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Cardiology519 papers

Lupus vasculitis

Last edited: 4/23/2026

Overview

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of autoimmune disorders characterized by inflammation of small blood vessels, leading to multisystem organ involvement including renal, pulmonary, and systemic manifestations 1 10 28.

Diagnosis

Key Diagnostic Criteria: Presence of ANCA (typically PR3-ANCA or MPO-ANCA), clinical features of systemic vasculitis, and often organ-specific manifestations 1 10 28.

Recommended Tests: Serological testing for ANCA, renal function tests, imaging studies (e.g., chest CT, renal ultrasound), and biopsy when indicated (e.g., renal, skin, or other affected tissues) 1 10 28.

Grading: Utilize Birmingham Vasculitis Activity Score (BVAS) or other validated scoring systems to assess disease activity 28.

Management

First-Line Treatments:

- Glucocorticoids: High-dose corticosteroids (e.g., prednisone) are foundational 1 10 28. - Immunosuppressants: Cyclophosphamide (IV or oral) or rituximab for severe disease 1 10 24 28.

Adjunctive Treatments:

- Avacopan: C5a receptor antagonist used as an adjunctive therapy to reduce glucocorticoid exposure 5 19 30 33 34. - Plasma Exchange: Considered in severe cases with rapidly progressive organ failure 29 55.

Special Populations

Pediatrics: Childhood-onset AAV requires careful monitoring; kidney transplantation can be effective with good outcomes 20.

Comorbidities: Pre-existing diabetes mellitus and PR3-ANCA positivity are predictors of relapse 18.

Elderly: Management considerations include tailored immunosuppression to mitigate side effects 28.

Key Recommendations

Initiate high-dose glucocorticoids and cyclophosphamide for severe AAV (Evidence: Strong 1 10 28).

Consider rituximab as an alternative to cyclophosphamide for maintenance therapy (Evidence: Moderate 24 28).

Use avacopan as an adjunctive therapy to reduce glucocorticoid dependency (Evidence: Moderate 5 30 33 34).

Monitor for and manage infections, especially in patients with comorbidities (Evidence: Moderate 39).

Regularly assess disease activity using validated scoring systems like BVAS (Evidence: Moderate 28).

References

Showing 100 most recent of 463 indexed papers.

