectively...Conclusion Kidney disease was frequent among patients with GPA/MPA who were dispensed avacopan. Most patients with ≥12mo follow-up remained on avacopan, and average daily oral GC exposure decreased at 3, 6, 9 and 12mo after avacopan."

Adherence • Clinical • Real-world • Real-world evidence • Inflammation • Nephrology • Rare Diseases • Renal Disease • Vasculitis

Addition of Avacopan to the Standard Care of Patients with Pauci-Immune Glomerulonephritis Is Associated with Higher Remission Rates During the First Year Following Diagnosis (KIDNEY WEEK 2025) - "Background The addition of avacopan to the standard of care,cyclophosphamide or rituximab with glucocorticoids,of pauci-immune glomerulonephritis and vasculitis aims to improve disease outcomes and reduce side effects of therapy by minimizing the cumulative exposure to glucocorticoids.The goal of this study was to compare the clinical characteristics and outcomes of patients who received avacopan in addition to standard therapy to those who did not. 28(12.4%) received avacopan. Within of 12.7 months from the KBx, 92.85% in the avacopan group achieved remission compared to 75.85% of those who received only the standard treatment Conclusion The addition of avacopan to the standard of care of patients with PIGN seems to be associated with higher remission rates during the 1st year, compared to the rates of patients who did not receive avacopan."

Clinical • Glomerulonephritis • Lupus Nephritis • Nephrology • Vasculitis

Under Pressure: Rethinking Plasma Exchange in the Management of ANCA-Associated Vasculitis with Alveolar Hemorrhage (KIDNEY WEEK 2025) - "Immunosuppressive therapy included high-dose glucocorticoids, Rituximab, and Avacopan (Table 1). Discussion DAH in AAV may appear refractory despite maximal immunosuppression, leading to consideration of PLEX. These cases suggest that hemodynamic derangements, rather than sustained immunologic activity, may underlie persistent or recurrent DAH, which may require meticulous fluid and blood pressure optimization instead of PLEX."

Acute Kidney Injury • ANCA Vasculitis • Glomerulonephritis • Hematological Disorders • Lupus Nephritis • Nephrology • Renal Disease • Vasculitis

Repeat Kidney Biopsy in Suspected Relapse of ANCA-Associated Vasculitis: A Case of De Novo IgAN (KIDNEY WEEK 2025) - "The prednisolone dose was increased, and avacopan and rituximab were initiated. It underscores the importance of considering alternative or coexisting glomerular pathologies and highlights the critical role of repeat kidney biopsy in establishing an accurate diagnosis when clinical deterioration occurs in a patient with established AAV. Given the rarity of concurrent AAV and IgA nephropathy, further collection and analysis of similar cases are warranted to elucidate their potential association."

Biopsy • Clinical • ANCA Vasculitis • Glomerulonephritis • IgA Nephropathy • Lupus Nephritis • Nephrology • Renal Disease • Vasculitis • MPO

Avacopan for Severe ANCA-Associated Vasculitis: Uncharted Waters (KIDNEY WEEK 2025) - "Induction therapy included pulse steroids and rituximab. AAV remains a life-threatening disease. Its increasing global prevalance and the evolving role of anti-complement therapy shed light on avacopan's potential role in more severe cases."

Acute Respiratory Distress Syndrome • ANCA Vasculitis • Hematological Disorders • Respiratory Diseases • Vasculitis

ANCA-Associated Glomerulonephritis with Severe Interstitial Infiltration (KIDNEY WEEK 2025) - "Treatment included methylprednisolone followed by prednisone, rituximab, and avacopan. Our case highlights the potential for a distinctly less common form of AAV subtype, manifesting severe anti-PR3 AAV with severe neutrophilic infiltrate. This AAV subtype warrants further investigation, as it may offer insights into the pathophysiology, treatment response, therapeutic strategies, and prognosis of PR3-positive AAV associated with AIN."

ANCA Vasculitis • Cough • Glomerulonephritis • Inflammation • Lupus Nephritis • Nephrology • Otorhinolaryngology • Pulmonary Disease • Rare Diseases • Renal Disease • Respiratory Diseases • Thrombosis • Vasculitis

Real-World Clinical Outcomes in a Large Health Care System After Avacopan Initiation for Granulomatosis with Polyangiitis (GPA) or Microscopic Polyangiitis (MPA) Involving the Kidneys (KIDNEY WEEK 2025) - "Among those with baseline UPCR >0.2 g/g, median UPCR declined from 1.4 at baseline to 0.3 g/g at 12mo (-71% change). Conclusion Real-world clinical outcomes with avacopan and improvement in kidney function were consistent with previous clinical trials."

