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Clinical significance of ANCA positivity in patients with IgA vasculitis: a retrospective monocentric study

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Abstract

We assessed the detection rate of antineutrophil cytoplasmic antibody (ANCA) and investigated the clinical significance of ANCA positivity at diagnosis in patients with IgA vasculitis (Henoch-Schönlein purpura). We retrospectively reviewed their medical records of 86 IgA vasculitis patients. We divided IgA vasculitis patients based on ANCA positivity and compared variables at diagnosis and poor outcomes and medication during follow-up between the two groups. All-cause mortality, relapse, chronic kidney disease (CKD) (stage 3–5) and end-stage renal disease (ESRD) were defined as poor outcomes. We assessed the renal histological features based on the International Study of Kidney Disease in Children (ISKDC) classification and Oxford classification. Comparison of cumulative survivals was analysed by the Kaplan–Meier survival analysis. Five of 86 IgA vasculitis patients (5.8%) had ANCA and all ANCA-positive patients had myeloperoxidase (MPO)-ANCA. IgA vasculitis patients with ANCA exhibited pulmonary and nervous involvement of IgA vasculitis more frequently than those without. There was no significant difference in renal involvement between the two groups. There were no significant differences in renal histological features and poor outcomes related to renal function between IgA vasculitis patients with and without ANCA. In addition, 5 IgA vasculitis patients did not meet the classification criteria for ANCA-associated vasculitis (AAV). Particularly, there were no significant differences in CKD and ESRD-free survival rates between IgA vasculitis patients with and without ANCA. 5.8% of IgA vasculitis patients had MPO-ANCA and poor outcomes of IgA vasculitis were not affected by the presence of ANCA.

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Funding

This research was supported by Basic Science Research Program through the National Research Foundation of Korea (NRF) funded by the Ministry of Education (2017R1D1A1B03029050) and a grant from the Korea Health Technology R&D Project through the Korea Health Industry Development Institute, funded by the Ministry of Health and Welfare, Republic of Korea (HI14C1324).

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Authors and Affiliations

  1. Deparment of Medicine, Yonsei University College of Medicine, Seoul, Republic of Korea

    Jae Yeon Kim, Hyeok Choi, Minyoung Kevin Kim & Soo Bin Lee

  2. Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul, 03722, Republic of Korea

    Yong-Beom Park & Sang-Won Lee

  3. Institute for Immunology and Immunological Diseases, Yonsei University College of Medicine, Seoul, Republic of Korea

    Yong-Beom Park & Sang-Won Lee

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  1. Jae Yeon Kim
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All authors contributed to conception and design, or acquisition of data, or analysis and interpretation of data and participated in drafting the manuscript or revising it critically for important intellectual content. Also, all authors gave final approval of the version to be published and agreed to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.

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Correspondence to Sang-Won Lee.

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This study was approved by the Institutional Review Board (IRB) of Severance Hospital (4-2017-0673).

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The patient’s written informed consent was waived by the approving IRB, as this was a retrospective study.

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Kim, J.Y., Choi, H., Kim, M.K. et al. Clinical significance of ANCA positivity in patients with IgA vasculitis: a retrospective monocentric study. Rheumatol Int 39, 1927–1936 (2019). https://doi.org/10.1007/s00296-019-04397-3

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