1 Chang WH, Chien SC, Chien SC, Hu TY. A fatal case of ANCA-associated vasculitis resulting in multiple organ failure: Case report and literature review. Medicine 2026. link 2 Zhou Z, Yu L, Zheng J, He Y, Xia L, Jia Q et al.. Profiling of Potential Postmarket Risk Tracking and Pharmacovigilance Data for Avacopan. Clinical therapeutics 2026. link 3 Dick J, Nagai K, Koo Yuk Cheong D, Le AC, Tan DSY, Alikhan M et al.. Low dose IL-2 administration prevents but fails to treat experimental autoimmune anti-myeloperoxidase glomerulonephritis. Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association 2026. link 4 Neary E, Healey K, Clements-Baker M, Makhzoum JP, Mendel A. Management of antineutrophil cytoplasmic antibody vasculitis-associated orbital inflammatory disease: A systematic literature review. Seminars in arthritis and rheumatism 2026. link 5 Han XY, Li ZY, Chen SF, Zhao MH, Little MA, Chen M. Real-world safety assessment of avacopan in patients with antineutrophil cytoplasmic antibody-associated vasculitis. RMD open 2026. link 6 Weng YC, Chen CL. Recurrent spontaneous renal hemorrhage in end-stage renal disease with ANCA-associated vasculitis: a case-based review. Clinical rheumatology 2026. link 7 Jayne DRW, Luqmani R, Terrier B, Obergfell A, Pollet C, Boff M et al.. 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. JMIR research protocols 2026. link 8 Mekraksakit P, Vargas-Brochero MJ, Russo I, Buglioni A, Inacio A, Domingues P et al.. Monoclonal gammopathy associates with kidney failure in ANCA-associated vasculitis. Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association 2026. link 9 Miyamoto AT, Kumanogoh A, Nishide M. Current Overview of Multi-omics Analyses in Microscopic Polyangiitis and Granulomatosis with Polyangiitis. Internal medicine (Tokyo, Japan) 2026. link 10 Sagy I, Jayne DRW. Recent pathogenetic insights and therapeutic advances in ANCA-associated vasculitis. Annals of the rheumatic diseases 2026. link 11 Nawachi S, Katsuyama T, Miyawaki Y, Sakamoto-Tokunaga M, Kubota N, Terajima Y et al.. Dental infection is associated with early relapse in patients with ANCA-associated vasculitis. RMD open 2026. link 12 Fan MH, Wu J, Min CC, Meng P, Jiang C, Chen H et al.. Hemoptysis as the initial presentation of ANCA-associated vasculitis in a patient with ulcerative colitis: a case report and literature review. Clinical rheumatology 2026. link 13 Junek M, Choi S, Garner S, Rawn S, Cox G, Khalidi N et al.. Pulmonary manifestations of granulomatosis with polyangiitis and microscopic polyangiitis. Seminars in arthritis and rheumatism 2026. link 14 Lim B, Qian W, Dowling F, Salama A, Smith RM. The Effect of Intranasal Niclosamide on Nasal Symptoms in Patients with Antineutrophil Cytoplasmic Antibody-Associated Vasculitis. Arthritis & rheumatology (Hoboken, N.J.) 2026. link 15 Narváez J, Maymó P. Isolated antineutrophil cytoplasmic antibody-associated interstitial lung disease: A systematic review of an emerging condition. Autoimmunity reviews 2026. link 16 Prieto-González S, Jordà-Sánchez R, Hernández-González F, Smith RM, Jayne DR. ANCA-associated vasculitis and interstitial lung disease: challenges and open questions. European journal of internal medicine 2026. link 17 Tian M, Wang T, Gao L, Zhang X, Liu H, Zhou M et al.. Antineutrophil cytoplasmic antibody-associated vasculitis with initial gastrointestinal symptoms: case series and literature review. European journal of gastroenterology & hepatology 2026. link 18 Tabei A, Takeuchi Y, Sakairi T, Ohishi Y, Watanabe M, Nakasatomi M et al.. Pre-existing diabetes mellitus and PR3-ANCA positivity as predictors of relapse in ANCA-associated vasculitis: a retrospective cohort study. Clinical rheumatology 2026. link 19 Blondel J, Froelicher-Bournaud L, Faguer S, Coindre