Prognostic Factors in the Acute Exacerbation of Idiopathic Pulmonary Fibrosis: A Retrospective Single-center Study

  • Atsumi Kenichiro
    Department of Pulmonary Medicine and Oncology, Graduate School of Medicine, Nippon Medical School, Japan
  • Saito Yoshinobu
    Department of Pulmonary Medicine and Oncology, Graduate School of Medicine, Nippon Medical School, Japan
  • Kuse Naoyuki
    Department of Pulmonary Medicine and Oncology, Graduate School of Medicine, Nippon Medical School, Japan
  • Kobayashi Kenichi
    Department of Pulmonary Medicine and Oncology, Graduate School of Medicine, Nippon Medical School, Japan
  • Tanaka Toru
    Department of Pulmonary Medicine and Oncology, Graduate School of Medicine, Nippon Medical School, Japan
  • Kashiwada Takeru
    Department of Pulmonary Medicine and Oncology, Graduate School of Medicine, Nippon Medical School, Japan
  • Inomata Minoru
    Department of Pulmonary Medicine and Oncology, Graduate School of Medicine, Nippon Medical School, Japan
  • Kokuho Nariaki
    Department of Pulmonary Medicine and Oncology, Graduate School of Medicine, Nippon Medical School, Japan
  • Hayashi Hiroki
    Department of Pulmonary Medicine and Oncology, Graduate School of Medicine, Nippon Medical School, Japan
  • Kamio Koichiro
    Division of Respiratory Medicine, Musashi Kosugi Hospital, Nippon Medical School, Japan
  • Fujita Kazue
    Department of Pulmonary Medicine and Oncology, Graduate School of Medicine, Nippon Medical School, Japan
  • Abe Shinji
    Department of Pulmonary Medicine and Oncology, Graduate School of Medicine, Nippon Medical School, Japan
  • Azuma Arata
    Division of Respiratory Medicine, Musashi Kosugi Hospital, Nippon Medical School, Japan
  • Kubota Kaoru
    Department of Pulmonary Medicine and Oncology, Graduate School of Medicine, Nippon Medical School, Japan
  • Gemma Akihiko
    Department of Pulmonary Medicine and Oncology, Graduate School of Medicine, Nippon Medical School, Japan

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Abstract

<p>Objectives Acute exacerbation of idiopathic pulmonary fibrosis (IPF-AE) has been recognized as a fatal pulmonary disorder, but the exact prognostic factors are unknown. The aim of the present study was to analyze the clinical characteristics of patients with IPF-AE and identify the prognostic factors. </p><p>Methods The medical records of 59 cases of IPF-AE were retrospectively reviewed. Clinical data, laboratory data, radiographic findings, treatment, and time from the onset of symptoms to the initiation of corticosteroid pulse therapy, i.e. symptom duration, and outcome were analyzed. </p><p>Results The IPF Stage, Gender-Age-Physiology (GAP) Index, symptom duration, and the high-resolution computed tomography (HRCT) score were significantly related to the prognosis in the univariate analysis. In the multivariate analysis, the symptom duration remained a significant prognostic factor (hazard ratio of 1-day increase, 1.11; 95% confidence interval, 1.01-1.15; p=0.0427). The area under the receiver operating characteristics curve of symptom duration was statistically significant for survivors versus non-survivors (area under the curve, 0.73; p=0.012). The survival period was significantly shorter in the late-treatment groups (≥5 days; n=30) than in the early-treatment groups (<5 days; n=29; log-rank test; p<0.0001). </p><p>Conclusion The time interval between the onset of symptoms and the initiation of corticosteroid pulse therapy may be an independent prognostic factor in patients with IPF-AE. </p>

Journal

  • Internal Medicine

    Internal Medicine 57 (5), 655-661, 2018

    The Japanese Society of Internal Medicine

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