Atsushi Suzuki1,2, Yasuhiro Kondoh1, Kevin K Brown3, Takeshi Johkoh4, Kensuke Kataoka1, Junya Fukuoka5, Tomoki Kimura1, Toshiaki Matsuda1, Toshiki Yokoyama1, Jun Fukihara2, Masahiko Ando6, Tomonori Tanaka7, Naozumi Hashimoto2, Koji Sakamoto2, Yoshinori Hasegawa2,8. 1. Department of Respiratory Medicine and Allergy, Tosei General Hospital, Seto, Japan. 2. Department of Respiratory Medicine, Nagoya University Graduate School of Medicine, Nagoya, Japan. 3. Department of Medicine, National Jewish Health, Denver, CO, USA. 4. Department of Radiology, Kansai Rosai Hospital, Amagasaki, Japan. 5. Department of Pathology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan. 6. Center for Advanced Medicine and Clinical Research, Nagoya University Hospital, Nagoya, Japan. 7. Department of Pathology, Kindai University Faculty of Medicine, Osakasayama, Japan. 8. National Hospitalization Organization Nagoya Medical Center, Nagoya, Japan.
Abstract
BACKGROUND AND OBJECTIVE: Acute exacerbation (AE) is a severe complication of idiopathic pulmonary fibrosis (AE-IPF). In 2016, an international working group revised its definition and diagnostic criteria; however, few studies have assessed the frequency and prognosis of AE in patients with other fibrotic interstitial lung diseases (FILD). METHODS: We used data from 1019 consecutive interstitial lung disease (ILD) patients initially evaluated between January 2008 and July 2015. All subject diagnoses were made by multidisciplinary discussion in December 2018. ILD was categorized as IPF (n = 462) and other FILD which included non-specific interstitial pneumonia (n = 22), chronic hypersensitivity pneumonitis (n = 29), connective tissue disease-associated ILD (n = 205) and unclassifiable ILD (n = 209). Using the 2016 definition of AE-IPF, we identified all subjects with an AE. RESULTS: During the observational period, 193 patients experienced a first AE (AE-FILD n = 69, AE-IPF n = 124). The time to first AE was significantly longer in FILD than IPF (log-rank test, P < 0.001). After adjusting for potentially influential confounders, FILD remained a significant predictor of longer time to first AE compared with IPF (hazard ratio: 0.453; 95% CI: 0.317-0.647, P = 0.006). In a multivariate Cox proportional analysis, baseline disease severity was closely associated with the incidence of AE-ILD. Even after adjustment for other clinical variables, AE had a negative impact on overall survival. AE-FILD and AE-IPF showed similar poor short-term outcomes. CONCLUSION: All forms of ILD are at risk of AE and have a similar outcome to AE-IPF.
BACKGROUND AND OBJECTIVE: Acute exacerbation (AE) is a severe complication of idiopathic pulmonary fibrosis (AE-IPF). In 2016, an international working group revised its definition and diagnostic criteria; however, few studies have assessed the frequency and prognosis of AE in patients with other fibrotic interstitial lung diseases (FILD). METHODS: We used data from 1019 consecutive interstitial lung disease (ILD) patients initially evaluated between January 2008 and July 2015. All subject diagnoses were made by multidisciplinary discussion in December 2018. ILD was categorized as IPF (n = 462) and other FILD which included non-specific interstitial pneumonia (n = 22), chronic hypersensitivitypneumonitis (n = 29), connective tissue disease-associated ILD (n = 205) and unclassifiable ILD (n = 209). Using the 2016 definition of AE-IPF, we identified all subjects with an AE. RESULTS: During the observational period, 193 patients experienced a first AE (AE-FILD n = 69, AE-IPF n = 124). The time to first AE was significantly longer in FILD than IPF (log-rank test, P < 0.001). After adjusting for potentially influential confounders, FILD remained a significant predictor of longer time to first AE compared with IPF (hazard ratio: 0.453; 95% CI: 0.317-0.647, P = 0.006). In a multivariate Cox proportional analysis, baseline disease severity was closely associated with the incidence of AE-ILD. Even after adjustment for other clinical variables, AE had a negative impact on overall survival. AE-FILD and AE-IPF showed similar poor short-term outcomes. CONCLUSION: All forms of ILD are at risk of AE and have a similar outcome to AE-IPF.
Authors: Maria Molina-Molina; Michael Kreuter; Vincent Cottin; Tamera J Corte; Frank Gilberg; Klaus-Uwe Kirchgaessler; Judit Axmann; Toby M Maher Journal: Front Med (Lausanne) Date: 2022-06-17