JP, Terrier B, Vidal M et al.. LC-MS/MS method for quantification of avacopan in human plasma from patients treated for antineutrophil cytoplasmic antibody-associated vasculitis. Journal of pharmaceutical and biomedical analysis 2026. link 20 Trivioli G, Allinovi M, Di Marcantonio E, Jawa NA, Trivelli A, Yang J et al.. Kidney Transplantation in Childhood-Onset ANCA-Associated Vasculitis: Long-Term Outcomes and Prognostic Factors. Clinical journal of the American Society of Nephrology : CJASN 2026. link 21 Rodelo-Ceballos J, Restrepo-Escobar M, Estacio-Estacio M, Orejuela-Erazo JA, Taborda-Murillo A. Kidney Prognosis in ANCA-associated Vasculitis: The ANCLA Risk Score From a Latin American Historical Cohort. Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases 2026. link 22 Brilland B, Despré M, Khatri R, Quéméneur T, Vandenbussche C, Merillon N et al.. A stronger type I interferon signature distinguishes ANCA-associated vasculitis phenotypes and predicts kidney prognosis. Kidney international 2026. link 23 Lu G, Wang X, Wang X, Zhuang H, Zhao G. Case Report: Rapid progression of inflammation-driven coronary artery lesions in a normolipidemic patient with ANCA-associated vasculitis complicated by Stanford type A aortic dissection. Frontiers in immunology 2026. link 24 Omura S, Kida T, Kitai J, Yanagida T, Nakagomi D, Abe Y et al.. Real-world effectiveness of rituximab or intravenous cyclophosphamide versus non-use in patients with severe microscopic polyangiitis and granulomatosis with polyangiitis: a retrospective cohort study of J-CANVAS. Modern rheumatology 2026. link 25 Cohen Tervaert JW. Drug- and vaccine induced ANCA-associated vasculitis: An overview. Autoimmunity reviews 2026. link 26 Kawakami E, Kamada Y, Fukui S, Jubashi T, Kojima K, Shimizu T et al.. Detection of Irpex lacteus in the Lung in a Case with Microscopic Polyangiitis-mimicking Manifestations Positive for Myeloperoxidase-antineutrophil Cytoplasmic Antibody. Internal medicine (Tokyo, Japan) 2026. link 27 Taniguchi T, Hiwa R, Iwasaka S, Shoji M, Manabe A, Kadoba K et al.. Renal outcomes in ANCA-associated vasculitis without clinically apparent kidney involvement: a multicentre REVEAL cohort study. Rheumatology (Oxford, England) 2026. link 28 Biddle K, Jade J, Wilson-Morkeh H, Adikari M, Yaghchi CA, Anastasa Z et al.. The 2025 British Society for Rheumatology management recommendations for ANCA-associated vasculitis. Rheumatology (Oxford, England) 2025. link 29 Odler B, Riedl R, Geetha D, Szpirt WM, Hawley C, Uchida L et al.. The effects of plasma exchange and glucocorticoids on early kidney function among patients with ANCA-associated vasculitis in the PEXIVAS trial. Kidney international 2025. link 30 Morales E, Rúa-Figueroa I, Callejas Rubio JL, Ávila Bernabéu A, Blanco Alonso R, Cid Xutgla MC et al.. Recommendations for the diagnosis and treatment of anti-neutrophil cytoplasmic autoantibody associated vasculitis. Nefrologia 2025. link 31 Kwon J, Choi YW, Kim H, Yoo SJ. Thoracic Manifestations of ANCA-associated Vasculitis: Review of the 2022 American College of Rheumatology-European Alliance of Associations of Rheumatology Classification Criteria. Radiographics : a review publication of the Radiological Society of North America, Inc 2025. link 32 de Groot K, Csernok E, van der Woude D. History of antineutrophil cytoplasmic autoantibodies : Milestones in rheumatology. Zeitschrift fur Rheumatologie 2025. link 33 Liang G, Liu X, Gao M, Yang B, Song Y, Liu Q. A Real-World Disproportionality Analysis of Avacopan in Anti-Neutrophil Cytoplasmic Antibodies Associated Vasculitis: Insights From FDA Adverse Event Reporting System. Pharmacology research & perspectives 2025. link 34 Yang Z, Wang C, Wei S, Liu Y, He M, Zheng C et al.. Post-marketing safety profile and