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Clinical differences among patients with myeloperoxidase–antineutrophil cytoplasmic antibody–positive interstitial lung disease

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Abstract

Introduction

Patients with antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis and idiopathic interstitial lung diseases (IIPs) are positive for myeloperoxidase (MPO)–ANCA. MPO–ANCA-positive vasculitis mainly comprises microscopic polyangiitis (MPA) and unclassifiable vasculitis. These diseases are frequently complicated by interstitial lung disease (ILD). Few studies have reported the clinical differences between the subtypes of MPO–ANCA-positive ILD. Therefore, this study aimed to examine the clinical findings and courses of MPO–ANCA-positive ILD.

Method

This retrospective study enrolled 100 patients with MPO–ANCA-positive ILD who were categorized into three groups: MPA (n = 44), unclassifiable vasculitis (n = 29), and IIP (n = 27). Our study compared the clinical findings and prognosis of these patients and analyzed the poor prognostic factors. Furthermore, we assessed the association between the patients with and without acute exacerbation of ILD (AE-ILD).

Results

Our study found clinical differences in serum markers, clinical symptoms, and treatment regimens among the three groups. ILD complications, as the main cause of death, differed among the three groups (P = 0.04). Patients with unclassifiable vasculitis showed higher survival rates than those with IIP (P = 0.046). Patients with AE-ILD showed fewer general symptoms (P = 0.02) and lower survival rates (P < 0.01) than those without AE-ILD. In multivariate analysis, AE-ILD development was a strong poor prognostic factor for MPO–ANCA-positive ILD.

Conclusions

The subtypes of MPO–ANCA-positive ILD have different clinical features and prognoses. Patients who develop AE-ILD require careful evaluation of clinical courses.

Key Points

• In myeloperoxidase (MPO)–antineutrophil cytoplasmic antibody (ANCA)–positive interstitial lung disease (ILD), patients with unclassifiable vasculitis showed a better prognosis than those with idiopathic ILD..

• Development of acute exacerbation in ILD was a strong poor prognostic factor in patients with MPO–ANCA-positive ILD..

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Data availability

All data of this study are included in this published article.

Code availability

Not applicable.

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Authors and Affiliations

  1. Department of Allergy and Respiratory Medicine, Gunma University Graduate School of Medicine, 3-39-15 Showa-machi, Gunma, 371-8511, Japan

    Koichi Yamaguchi, Aya Yamaguchi, Masashi Ito, Ikuo Wakamatsu, Sohei Muto, Masaki Aikawa, Shunichi Kouno, Masakiyo Yatomi, Haruka Aoki-Saito, Yasuhiko Koga, Kenichiro Hara & Toshitaka Maeno

  2. Department of Respiratory Medicine, Fujioka General Hospital, Fujioka, Japan

    Miki Itai, Shogo Uno, Masao Takemura & Mitsuru Motegi

  3. Division of Rheumatology and Nephrology, Fujioka General Hospital, Fujioka, Japan

    Shinsuke Motegi, Mayuko Tsukida, Fumie Ota & Yoshito Tsukada

  4. Department of Nephrology and Rheumatology, Gunma University Graduate School of Medicine, Maebashi, Gunma, Japan

    Masao Nakasatomi, Toru Sakairi, Hidekazu Ikeuchi, Yoriaki Kaneko & Keiju Hiromura

Authors
  1. Koichi Yamaguchi
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  2. Aya Yamaguchi
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Contributions

Koichi Yamaguchi: conceptualization, methodology, formal analysis, investigation, writing—original draft. Aya Yamaguchi: investigation. Masashi Ito: investigation. Ikuo Wakamatsu: investigation. Miki Itai: investigation. Sohei Muto: investigation. Shogo Uno: investigation. Masaki Aikawa: investigation. Shunichi Kouno: investigation. Masao Takemura: investigation. Masakiyo Yatomi: investigation, writing—review and editing. Haruka Aoki-Saito: writing—review and editing. Yasuhiko Koga: investigation, writing—review and editing. Kenichiro Hara: investigation, writing—review and editing. Shinsuke Motegi: investigation. Mayuko Tsukida: investigation. Fumie Ota: investigation. Yoshito Tsukada: investigation, writing—review and editing. Mitsuru Motegi: investigation, writing—review and editing. Masao Nakasatomi: investigation. Toru Sakairi: investigation, writing—review and editing. Hidekazu Ikeuchi: investigation, writing—review and editing. Yoriaki Kaneko: writing—review and editing. Keiju Hiromura: writing—review and editing. Toshitaka Maeno: writing—review and editing.

Corresponding author

Correspondence to Koichi Yamaguchi.

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Ethics approval

This study was recognized by the Ethical Review Committee for Medical Research Involving Human Subjects of Gunma University. This study was performed according to the principles of the Declaration of Helsinki.

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Yamaguchi, K., Yamaguchi, A., Ito, M. et al. Clinical differences among patients with myeloperoxidase–antineutrophil cytoplasmic antibody–positive interstitial lung disease. Clin Rheumatol 42, 479–488 (2023). https://doi.org/10.1007/s10067-022-06388-5

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