Prognosis of microscopic polyangiitis is well predictable in the first 2 weeks of treatment

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Abstract

Background

Kidney and life outcomes remain unsatisfactory in patients with microscopic polyangiitis (MPA). Appropriate treatment intensity must be provided to the appropriate patients. To identify severe cases early, we investigated the factors related to kidney and life outcomes.

Methods

We included patients diagnosed with MPA based on myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA) positivity and kidney histopathology results after kidney biopsies between January 1, 2021, and May 11, 2023, at 10 affiliated centers, including our hospital. Death, maintenance dialysis, and estimated glomerular filtration rate (eGFR) < 15 after 6 months of treatment were defined as poor prognosis groups, and factors associated with these conditions were investigated.

Results

We included 84 (36 men and 48 women) patients in this study. Median age was 73.8 (interquartile range: 71–81) years. After 6 months of treatment, the proportion of patients in the poor prognosis group was 16.7 %, with a mortality of 7.1 % and a poor kidney prognosis rate of 9.5 %. Area under the receiver operating characteristic curve showed that eGFR at 2 weeks had a comparable prognostic performance equal as eGFR at 4 weeks (area under the curve: 0.875 and 0.896, respectively). After adjustment by various factors, eGFR at 2 weeks was related with prognosis significantly (p = 0.031).

Conclusion

Kidney function 2 weeks after the start of treatment for MPA can predict prognosis.

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Most cited references 12

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Rituximab versus cyclophosphamide for ANCA-associated vasculitis.

John Stone, Peter Merkel, Robert Spiera … (2010)

Cyclophosphamide and glucocorticoids have been the cornerstone of remission-induction therapy for severe antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis for 40 years. Uncontrolled studies suggest that rituximab is effective and may be safer than a cyclophosphamide-based regimen. We conducted a multicenter, randomized, double-blind, double-dummy, noninferiority trial of rituximab (375 mg per square meter of body-surface area per week for 4 weeks) as compared with cyclophosphamide (2 mg per kilogram of body weight per day) for remission induction. Glucocorticoids were tapered off; the primary end point was remission of disease without the use of prednisone at 6 months. Nine centers enrolled 197 ANCA-positive patients with either Wegener's granulomatosis or microscopic polyangiitis. Baseline disease activity, organ involvement, and the proportion of patients with relapsing disease were similar in the two treatment groups. Sixty-three patients in the rituximab group (64%) reached the primary end point, as compared with 52 patients in the control group (53%), a result that met the criterion for noninferiority (P<0.001). The rituximab-based regimen was more efficacious than the cyclophosphamide-based regimen for inducing remission of relapsing disease; 34 of 51 patients in the rituximab group (67%) as compared with 21 of 50 patients in the control group (42%) reached the primary end point (P=0.01). Rituximab was also as effective as cyclophosphamide in the treatment of patients with major renal disease or alveolar hemorrhage. There were no significant differences between the treatment groups with respect to rates of adverse events. Rituximab therapy was not inferior to daily cyclophosphamide treatment for induction of remission in severe ANCA-associated vasculitis and may be superior in relapsing disease. (Funded by the National Institutes of Allergy and Infectious Diseases, Genentech, and Biogen; ClinicalTrials.gov number, NCT00104299.) 2010 Massachusetts Medical Society

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ANCA-associated vasculitis

A. Kitching, Hans-Joachim Anders, Neil Basu … (2020)

The anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAVs) are a group of disorders involving severe, systemic, small-vessel vasculitis and are characterized by the development of autoantibodies to the neutrophil proteins leukocyte proteinase 3 (PR3-ANCA) or myeloperoxidase (MPO-ANCA). The three AAV subgroups, namely granulomatosis with polyangiitis (GPA), microscopic polyangiitis and eosinophilic GPA (EGPA), are defined according to clinical features. However, genetic and other clinical findings suggest that these clinical syndromes may be better classified as PR3-positive AAV (PR3-AAV), MPO-positive AAV (MPO-AAV) and, for EGPA, by the presence or absence of ANCA (ANCA+ or ANCA-, respectively). Although any tissue can be involved in AAV, the upper and lower respiratory tract and kidneys are most commonly and severely affected. AAVs have a complex and unique pathogenesis, with evidence for a loss of tolerance to neutrophil proteins, which leads to ANCA-mediated neutrophil activation, recruitment and injury, with effector T cells also involved. Without therapy, prognosis is poor but treatments, typically immunosuppressants, have improved survival, albeit with considerable morbidity from glucocorticoids and other immunosuppressive medications. Current challenges include improving the measures of disease activity and risk of relapse, uncertainty about optimal therapy duration and a need for targeted therapies with fewer adverse effects. Meeting these challenges requires a more detailed knowledge of the fundamental biology of AAV as well as cooperative international research and clinical trials with meaningful input from patients.