clinical prioritization of adverse events with avacopan in ANCA-associated vasculitis: a FAERS pharmacovigilance analysis. Renal failure 2025. link 35 Ergun T. Urticarial vasculitis. Current opinion in rheumatology 2025. link 36 Ubara Y, Oba Y, Kurihara S, Sekine A, Yamanouchi M, Hasegawa E et al.. A case of renal limited myeloperoxidase anti-neutrophil cytoplasmic antibody-positive vasculitis treated with maintenance avacopan monotherapy. CEN case reports 2025. link 37 Katz G, Cook CE, Fu X, King AJ, Stone JH, Choi HK et al.. Defining cause of death in a contemporary cohort with ANCA-associated vasculitis (AAV): A comparison of electronic health record and death certificate data. Seminars in arthritis and rheumatism 2025. link 38 Ueda Y, Nunokawa T, Takamasu E, Okada H, Shimada K, Yokogawa N. Impact of glucocorticoids on muscle biopsy findings in antineutrophil cytoplasmic antibody-associated vasculitis. Clinical rheumatology 2025. link 39 Luo W, Liu C, Zhang L, Tang J, Chen J, Zhao Y et al.. Characteristics and risk factors for infection in patients with ANCA-associated vasculitis: A systematic review and meta-analysis. Autoimmunity reviews 2025. link 40 Yoon T, Ha JW, Park YB, Lee SW. Serum Wnt3A levels are significantly associated with cross-sectional vasculitis activity and end-stage kidney disease during follow-up of patients with antineutrophil cytoplasmic antibody-associated vasculitis. Clinical and experimental rheumatology 2025. link 41 Shimamura T, Furusawa H, Ejima M, Ozawa A, Adachi T, Tateishi U et al.. Clinical characteristics and prognostic factors for MPO-ANCA positive interstitial lung disease: A comparative study of ANCA associated vasculitis (AAV)-ILD and pulmonary limited vasculitis. Respiratory investigation 2025. link 42 Rothermel ND, Vera Ayala C, Gonçalo M, Fok JS, Herzog LS, Kocatürk E et al.. Managing Urticarial Vasculitis: A Clinical Decision-Making Algorithm Based on Expert Consensus. American journal of clinical dermatology 2025. link 43 Bhonsle A, Coughlan T, Graven R, Bussa P, Gingold M, Polkinghorne KR et al.. Relationships between patient and physician global assessments in anti-neutrophil cytoplasmic antibody-associated vasculitis. Clinical rheumatology 2025. link 44 Agarwal A, Al Ali S, Mubashir A, Shkoukani M, Belletti M, Saeed M et al.. Serpiginous-Like Choroiditis with ANCA-PR3 Vasculitis in a Patient from Endemic Country: A Complex Phenotypic, Immunological and Epidemiological Puzzle. Ocular immunology and inflammation 2025. link 45 Palacz W, Lichołai S, Musiał J, Wawrzycka-Adamczyk K, Ślusarczyk G, Strug B et al.. Ontology-based integration and querying of heterogeneous rare disease data sources - POLVAS perspective. Computers in biology and medicine 2025. link 46 Li W, Hao W, Cao W, Wang Y, Li Y, Ma Y et al.. Serum CXCL6 as a biomarker for diagnosing and predicting progression of interstitial lung disease in ANCA-associated vasculitis. Arthritis research & therapy 2025. link 47 Guillet C, Hauser X, Stillhard A, Schmid-Grendelmeier P, Kolm I. Nodular Vasculitis: Retrospective Study of an Uncommon Disease in a Non-Tuberculosis Endemic Country with Focus on Treatment Modalities and Efficacy. Dermatology (Basel, Switzerland) 2025. link 48 Floege J, Jayne DRW, Sanders JF, Tesar V, Balk EM, Gordon CE et al.. Executive summary of the KDIGO 2024 Clinical Practice Guideline for the Management of ANCA-Associated Vasculitis. Kidney international 2024. link 49 Hellmich B, Sanchez-Alamo B, Schirmer JH, Berti A, Blockmans D, Cid MC et al.. EULAR recommendations for the management of ANCA-associated vasculitis: 2022 update. Annals of the rheumatic diseases 2024. link 50 Floyd L, Ahmed M, Morris AD, Nixon AC, Mitra S, Dhaygude A et al.. A systematic review of patient-reported outcome measures in patients with