The Association of Airway Comorbidities With the Clinical Phenotypes and Outcomes of Patients With Antineutrophil Cytoplasmic Autoantibody-associated Vasculitis

Nobuyuki Ono; Yasushi Inoue; Tomoya Miyamura; Naoyasu Ueda; Shuji Nagano; Hisako Inoue; Kensuke Oryoji; Shun-Ichiro Ota; Takuya Sawabe; Seiji Yoshizawa; Yukiko Takeyama; Yuri Sadanaga; Ayako Takamori; Yasutaka Kimoto; Katsuhisa Miyake; Takahiko Horiuchi; Hitoshi Nakashima; Hiroaki Niiro; Yoshifumi Tada

doi:10.3899/jrheum.190373

The Association of Airway Comorbidities With the Clinical Phenotypes and Outcomes of Patients With Antineutrophil Cytoplasmic Autoantibody-associated Vasculitis

J Rheumatol. 2021 Mar;48(3):417-425. doi: 10.3899/jrheum.190373. Epub 2019 Sep 15.

Authors

Nobuyuki Ono¹, Yasushi Inoue², Tomoya Miyamura³, Naoyasu Ueda⁴, Shuji Nagano⁵, Hisako Inoue⁶, Kensuke Oryoji⁷, Shun-Ichiro Ota⁸, Takuya Sawabe⁹, Seiji Yoshizawa¹⁰, Yukiko Takeyama¹¹, Yuri Sadanaga¹², Ayako Takamori¹³, Yasutaka Kimoto¹⁴, Katsuhisa Miyake¹⁵, Takahiko Horiuchi¹⁴, Hitoshi Nakashima¹⁵, Hiroaki Niiro¹⁶, Yoshifumi Tada¹²

Affiliations

¹ N. Ono, MD, PhD, Y. Sadanaga, MD, Y. Tada, MD, PhD, Department of Rheumatology, Faculty of Medicine, Saga University, Saga; nono@cc.saga-u.ac.jp.
² Y. Inoue, MD, PhD, Department of Rheumatology, Fukuoka Red Cross Hospital.
³ T. Miyamura, MD, PhD, Department of Rheumatology, National Hospital Organization Kyushu Medical Center, Fukuoka.
⁴ N. Ueda, MD, PhD, Department of Internal Medicine, Miyazaki Prefectural Miyazaki Hospital, Miyazaki.
⁵ S. Nagano, MD, Department of Rheumatology, Iizuka Hospital.
⁶ H. Inoue, MD, PhD, Department of Rheumatology, Saiseikai Fukuoka Hospital, Fukuoka.
⁷ K. Oryoji, MD, Department of Rheumatology, Matsuyama Red Cross Hospital, Matsuyama.
⁸ S. Ota, MD, Department of Rheumatology, Shimonoseki City Hospital, Shimonoseki.
⁹ T. Sawabe, MD, PhD, Department of Rheumatology, Hiroshima Red Cross Hospital, Hiroshima.
¹⁰ S. Yoshizawa, MD, PhD, Department of Rheumatology, Hamanomachi Hospital, Fukuoka.
¹¹ Y. Takeyama, MD, Department of Rheumatology, Faculty of Medicine, Saga University, Saga, Department of Rheumatology, Saiseikai Karatsu Hospital, Karatsu.
¹² N. Ono, MD, PhD, Y. Sadanaga, MD, Y. Tada, MD, PhD, Department of Rheumatology, Faculty of Medicine, Saga University, Saga.
¹³ A. Takamori, MD, PhD, Clinical Research Center, Saga University Hospital, Saga.
¹⁴ Y. Kimoto, MD, T. Horiuchi, MD, PhD, Department of Rheumatology, Kyushu University Beppu Hospital, Beppu.
¹⁵ K. Miyake, MD, PhD, H. Nakashima, MD, PhD, Department of Rheumatology, Fukuoka University.
¹⁶ H. Niiro, MD, PhD, Department of Rheumatology, Kyushu University, Fukuoka, Japan.

PMID: 31523048
DOI: 10.3899/jrheum.190373

Abstract

Objective: We investigated the association of airway comorbidities with the clinical phenotypes and outcomes of myeloperoxidase (MPO)-antineutrophil cytoplasmic antibodies (ANCA)-positive ANCA-associated vasculitis (AAV).

Methods: An AAV patient multicenter cohort trial was established in 13 hospitals in western Japan between 2012 and 2018. We examined 143 of the new-onset MPO-ANCA-positive AAV patients. Their clinical characteristics and comorbidities at disease onset were compared based on clinical phenotypes. Multivariate analysis was performed to identify factors predictive of remission and death.

Results: Twenty-seven cases with granulomatosis with polyangiitis (GPA), 10 with eosinophilic GPA (EGPA), 81 with microscopic polyangiitis (MPA), and 25 with unclassified AAV were identified. The average age of MPO-ANCA-positive patients was 71.4 years. Comorbidity (87.4%) and airway comorbidity (70.6%) were frequently observed in these patients. Examination of the clinical phenotypes revealed that the cases of GPA were frequently accompanied by infectious airway comorbidity (upper airway disease, bronchiectasis, pulmonary infections), and most of the cases of MPA and unclassified AAV were accompanied by fibrotic interstitial lung disease (fILD) or emphysema. Among MPO-ANCA-positive patients, infectious airway comorbidity was predictive of both remission (HR 1.58, P = 0.03) and mortality (HR 2.64, P = 0.04), and fILD was predictive of mortality (HR 7.55, P = 0.008). The combination of infectious airway comorbidities and fILD caused the worst survival outcomes in patients.

Conclusion: MPO-ANCA-positive AAV was frequently accompanied by airway comorbidities. In addition to fILD, infectious airway comorbidities were closely associated with those clinical phenotypes and outcomes.

Keywords: ANCA; ANCA-associated vasculitis; MPO-ANCA; comorbidity; vasculitis.

Publication types

Multicenter Study

MeSH terms

Aged
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis* / complications
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis* / epidemiology
Antibodies, Antineutrophil Cytoplasmic
Comorbidity
Granulomatosis with Polyangiitis* / complications
Granulomatosis with Polyangiitis* / epidemiology
Humans
Myeloblastin
Peroxidase
Phenotype

Substances

Antibodies, Antineutrophil Cytoplasmic
Peroxidase
Myeloblastin