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

Nobuyuki Ono, Hiroaki Niiro, Akira Ueda, Takuya Sawabe, Hiroaki Nishizaka, Isao Furugo, Seiji Yoshizawa, Shigeru Yoshizawa, Hiroshi Tsukamoto, Chikako Kiyohara, Yoshifumi Tada, Takahiko Horiuchi

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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.

Original languageEnglish
Pages (from-to)555-559
Number of pages5
JournalRheumatology International
Volume35
Issue number3
DOIs
Publication statusPublished - Jan 1 2015

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Antineutrophil Cytoplasmic Antibodies
Granulomatosis with Polyangiitis
Japan
Microscopic Polyangiitis
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis
Pharynx
Nose
Nervous System
Ear
Kidney
Otitis Media

All Science Journal Classification (ASJC) codes

  • Rheumatology
  • Immunology and Allergy
  • Immunology

Cite this

Characteristics of MPO-ANCA-positive granulomatosis with polyangiitis : a retrospective multi-center study in Japan. / Ono, Nobuyuki; Niiro, Hiroaki; Ueda, Akira; Sawabe, Takuya; Nishizaka, Hiroaki; Furugo, Isao; Yoshizawa, Seiji; Yoshizawa, Shigeru; Tsukamoto, Hiroshi; Kiyohara, Chikako; Tada, Yoshifumi; Horiuchi, Takahiko.

In: Rheumatology International, Vol. 35, No. 3, 01.01.2015, p. 555-559.

Research output: Contribution to journalArticle

Ono, Nobuyuki ; Niiro, Hiroaki ; Ueda, Akira ; Sawabe, Takuya ; Nishizaka, Hiroaki ; Furugo, Isao ; Yoshizawa, Seiji ; Yoshizawa, Shigeru ; Tsukamoto, Hiroshi ; Kiyohara, Chikako ; Tada, Yoshifumi ; Horiuchi, Takahiko. / Characteristics of MPO-ANCA-positive granulomatosis with polyangiitis : a retrospective multi-center study in Japan. In: Rheumatology International. 2015 ; Vol. 35, No. 3. pp. 555-559.
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AU - Ono, Nobuyuki

AU - Niiro, Hiroaki

AU - Ueda, Akira

AU - Sawabe, Takuya

AU - Nishizaka, Hiroaki

AU - Furugo, Isao

AU - Yoshizawa, Seiji

AU - Yoshizawa, Shigeru

AU - Tsukamoto, Hiroshi

AU - Kiyohara, Chikako

AU - Tada, Yoshifumi

AU - Horiuchi, Takahiko

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