anti-neutrophil cytoplasmic antibody associated vasculitis. Rheumatology (Oxford, England) 2024. link 51 Dhooria A, Naidu GSRSNK, Misra DP, Pinto B, Adarsh MB, Jha S et al.. Indian Rheumatology Association guidelines for the management of ANCA associated vasculitis. Autoimmunity reviews 2024. link 52 Farrugia S, Cachia M, Betts A, Clark E. Nodular vasculitis (erythema induratum) associated with systemic minocycline. The Australasian journal of dermatology 2024. link 53 Nettleton E, Sattui SE, Wallace Z, Putman M. Incidence of Pneumocystis Jiroveci Pneumonia in Patients With ANCA-Associated Vasculitis Initiating Therapy With Rituximab or Cyclophosphamide. Arthritis care & research 2024. link 54 Yoon T, Ha JW, Pyo JY, Song JJ, Park YB, Ahn SS et al.. Circulating cold-inducible RNA-binding protein levels in microscopic polyangiitis and granulomatosis with polyangiitis : Correlation with disease activity. Zeitschrift fur Rheumatologie 2024. link 55 Chalkia A, Petras D. Plasma exchange in patients with ANCA-associated vasculitis. Transfusion and apheresis science : official journal of the World Apheresis Association : official journal of the European Society for Haemapheresis 2024. link 56 Duarte R, Trigo F, Luz I, Santos P. Small-vessel vasculitis leading to severe acute kidney injury after ipilimumab: a case report. Melanoma research 2024. link 57 Muhammad A, Xiao Z, Lin W, Zhang Y, Meng T, Ning J et al.. Acute interstitial nephritis caused by ANCA-associated vasculitis: a case based review. Clinical rheumatology 2024. link 58 Lee YJ, Ahn SM, Oh JS, Kim YG, Lee CK, Yoo B et al.. Relapse in patients with antineutrophil cytoplasmic antibody-associated vasculitis undergoing dialysis: a single-centre retrospective study in South Korea. Clinical and experimental rheumatology 2024. link 59 Ono N, Kai T, Takeyama Y, Inoue Y, Ueda N, Nagano S et al.. Recent advances in the treatment strategy for AAV improved outcomes with intensive GC tapering. International journal of rheumatic diseases 2024. link 60 Atrens AD, Hutton H, O'Sullivan E, Moore J, Madhan K. Avacopan as an additional therapeutic intervention to promote renal recovery in severe ANCA-associated vasculitis: A case report. Nephrology (Carlton, Vic.) 2024. link 61 Baliga S, Yadav S, Sagdeo P, Balakrishnan C. Invasive fungal infection in ANCA-associated vasculitis: Between the Devil and Deep blue sea. Case series and review of the literature. Clinical rheumatology 2024. link 62 Tan LW, Wan JL, Zhu CH, Xu H, Xia ZK, Chen LZ et al.. Risk factors for renal outcomes in children with antineutrophil cytoplasmic antibody-associated vasculitis: a nationwide retrospective study in China. World journal of pediatrics : WJP 2024. link 63 Spadaro JZ, Sinard J, Habib L. Bilateral dacryoadenitis as the initial presentation of ANCA-associated vasculitis in a pediatric patient. Orbit (Amsterdam, Netherlands) 2024. link 64 Sayer M, Chapman GB, Thomas M, Dhaun N. Cardiovascular Disease in Anti-neutrophil Cytoplasm Antibody-Associated Vasculitis. Current rheumatology reports 2024. link 65 van Leeuwen JR, Penne EL, Rabelink T, Knevel R, Teng YKO. Using an artificial intelligence tool incorporating natural language processing to identify patients with a diagnosis of ANCA-associated vasculitis in electronic health records. Computers in biology and medicine 2024. link 66 Cohen SP, Wodarcyk AJ, Wong A, Patterson KC, Lyons MI, Barnes A et al.. Impact of concurrent glomerulonephritis on outcomes of diffuse alveolar haemorrhage in antineutrophil cytoplasmic antibody-associated vasculitis. Clinical and experimental rheumatology 2024. link 67 Oleson I, Fecker A, Richardson K, Bauer A, Andeen NK, Kung VL. ANCA-associated kidney disease preceded by orbital pseudotumor. Pediatric nephrology (Berlin, Germany) 2024. link 68 