Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2025 Aug 1:17:537-546.
doi: 10.2147/CEOR.S529853. eCollection 2025.

Clinical Burden and Costs of Anti-Neutrophil Cytoplasmic Antibody-ANCA-Associated Vasculitis: Main Findings from REDCap Registry of a University Hospital in Spain

Affiliations

Clinical Burden and Costs of Anti-Neutrophil Cytoplasmic Antibody-ANCA-Associated Vasculitis: Main Findings from REDCap Registry of a University Hospital in Spain

Juliana Draibe et al. Clinicoecon Outcomes Res. .

Abstract

Purpose: Anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) are rare chronic autoimmune diseases, potentially fatal, with frequent relapses. They are associated with vital organ damage, especially renal, often resulting in end-stage renal disease. While current standard of care with immunosuppressants has improved renal function and survival, the main risks for patients under life-long immunosuppression are infections and other concomitant diseases. This study evaluated the burden of AAV using patient-level data from a disease-specific registry.

Patients and methods: The cohort of incident AVV patients (2013-2022) in the REDCap registry in a university hospital in Spain was studied. Patients with Granulomatosis with Polyangiitis (GPA), Microscopic Polyangiitis (MPA) and Eosinophilic Granulomatosis with Polyangiitis (EGPA) with at least one year of follow-up (or deceased during the period) were included. Clinical outcomes, including Birmingham Vasculitis Activity Score (BVAS) and healthcare resource consumption were analysed for the first year after diagnosis. Mean annual costs were calculated using unitary costs from the hospital accounting department.

Results: Seventy-five patients (12% EGPA, 32% GPA, and 56% MPA) were included. Fifty-two percent were women. Mean age at diagnosis was 65.20±14.70 years. At baseline, mean BVAS was 17.35±5.70, 93.33% of patients showed renal affectation, mean estimated glomerular filtration rate was 33.32±29.93mL/min/1.73m2. As induction treatment, 62.67% received methylprednisolone, 37.33% rituximab, 25.33% cyclophosphamide, 14.67% rituximab plus cyclophosphamide, 34.67% plasmapheresis. During the first year after diagnosis, 17.33% relapsed and 78.67% had at least 1 hospitalisation; 97.33% received steroids; 13.33% were on dialysis at some point; one patient received a kidney transplant; 46.67% presented infections and 28% suffered corticosteroid-associated complications; 4 patients died, being 50% of deaths treatment-related. The highest observed mean cost per patient for the first year was €11,647.95 for hospital care.

Conclusion: This study revealed a considerable burden of AAV, as evidenced by high rates of hospitalisation, relapses, and the need for intensive medical interventions.

Keywords: anti-neutrophil cytoplasmic antibody-associated vasculitis; disease burden; registry; treatment outcome; vasculitis.

PubMed Disclaimer

Conflict of interest statement

ARA is an employee of CSL Vifor and may hold shares and/or stock options in the company. HDC, CC and JC are consultants of Axentiva Solutions, which received consultancy fees from CSL Vifor, Spain and, during the conduct of this study, from other pharmaceutical companies (Amgen, Pfizer, and Biogen) in unrelated projects. JD, FGP and LMV report having received a consulting fee from Axentiva Solutions for collaboration in this study and, during the conduct of this study, from other CSL Vifor-funded projects. The authors report no other conflicts of interest in this work.

Figures

Figure 1
Figure 1
Main comorbidities at baseline.
Figure 2
Figure 2
Main clinical outcomes at one year of follow-up (% of patients).
Figure 3
Figure 3
(a) Main types of infections (%) at one-year follow-up (n=35); (b) Main types of clinical Steroid-associated complications (%) at one-year follow-up (n=21).
Figure 4
Figure 4
Change in eGFR according to categories, from baseline to 1 year of follow-up (%).
Figure 5
Figure 5
Main resource consumption items during the first year of follow-up.

Similar articles

References

    1. Casal-Moura M, Branco C, Martins-Martinho J, et al. A glance into the future of anti-neutrophil cytoplasmic antibody-associated vasculitis. Ther Adv Musculoskelet Dis. 2022;14:1759720X2211259. doi: 10.1177/1759720X221125979 - DOI - PMC - PubMed
    1. Koike H, Furukawa S, Mouri N, Fukami Y, Iijima M, Katsuno M. Early ultrastructural lesions of anti-neutrophil cytoplasmic antibody- versus complement-associated vasculitis. Neuropathology. 2022;42(5):420–429. doi: 10.1111/NEUP.12821 - DOI - PubMed
    1. Massicotte-Azarniouch D, Herrera CA, Jennette JC, Falk RJ, Free ME. Mechanisms of vascular damage in ANCA vasculitis. Semin Immunopathol. 2022;44(3):325. doi: 10.1007/S00281-022-00920-0 - DOI - PMC - PubMed
    1. Gibson A, Stamp LK, Chapman PT, O’Donnell JL. The epidemiology of Wegener’s granulomatosis and microscopic polyangiitis in a Southern Hemisphere region. Rheumatology. 2006;45(5):624–628. doi: 10.1093/rheumatology/kei259 - DOI - PubMed
    1. Kitching AR, Anders HJ, Basu N, et al. ANCA-associated vasculitis. Nat Rev Dis Primers. 2020;6(1):71. doi: 10.1038/S41572-020-0204-Y - DOI - PubMed

LinkOut - more resources