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Interstitial lung disease with myeloperoxidase-antineutrophil cytoplasmic antibody-associated vasculitis in elderly patients

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Abstract

Anti-neutrophil cytoplasmic antibodies-associated vasculitis (AAV) occurs in elderly people, and patients with anti-myeloperoxidase autoantibodies (MPO-ANCA)-positive AAV are often complicated with interstitial lung disease (ILD). This study aimed to evaluate the age-related clinical features of elderly patients with MPO-ANCA-positive AAV-ILD. This study retrospectively investigated 63 patients with MPO-ANCA-positive AAV-ILD, all of whom were 65 years or older at diagnosis. Clinical characteristics, causes of death and survival rates among three groups stratified by age (65–74 years, n = 29; 75–79 years, n = 18; over 80 years, n = 16) were compared. This study also examined the association with severe infections in these patients. Among the three age groups, there were significant differences in sex (P = 0.032), serum Krebs von den Lungen-6 (P < 0.01), and total ground-glass opacity score (P = 0.011). The causes of death were mainly severe infections and complications of ILD. Kaplan–Meier curve analysis showed a significantly lower 5-year survival rate in the oldest group (P < 0.01). Regarding severe infections in these patients, the 5-year cumulative incidence of severe infections was higher in the patients receiving steroid pulse therapy (P = 0.034). The clinical characteristics of MPO-ANCA-positive AAV-ILD differ with age in elderly patients, with age being an important poor prognostic factor in these patients. The administration of steroid pulse therapy is a significant risk factor of severe infection in MPO-ANCA-positive elderly patients with AAV-ILD.

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Acknowledgements

We thank Angela Morben, DVM, ELS, J. Ludovic Croxford, PhD, and Gillian Campbell, PhD, from Edanz Group (https://en-author-services.edanz.com/) for editing a draft of this manuscript.

Funding

This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.

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KY designed the study, collected and analyzed data, and wrote the manuscript. AY, MI, YO, YS, SU, CH, KT, KU, MA, SK, MT, KH, SM, MT, FO, YT, MM and MN contributed to collect the date. TS, HI, YK, KH and TM reviewed the original manuscript. All the authors read and approved the final manuscript.

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Correspondence to Koichi Yamaguchi.

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The authors declare no conflicts of interest.

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This study was approved by the Gunma University Ethical Review Board for Medical Research Involving Human Subjects (Approval Number HS2020-064) and that it conformed to the provisions of the Declaration of Helsinki.

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Yamaguchi, K., Yamaguchi, A., Itai, M. et al. Interstitial lung disease with myeloperoxidase-antineutrophil cytoplasmic antibody-associated vasculitis in elderly patients. Rheumatol Int 41, 1641–1650 (2021). https://doi.org/10.1007/s00296-021-04891-7

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