Zhou P, Gao L, Li Z, Que C, Li H, Ma J et al.. Clinical features and outcomes of small airway disease in ANCA-associated vasculitis. Respirology (Carlton, Vic.) 2024. link 69 Oakman G, Bach CA, Ong C. Incidence of anti-neutrophil cytoplasmic antibody-associated renal vasculitis: a retrospective study in rural and regional Victoria, Australia. Internal medicine journal 2024. link 70 Yu M, Yang Y, Zhang J, Liu R, Huang L, Wu J et al.. Association between . Immunological investigations 2024. link 71 Ogrič M, Švec T, Poljšak KM, Žigon P, Hočevar A, Čučnik S. Verification, implementation and harmonization of automated chemiluminescent immunoassays for MPO- and PR3-ANCA detection. Clinical chemistry and laboratory medicine 2024. link 72 Miyake H, Tanabe K, Yamaji S, Kihara T. Early transition to avacopan from glucocorticoids applied during induction therapy for microscopic polyangiitis with rapidly progressive glomerulonephritis. CEN case reports 2024. link 73 Sanchez-Alamo B, Schirmer JH, Hellmich B, Jayne D, Monti S, Tomasson G et al.. Systematic literature review informing the 2022 update of the EULAR recommendations for the management of ANCA-associated vasculitis (AAV): Part 2 - Treatment of eosinophilic granulomatosis with polyangiitis and diagnosis and general management of AAV. RMD open 2023. link 74 Schirmer JH, Sanchez-Alamo B, Hellmich B, Jayne D, Monti S, Luqmani RA et al.. Systematic literature review informing the 2022 update of the EULAR recommendations for the management of ANCA-associated vasculitis (AAV): part 1-treatment of granulomatosis with polyangiitis and microscopic polyangiitis. RMD open 2023. link 75 Krishnan A, Walsh M, Collister D. Management of antineutrophil cytoplasmic antibody-associated vasculitis: a changing tide. Current opinion in nephrology and hypertension 2023. link 76 Yazici H, Tascilar K, Yazici Y. 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria sets for three types of antineutrophilic cytoplasmic antibody-associated vasculitis. Current opinion in rheumatology 2023. link 77 Pyo JY, Lee LE, Park YB, Lee SW. Comparison of the 2022 ACR/EULAR Classification Criteria for Antineutrophil Cytoplasmic Antibody-Associated Vasculitis with Previous Criteria. Yonsei medical journal 2023. link 78 Magri SJ, Ugarte-Gil MF, Brance ML, Flores-Suárez LF, Fernández-Ávila DG, Scolnik M et al.. Pan American League of Associations for Rheumatology Guidelines for the treatment of ANCA-associated vasculitis. The Lancet. Rheumatology 2023. link00128-5) 79 Antovic A, Bruchfeld A, Ekland J, Lövström B, Hugelius A, Börjesson O et al.. Risks and treatment related aspects of COVID-19 infection in patients with ANCA-associated vasculitis. Scandinavian journal of rheumatology 2023. link 80 Jiang C, Liu J, Wang H, Liu H, Fu Z, Li M et al.. Anti-neutrophil cytoplasmic antibody patterns can predict clinical relapse in ANCA-associated vasculitis: overall population and subgroups. Clinical and experimental rheumatology 2023. link 81 Osman M, Cohen Tervaert JW, Pagnoux C. Avacopan for the treatment of ANCA-associated vasculitis: an update. Expert review of clinical immunology 2023. link 82 Ahn JK, Hwang J, Choi CB, Seo GH. Risk of acute myocardial infarction, stroke, and venous thromboembolism among patients with anti-neutrophil cytoplasmic antibody-associated vasculitis in South Korea: A nationwide population-based study. Joint bone spine 2023. link 83 Garbe N, Schäfer C, Pilz A, Schmidt F, Raberger K, Wienke A et al.. The impact of a structured one-day seminar on disease-specific knowledge, lifestyle habits and disease impairment in ANCA-associated vasculitis. Results of a randomized, controlled study. Scandinavian journal of rheumatology 2023. link 84 Shakshouk H, Gibson LE. Cutaneous manifestations of ANCA-associated vasculitis: