Clinical characteristics of patients with vasculitis positive for anti-neutrophil cytoplasmic antibody targeting both proteinase 3 and myeloperoxidase: a retrospective study


Anti-neutrophil cytoplasmic antibody (ANCA) may target proteinase 3 (PR3) or myeloperoxidase (MPO). Although a few patients with vasculitis have both MPO- and PR3-ANCA, the details of their clinical characteristics are not known. The objective of this study was to analyze the characteristics of patients with dual MPO- and PR3-ANCA-positive vasculitis. The medical records of patients with ANCA and vasculitis confirmed by biopsy were reviewed. The age at diagnosis, sex, and data on organ involvement of the kidney, lung, upper airways, skin, nervous system, and gastrointestinal tract were collected. Clinical variables were analyzed according to ANCA specificity. Of 85 patients with ANCA and vasculitis included in this study, 67 (78.8%) had MPO-ANCA, 10 (11.8%) had PR3-ANCA, and 8 (9.4%) had both MPO- and PR3-ANCA. Patients with MPO− PR3 + ANCA-associated vasculitis (AAV) were younger at diagnosis (median, 54.4 years; p < 0.05) than patients with MPO + PR3− AAV (67.0 years) or dual-ANCA AAV (MPO + PR3 + , 68.5 years). The initial glomerular filtration rate in patients with MPO + PR3− AAV (22.0 ml/min) was significantly lower than that in patients with MPO− PR3 + AAV (108.6 ml/min, p < 0.05), but was not different from that in dual-ANCA AAV patients (16.5 ml/min). Upper airway involvement also differed with ANCA type (MPO+ PR3− , 35.8% vs. MPO− PR3 + , 70.0% vs. MPO + PR3+ , 75.0%, p < 0.05). The involvement of other organs did not differ according to ANCA type. Age at diagnosis, kidney involvement, and upper airway involvement were associated with ANCA type. Patients with dual-ANCA-positive vasculitis had considerably more kidney dysfunction than patients with MPO− PR3+ AAV. They also had more upper airway involvement than patients with MPO+ PR3− AAV.

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This research was supported by the Basic Science Research Program through the National Research Foundation funded by the Ministry of Education of Korea (NRF-2018R1D1A1B07043681).

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SMK designed the study, analyzed data and wrote the initial draft of the manuscript. SYC contributed to data analysis and data interpretation of data, and preparation of the manuscript. SYK contributed to data collection and data interpretation, and critical review of the manuscript. JK designed the study, analyzed data, and wrote the initial draft of the manuscript. All the authors approved the final version of the manuscript, and agree 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 Jinhyun Kim.

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Ethical approval

This study was approved by Chungnam National University Hospital Institutional Review Board (2017-1-31/2017-01-023) and Chungbuk National University Hospital Institutional Review Board (2019-4-25/CBNUH 2017-04-001) and informed consent was waived by each ethics committees. This study was conducted in accordance with the Declaration of Helsinki.

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Kim, S.M., Choi, SY., Kim, S.Y. et al. Clinical characteristics of patients with vasculitis positive for anti-neutrophil cytoplasmic antibody targeting both proteinase 3 and myeloperoxidase: a retrospective study. Rheumatol Int 39, 1919–1926 (2019).

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  • Antibodies, antineutrophil cytoplasmic
  • Systemic vasculitis
  • Anti-neutrophil cytoplasmic antibody-associated vasculitis
  • Microscopic polyangiitis
  • Granulomatosis with polyangiitis