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Characteristics of MPO-ANCA-positive granulomatosis with polyangiitis: a retrospective multi-center study in Japan

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Abstract

We studied the clinico-pathological differences among PR3-ANCA-positive granulomatosis with polyangiitis (PR3-GPA), MPO-ANCA-positive GPA (MPO-GPA) and microscopic polyangiitis (MPA). ANCA-associated vasculitis (AAV) was classified using the European Medicines Agency classification. We retrospectively analyzed 38 patients with GPA and 41 with MPA treated in eight hospitals in Japan. Of the patients with GPA, 17 were positive for MPO-ANCA, and 15 for PR3-ANCA. All patients with MPA were MPO-ANCA positive. The mean ages of those with MPO-GPA were 69.6 years old, 10 years older than those with PR3-GPA. The majority (82 %) of patients with MPO-GPA were woman, a significantly greater proportion than for PR3-GPA. We also found that ear, nose and throat (ENT), nervous system involvement were significantly more common in MPO-GPA, but renal function was less impaired than those with MPA. Both PR3-GPA and MPO-GPA relapsed more frequently than MPA, but overall survival was significantly better (P < 0.01 and P < 0.05, respectively). Univariate analysis identified the following factors as predictors of a poor prognosis: MPA (P < 0.01), pulmonary UIP pattern (P < 0.005) Cr ≥ 1.7 mg/dl (P < 0.01) and absence of ENT involvement (P < 0.05), which were characteristics of MPA. In our cohort, MPO-GPA was most likely to affect older women and was associated with otitis media, nervous system involvement, mild renal impairment and more favorable outcome. It is clinically useful to differentiate MPO-GPA from MPA and PR3-GPA in patients with AAV.

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Fig. 1
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Abbreviations

MPA:

Microscopic polyangiitis

GPA:

Granulomatosis with polyangiitis

PR3-ANCA:

Proteinase-3-antineutrophil cytoplasmic antibody

MPO-ANCA:

Myeloperoxidase-antineutrophil cytoplasmic antibody

FFS:

Five factor score

ENT:

Ear, nose and throat

UIP:

Usual interstitial pneumonia

OB:

Occult blood in urine

Cr:

Creatinine

GI:

Gastrointestinal system

CNS:

Central nervous system

HR:

Hazard ratios

HP:

Hypertrophic pachymeningitis

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Acknowledgments

The authors thank all the patients and other medical staffs who participated in this study. We would like to thanks to Dr D Himeji and Dr S Uezono of Miyazaki Prefectural Miyazaki Hospital, Dr M Ayano, Dr Y Kimoto, Dr Y Arinobu and Dr K Akashi of Kyushu University Hospital, Dr K Nagasawa of Sawara Hospital, Dr S Kobayashi of Juntendo University Hospital for their kind collaboration and suggestions in this study.

Conflict of interest

The authors declare that they have no conflict of interests.

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Authors and Affiliations

  1. Department of Rheumatology, Faculty of Medicine, Saga University, 5-1-1 Nabeshima, Saga, 849-8501, Japan

    Nobuyuki Ono & Yoshifumi Tada

  2. Department of Internal Medicine, Miyazaki Prefectural Miyazaki Hospital, Miyazaki, Japan

    Nobuyuki Ono & Akira Ueda

  3. Department of Medicine and Biosystemic Science, Kyushu University Graduate School of Medical Sciences, Fukuoka, Japan

    Hiroaki Niiro & Hiroshi Tsukamoto

  4. Department of Rheumatology, Hiroshima Red Cross Hospital, Hiroshima, Japan

    Takuya Sawabe

  5. Department of Rheumatology, Kitakyushu Medical Center, Kitakyushu, Japan

    Hiroaki Nishizaka

  6. Department of Rheumatology, JR Kyushu Hospital, Kitakyushu, Japan

    Isao Furugo

  7. Department of Rheumatology, Hamanomachi Hospital, Fukuoka, Japan

    Seiji Yoshizawa

  8. Department of Rheumatology, Fukuoka National Hospital, Fukuoka, Japan

    Shigeru Yoshizawa

  9. Department of Public Health, Kyushu University Graduate School of Medical Sciences, Fukuoka, Japan

    Chikako Kiyohara

  10. Department of Rheumatology, Kyushu University Beppu Hospital, Beppu, Japan

    Takahiko Horiuchi

Authors
  1. Nobuyuki Ono
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  2. Hiroaki Niiro
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  3. Akira Ueda
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  4. Takuya Sawabe
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  5. Hiroaki Nishizaka
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  6. Isao Furugo
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  7. Seiji Yoshizawa
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  8. Shigeru Yoshizawa
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  9. Hiroshi Tsukamoto
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  10. Chikako Kiyohara
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  11. Yoshifumi Tada
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  12. Takahiko Horiuchi
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Corresponding author

Correspondence to Nobuyuki Ono.

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Ono, N., Niiro, H., Ueda, A. et al. Characteristics of MPO-ANCA-positive granulomatosis with polyangiitis: a retrospective multi-center study in Japan. Rheumatol Int 35, 555–559 (2015). https://doi.org/10.1007/s00296-014-3106-z

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  • DOI: https://doi.org/10.1007/s00296-014-3106-z

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