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Rituximab versus cyclophosphamide in ANCA-associated renal vasculitis.

Adam D Walsh, Caroline Savage, K Westman … (2010)

Cyclophosphamide induction regimens for antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis are effective in 70 to 90% of patients, but they are associated with high rates of death and adverse events. Treatment with rituximab has led to remission rates of 80 to 90% among patients with refractory ANCA-associated vasculitis and may be safer than cyclophosphamide regimens. We compared rituximab with cyclophosphamide as induction therapy in ANCA-associated vasculitis. We randomly assigned, in a 3:1 ratio, 44 patients with newly diagnosed ANCA-associated vasculitis and renal involvement to a standard glucocorticoid regimen plus either rituximab at a dose of 375 mg per square meter of body-surface area per week for 4 weeks, with two intravenous cyclophosphamide pulses (33 patients, the rituximab group), or intravenous cyclophosphamide for 3 to 6 months followed by azathioprine (11 patients, the control group). Primary end points were sustained remission rates at 12 months and severe adverse events. The median age was 68 years, and the glomerular filtration rate (GFR) was 18 ml per minute per 1.73 m(2) of body-surface area. A total of 25 patients in the rituximab group (76%) and 9 patients in the control group (82%) had a sustained remission (P=0.68). Severe adverse events occurred in 14 patients in the rituximab group (42%) and 4 patients in the control group (36%) (P=0.77). Six of the 33 patients in the rituximab group (18%) and 2 of the 11 patients in the control group (18%) died (P=1.00). The median increase in the GFR between 0 and 12 months was 19 ml per minute in the rituximab group and 15 ml per minute in the control group (P=0.14). A rituximab-based regimen was not superior to standard intravenous cyclophosphamide for severe ANCA-associated vasculitis. Sustained-remission rates were high in both groups, and the rituximab-based regimen was not associated with reductions in early severe adverse events. (Funded by Cambridge University Hospitals National Health Service Foundation Trust and F. Hoffmann-La Roche; Current Controlled Trials number, ISRCTN28528813.) 2010 Massachusetts Medical Society

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Author and article information

Contributors

Kazuhiro Furuhashi:

ORCID: http://orcid.org/0000-0003-2476-688X

furu13@med.nagoya-u.ac.jp

Journal

Journal ID (nlm-ta): Clin Exp Nephrol

Journal ID (iso-abbrev): Clin Exp Nephrol

Title: Clinical and Experimental Nephrology

Publisher: Springer Nature Singapore (Singapore )

ISSN (Print): 1342-1751

ISSN (Electronic): 1437-7799

Publication date (Electronic): 8 June 2024

Publication date PMC-release: 8 June 2024

Publication date (Print): 2024

Volume: 28

Issue: 7

Pages: 701-706

Affiliations

[1 ]Department of Nephrology, Nagoya University Graduate School of Medicine, ( https://ror.org/04chrp450) Nagoya, Aichi Japan

[2 ]Department of Nephrology, Nagoya University Hospital, ( https://ror.org/008zz8m46) Nagoya, Aichi Japan

[3 ]Department of Advanced Medicine, Nagoya University Hospital, ( https://ror.org/008zz8m46) Nagoya, Aichi Japan

Author information

Kazuhiro Furuhashi http://orcid.org/0000-0003-2476-688X

Article

Publisher ID: 2522

DOI: 10.1007/s10157-024-02522-6

PMC ID: 11189982

PubMed ID: 38851645

SO-VID: 0c024545-7674-41c9-8007-81425be853fc

License:

Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/.

History

Date received : 20 February 2024

Date accepted : 28 May 2024

Funding

Funded by: Nagoya University

Open Access :

Open Access funding provided by Nagoya University.

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ScienceOpen disciplines: Nephrology

Keywords: prognosis,microscopic polyangiitis,kidney function,kidney histopathology

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ScienceOpen disciplines: Nephrology

Keywords: prognosis, microscopic polyangiitis, kidney function, kidney histopathology

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Prognosis of microscopic polyangiitis is well predictable in the first 2 weeks of treatment

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Abstract

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Karger: Nephrology

Most cited references 12

Rituximab versus cyclophosphamide for ANCA-associated vasculitis.

ANCA-associated vasculitis

Rituximab versus cyclophosphamide in ANCA-associated renal vasculitis.

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