a retrospective review of 211 cases with emphasis on clinicopathologic correlation and ANCA status. International journal of dermatology 2023. link 85 Liu Y, Zhang X, Ji Z, Ma L, Chen H, Jiang L. Circular RNA expression profiles and identification of hsa_circ_0028381 as a potential biomarker of anti-neutrophil cytoplasmic antibody-associated vasculitis. Clinical and experimental rheumatology 2023. link 86 Singh J, Minz RW, Saikia B, Nada R, Sharma A, Jha S et al.. Diminished PD-L1 regulation along with dysregulated T lymphocyte subsets and chemokine in ANCA-associated vasculitis. Clinical and experimental medicine 2023. link 87 Gamerith G, Mildner F, Merkel PA, Harris K, Cooney L, Lim N et al.. Association of baseline soluble immune checkpoints with the risk of relapse in PR3-ANCA vasculitis following induction of remission. Annals of the rheumatic diseases 2023. link 88 Auanassova A, Yessirkepov M, Zimba O, Gasparyan AY, Joshi M, Agarwal V et al.. Physicians' perceptions about antineutrophil cytoplasmic antibody-associated vasculitis: an online survey report in the time of the COVID-19 pandemic. Clinical rheumatology 2023. link 89 Gao R, Wu Z, Xu X, Pu J, Pan S, Zhang Y et al.. Predictors of poor prognosis in ANCA-associated vasculitis (AAV): a single-center prospective study of inpatients in China. Clinical and experimental medicine 2023. link 90 Jonasdottir AD, Antovic A, Qureshi AR, Nordin A, Malmström V, Gunnarsson I et al.. Pentraxin-3 - a potential biomarker in ANCA-associated vasculitis. Scandinavian journal of rheumatology 2023. link 91 Schaap CM, Krol RM, Remmelts HHF, Klaasen R, Hagen EC, Spierings J et al.. The effect of nasal Staphylococcus aureus colonization and antibiotic treatment on disease activity in ANCA-associated vasculitis: a retrospective cohort study in the Netherlands. Rheumatology international 2023. link 92 Guo Q, Yu L, Zhang X, Shang J, Duan X. Analysis of the risk factors for end‑stage renal disease and mortality in ANCA-associated vasculitis: a study from a single center of the Chinese Rheumatism Data Center. Clinical rheumatology 2023. link 93 Akao S, Yamagiwa G, Hazue R, Rokutanda R, Suzuki T. Kidney biopsy in patients with antineutrophil cytoplasmic antibody-associated vasculitis with mild renal abnormality. CEN case reports 2023. link 94 Baier E, Kluge IA, Hakroush S, Tampe D, Tampe B. Leukocyturia and hematuria enable non-invasive differentiation of Bowman's capsule rupture severity in PR3-ANCA glomerulonephritis. Journal of nephrology 2023. link 95 Sims C, Golenbiewski J, Eudy AM, Allen NB, Clowse MEB. Hospital Admissions and Mortality in Patients With Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis. Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases 2023. link 96 Liu R, Li M, Zhang L, Wang Y, Zhang Y, Li W et al.. Utility of the neutrophil-to-lymphocyte ratio in the prediction of anti-neutrophil cytoplasmic antibody-associated vasculitis with infection. Clinical rheumatology 2023. link 97 Wang J, Xu Y, Wang G. ANCA-associated vasculitis with abdominal aortic aneurysm : Case report and literature review. Vascular 2023. link 98 Pecher AC, Henes M, Henes JC. Optimal management of ANCA-associated vasculitis before and during pregnancy: current perspectives. Archives of gynecology and obstetrics 2023. link 99 Alamoudi WA, Sollecito TP, Stoopler ET, France K. Oral manifestations of anti-neutrophil cytoplasmic antibody-associated vasculitis: an update and narrative review of the literature. Oral surgery, oral medicine, oral pathology and oral radiology 2023. link 100 Maskery MP, Whittam D, Nawaraj S, Chakraborti S, Arunachalam C, Munavvar M et al.. Myeloperoxidase-positive ANCA-associated vasculitis presenting as myalgia, proximal weakness and a normal CK. Practical neurology 2023. link