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

Changes in Urinary sCD163 and Plasma Endotrophin Levels in Response to Treatment with Avacopan for Granulomatosis with Polyangiitis (GPA) and Microscopic Polyangiitis (MPA) with Kidney Involvement (KIDNEY WEEK 2025) - "Conclusion This secondary analysis suggests that avacopan and prednisone taper regimens affect macrophage activation, yet exhibit marked early differences in plasma endotrophin levels. Early differences could indicate that an avacopan regimen with earlier GC discontinuation may have divergent effects on fibrotic remodeling, potentially influencing clinical outcomes that need to be explored."

Fibrosis • Immunology • Inflammation • Nephrology • Rare Diseases • Renal Disease • Vasculitis • CD163

Meta-Analysis of Safety and Efficacy of Reduced Glucocorticoid Induction Regimens in ANCA-Associated Vasculitis (KIDNEY WEEK 2025) - "Interventions included reduced GC-dose, rapid GC tapering, IV methylprednisolone omission, avacopan use, and RTX/CYC combination. However, substantial heterogeneity limits strong comparative conclusions. Standardised, long-term trials are needed"

Retrospective data • ANCA Vasculitis • Infectious Disease • Vasculitis

Effect of Avacopan on Kidney Function in Maintenance Therapy of ANCA-Associated Vasculitis: Single-Center Experience in Japan (KIDNEY WEEK 2025) - "At the time of AVA initiation, the mean eGFR was 41.7±13.4 mL/min/1.73m 2 , PSL was used 6.1±2.2 mg/day, and rituximab was used as a concomitant immunosuppressant in 5 cases (63%). Compared to the time when AVA was initiated, the rate of change in eGFR at the last observation increased by 7.7 ± 9.1%, and some cases showed an improvement in renal function of up to 24.5%. Conclusion These results indicated that AVA may be effective in inhibiting the progression of renal damage and reducing GC dosage even in the maintenance phase of AAV therapy in daily practice in Japan."

Clinical • ANCA Vasculitis • Vasculitis

Immune Checkpoint Inhibitor-Associated, Pauci-immune Glomerulonephritis with a Novel Therapeutic Approach Using Apheresis, Rituximab, and Avacopan (KIDNEY WEEK 2025) - "Case Description We report the case of a 69-year-old with stage IIIB PD -L1 (program death ligand -1) positive squamous cell lung carcinoma undergoing Durvalumab therapy who developed outpatient non-oliguric AKI...Prednisone 1 mg/kg was empirically initiated, with only a partial renal response...She was readmitted 2 weeks later with upper respiratory symptoms and diagnosed with CMV viremia, leading to rituximab discontinuation and initiation of valganciclovir...Discussion This case highlights the diagnostic and therapeutic complexity of ICI-associated pauci-immune glomerulonephritis. Specifically, as it relates to early kidney biopsy in delayed steroid responsiveness, absence of PR3/MPO in ICI pauci-immune disease, and possible treatment strategies with its associated complications."

Checkpoint inhibition • Acute Kidney Injury • Glomerulonephritis • Immunology • Lung Cancer • Lupus Nephritis • Nephrology • Non Small Cell Lung Cancer • Oncology • Renal Disease • Solid Tumor • MPO

A Patient with Granulomatosis with Polyangiitis Presenting with Abdominal Pain and IgA Deposition in Cutaneous Vessel Walls, Mimicking IgA Vasculitis (KIDNEY WEEK 2025) - "Oral prednisolone was started, but within a week, kidney function deteriorated rapidly, with serum creatinine rising from 1.0 mg/dL to 6.3 mg/dL, requiring hemodialysis...The patient was treated with high-dose intravenous steroids , avacopan, rituximab, and plasma exchange therapy...IgA deposition on the skin in cases of GPA was reported to be attributed to the presence of circulating immune complexes. In cases of RPGN with IgA deposition on the skin and concurrent abdominal pain, GPA and IgA vasculitis should be considered as potential diagnoses."

Clinical • Glomerulonephritis • Inflammation • Lupus Nephritis • Nephrology • Otorhinolaryngology • Pain • Pediatrics • Rare Diseases • Renal Disease • Respiratory Diseases • Sinusitis • Vasculitis