RT Journal Article
SR Electronic
T1 The association of airway comorbidities with the clinical phenotypes and outcomes of ANCA-associated vasculitis patients.
JF The Journal of Rheumatology
JO J Rheumatol
FD The Journal of Rheumatology
SP jrheum.190373
DO 10.3899/jrheum.190373
A1 Nobuyuki Ono
A1 Yasushi Inoue
A1 Tomoya Miyamura
A1 Naoyasu Ueda
A1 Shuji Nagano
A1 Hisako Inoue
A1 Kensuke Oryoji
A1 Shun-ichiro Ota
A1 Takuya Sawabe
A1 Seiji Yoshizawa
A1 Yukiko Takeyama
A1 Yuri Sadanaga
A1 Ayako Takamori
A1 Yasutaka Kimoto
A1 Katsuhisa Miyake
A1 Takahiko Horiuchi
A1 Hitoshi Nakashima
A1 Hiroaki Niiro
A1 Yoshifumi Tada
YR 2019
UL http://www.jrheum.org/content/early/2019/09/11/jrheum.190373.abstract
AB Objective We investigated the association of airway comorbidities with the clinical phenotypes and outcomes of myeloperoxidase (MPO)-anti-neutrophil cytoplasmic antibodies (ANCA)-positive ANCA-associated vasculitis (AAV). Methods An AAV patient multi-center 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 for remission and death. Results Ten cases with eosinophilic granulomatosis with polyangitis (EGPA), 27 with granulomatosis with polyangitis (GPA), 81 with microscopic polyangitis (MPA) and 25 with unclassified AAV were identified. The average age was 71.4 years old. 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=0.027) and mortality (HR 2.64, p=0.040), and fILD was predictive of mortality (HR 7.55, p=0.0078). The combination of infectious airway comorbidities and fILD caused the worst survival outcomes in those 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.