Original source

[1]
A fatal case of ANCA-associated vasculitis resulting in multiple organ failure: Case report and literature review. — Chang WH, Chien SC, Chien SC, Hu TY Medicine (2026)
[2]
Profiling of Potential Postmarket Risk Tracking and Pharmacovigilance Data for Avacopan. — Zhou Z, Yu L, Zheng J, He Y, Xia L, Jia Q et al. Clinical therapeutics (2026)
[3]
Low dose IL-2 administration prevents but fails to treat experimental autoimmune anti-myeloperoxidase glomerulonephritis. — Dick J, Nagai K, Koo Yuk Cheong D, Le AC, Tan DSY, Alikhan M et al. Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association (2026)
[4]
Management of antineutrophil cytoplasmic antibody vasculitis-associated orbital inflammatory disease: A systematic literature review. — Neary E, Healey K, Clements-Baker M, Makhzoum JP, Mendel A Seminars in arthritis and rheumatism (2026)
[5]
Real-world safety assessment of avacopan in patients with antineutrophil cytoplasmic antibody-associated vasculitis. — Han XY, Li ZY, Chen SF, Zhao MH, Little MA, Chen M RMD open (2026)
[6]
Recurrent spontaneous renal hemorrhage in end-stage renal disease with ANCA-associated vasculitis: a case-based review. — Weng YC, Chen CL Clinical rheumatology (2026)
[7]
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. — Jayne DRW, Luqmani R, Terrier B, Obergfell A, Pollet C, Boff M et al. JMIR research protocols (2026)
[8]
Monoclonal gammopathy associates with kidney failure in ANCA-associated vasculitis. — Mekraksakit P, Vargas-Brochero MJ, Russo I, Buglioni A, Inacio A, Domingues P et al. Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association (2026)
[9]
Current Overview of Multi-omics Analyses in Microscopic Polyangiitis and Granulomatosis with Polyangiitis. — Miyamoto AT, Kumanogoh A, Nishide M Internal medicine (Tokyo, Japan) (2026)
[10]
Recent pathogenetic insights and therapeutic advances in ANCA-associated vasculitis. — Sagy I, Jayne DRW Annals of the rheumatic diseases (2026)
[11]
Dental infection is associated with early relapse in patients with ANCA-associated vasculitis. — Nawachi S, Katsuyama T, Miyawaki Y, Sakamoto-Tokunaga M, Kubota N, Terajima Y et al. RMD open (2026)
[12]
Hemoptysis as the initial presentation of ANCA-associated vasculitis in a patient with ulcerative colitis: a case report and literature review. — Fan MH, Wu J, Min CC, Meng P, Jiang C, Chen H et al. Clinical rheumatology (2026)
[13]
Pulmonary manifestations of granulomatosis with polyangiitis and microscopic polyangiitis. — Junek M, Choi S, Garner S, Rawn S, Cox G, Khalidi N et al. Seminars in arthritis and rheumatism (2026)
[14]
The Effect of Intranasal Niclosamide on Nasal Symptoms in Patients with Antineutrophil Cytoplasmic Antibody-Associated Vasculitis. — Lim B, Qian W, Dowling F, Salama A, Smith RM Arthritis & rheumatology (Hoboken, N.J.) (2026)
[15]
Isolated antineutrophil cytoplasmic antibody-associated interstitial lung disease: A systematic review of an emerging condition. — Narváez J, Maymó P Autoimmunity reviews (2026)
[16]
ANCA-associated vasculitis and interstitial lung disease: challenges and open questions. — Prieto-González S, Jordà-Sánchez R, Hernández-González F, Smith RM, Jayne DR European journal of internal medicine (2026)
[17]
Antineutrophil cytoplasmic antibody-associated vasculitis with initial gastrointestinal symptoms: case series and literature review. — Tian M, Wang T, Gao L, Zhang X, Liu H, Zhou M et al. European journal of gastroenterology & hepatology (2026)
[18]
Pre-existing diabetes mellitus and PR3-ANCA positivity as predictors of relapse in ANCA-associated vasculitis: a retrospective cohort study. — Tabei A, Takeuchi Y, Sakairi T, Ohishi Y, Watanabe M, Nakasatomi M et al. Clinical rheumatology (2026)
[19]
LC-MS/MS method for quantification of avacopan in human plasma from patients treated for antineutrophil cytoplasmic antibody-associated vasculitis. — Blondel J, Froelicher-Bournaud L, Faguer S, Coindre JP, Terrier B, Vidal M et al. Journal of pharmaceutical and biomedical analysis (2026)
[20]
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