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
Multicenter Study
. 2024 Dec 24;45(1):7.
doi: 10.1007/s00296-024-05757-4.

Remission induction therapies and long-term outcomes in granulomatosis with polyangiitis and microscopic polyangiitis: real-world data from a European cohort

Stefan Krämer  1 , Kristian Vogt  1 , Theresa Maria Schreibing  1 , Martin Busch  2 , Tobias Schmitt  2 , Raoul Bergner  3 , Sebastian Mosberger  3 , Thomas Neumann #  4 , Thomas Rauen #  5
Affiliations
Multicenter Study

Remission induction therapies and long-term outcomes in granulomatosis with polyangiitis and microscopic polyangiitis: real-world data from a European cohort

Stefan Krämer et al. Rheumatol Int. .

Abstract

To explore disease characteristics, renal involvement and induction treatment strategies over the last decades and evaluate relapse rates and renal outcomes in ANCA-associated vasculitides (AAV). We retrospectively analyzed remission, relapse rates and the occurrence of the composite endpoint (comprising death and renal failure) in newly diagnosed AAV cases in four tertial referral centers in Germany and Switzerland diagnosed between 1999 and 2022. Hazard ratios were computed by Cox proportional hazard and Kaplan-Meier curves were plotted to compare therapeutic strategies after propensity-matching. In our cohort of 358 AAV patients, 203 (58.1%) were classified as granulomatosis with polyangiitis (GPA) based on the novel 2022 ACR/EULAR criteria, 139 (38.8%) as microscopic polyangiitis (MPA). The proportion of MPA cases among all AAV patients increased from 28.9% between 1999 and 2013 up to 46.7% thereafter. Cyclophosphamide (CYC) was chosen most frequently for remission induction (74.8% before 2013 and 57.3% thereafter), whereas the use of rituximab (RTX) increased from 5 to 26% within these periods. GPA patients had a higher relapse rate as compared to MPA patients (41.3% vs. 25.9%, p = 0.006). However, in AAV patients with renal involvement, renal events (i.e. end-stage kidney disease or a persistent drop in the estimated glomerular filtration rate (eGFR) below 15 ml/min/1.73 m2) occurred more frequently in MPA patients, particularly under RTX treatment as compared to matched CYC counterparts (11.8% vs. 7.5%, p = 0.011). In our cohort, GPA patients exhibited frequent relapses, advocating for a more intense and extended treatment. MPA patients had lower relapse rates, however, RTX was less effective to prevent renal endpoints in MPA as compared to CYC, highlighting the need to further investigate additional treatment strategies.

Keywords: AAV; ANCA; Cyclophosphamide; GPA; MPA; Relapse; Renal outcome; Rituximab; Vasculitis.

PubMed Disclaimer

Conflict of interest statement

Declarations. Conflict of interests: S. Krämer, K. Vogt, T. Schreibing, T. Schmitt, S. Mosberger: no disclosures. T. Rauen, M. Busch, R. Bergner, T. Neumann: received consultancy honoraria from Vifor Pharma. Ethical approval: This retrospective analysis was approved by the involved local ethic committees (Aachen: EK 164/19, St. Gallen: 2020-01350, Jena: 2020-1837-Daten, Ludwigshafen: 2020-14843-Retrospective).

Figures

Fig. 1
Fig. 1
Classification and therapy for induction over time
Fig. 2
Fig. 2
Relapse events regarding classification (A) and therapy for induction (B)
Fig. 3
Fig. 3
Risk factors for renal events and composite endpoint analyzes in all AAV patients with renal involvement under RTX as compared to propensity matched CYC counterparts in A all cases, B GPA patients and C MPA patients
See this image and copyright information in PMC

References

    1. Treppo E, Binutti M, Agarinis R, De Vita S, Quartuccio L (2021) Rituximab induction and maintenance in ANCA-associated vasculitis: state of the art and future perspectives. J Clin Med 10(17):10. 10.3390/JCM10173773 - DOI - PMC - PubMed
    1. Stone JH, Merkel PA, Spiera R, Seo P, Langford CA, Hoffman GS et al (2010) Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N Engl J Med 363(4):221–232. 10.1056/nejmoa0909905 - DOI - PMC - PubMed
    1. McAdoo SP, Medjeral-Thomas N, Gopaluni S, Tanna A, Mansfield N, Galliford J et al (2019) Long-term follow-up of a combined rituximab and cyclophosphamide regimen in renal anti-neutrophil cytoplasm antibody-associated vasculitis. Nephrol Dialy Transplant 34(1):63. 10.1093/NDT/GFX378 - DOI - PMC - PubMed
    1. Robson JC, Grayson PC, Ponte C, Suppiah R, Craven A, Judge A et al (2022) 2022 American college of rheumatology/European alliance of associations for rheumatology classification criteria for granulomatosis with polyangiitis. Arthritis Rheumatol 74(3):393–399. 10.1002/art.41986 - DOI - PubMed
    1. Suppiah R, Robson JC, Grayson PC, Ponte C, Craven A, Khalid S et al (2022) 2022 American college of rheumatology/European alliance of associations for rheumatology classification criteria for microscopic polyangiitis. Arthritis Rheumatol 74(3):400–406. 10.1002/art.41983 - DOI - PubMed

Publication types