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Clinical course and prognosis in survivors of acute exacerbations of idiopathic pulmonary fibrosis
Authors:Ryo Yamazaki  Osamu Nishiyama  Kazuya Yoshikawa  Sho Saeki  Hiroyuki Sano  Takashi Iwanaga  Yuji Tohda
Affiliation:1. Department of Respiratory Medicine and Allergology, Kindai University Faculty of Medicine, Osakasayama, Osaka 589-8511, Japan;2. Department of Respiratory Medicine and Allergology, Kindai University Nara Hospital, Ikoma, Nara 630-0293, Japan;1. Department of Respiratory Medicine, Graduate School of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-0033, Japan;2. Department of Radiology, Graduate School of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan;3. Department of Infectious Diseases, Graduate School of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-0033, Japan;4. Department of Infection Control and Prevention, The University of Tokyo Hospital, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-0033, Japan;5. Department of Diabetes and Metabolic Diseases, Graduate School of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-0033, Japan;6. Department of Orthopaedic Surgery, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan;1. Kanagawa Cardiovascular and Respiratory Center, Department of Respiratory Medicine, Tomioka-Higashi 6-16-1, Kanazawa-ku, Yokohama, 236-0051, Japan;2. University of Yamanashi Hospital, Department of Internal Medicine 2, Shimokato 1110, Chuo-shi, Yamanashi, 409-3898, Japan;1. Department of Respiratory Medicine, Toho University School of Medicine, 6-11-1 Omori-Nishi, Ota-ku, Tokyo, 143-8541, Japan;2. Department of Physiology, School of Medicine, Faculty of Medicine, Toho University, 5-21-16 Omori-Nishi, Ota-ku, Tokyo, 143-8540, Japan;3. Division of Chest Surgery, Toho University School of Medicine, Omori-Nishi, Ota-ku, Tokyo, 6-11-1, Japan;4. Department of Advanced and Integrated Interstitial Lung Diseases Research, School of Medicine, Toho University, 5-21-16 Omori-Nishi, Ota-ku, Tokyo, 143-8540, Japan;1. Department of Pharmaceutics, Faculty of Pharmaceutical Science, Kobe Gakuin University, 1?1?3 Minatojima Chuo?ku Kobe City 650?8586, Japan;2. Department of Pharmacy, Kobe City Medical Center General Hospital, 2?1?1 Minatojima Minamimachi Chuo?ku Kobe City 650?0047, Japan;3. Department of Regulatory Science, Faculty of Pharmacy, Musashino University, 1?1?20 Shinmachi Nishitokyo City 202?8585, Japan;4. Department of Medical Oncology, Kobe City Medical Center General Hospital, 2?1?1 Minatojima Minamimachi Chuo?ku Kobe City 650?0047, Japan;5. Department of Medical Oncology, Takarazuka City Hospital, 4?5?1 Kohama Takarazuka City 655?0827, Japan
Abstract:
BackgroundPatients with idiopathic pulmonary fibrosis (IPF) are at risk of acute exacerbations (AEs) that manifest as respiratory distress. However, the clinical course after AEs of IPF (AE-IPFs) has not been well described. Therefore, we aimed to elucidate the clinical course and prognosis in survivors of AE-IPFs.MethodsConsecutive patients with IPF who presented to our institution with their first AE-IPFs between January 2008 and December 2019 were included in this study. Data were retrospectively collected, and the clinical course, survival, and cause of death were further analyzed.ResultsNinety-seven patients were included in this retrospective study. Among them, 67 (69.1%) were discharged alive, with a median survival time after discharge of 1081 days. AE recurrence and pneumonia were the most common causes of death, each accounting for 22.2% of cases among survivors of AE-IPFs. AEs were the most frequent during the first 3 years after discharge, whereas pneumonia was more common thereafter.ConclusionsSurvivors of AE-IPFs have a relatively favorable long-term prognosis. Among the survivors of first AE-IPFs, AE recurrence and pneumonia were the most common causes of death after discharge. Therefore, preventing AE recurrence and lung infections is crucial for prolonging survival in survivors of AE-IPFs.
Keywords:Acute exacerbation  Idiopathic pulmonary fibrosis  Pneumonia  Survivors  AE"  },{"  #name"  :"  keyword"  ,"  $"  :{"  id"  :"  kwrd0035"  },"  $$"  :[{"  #name"  :"  text"  ,"  _"  :"  acute exacerbation  AE-IPF"  },{"  #name"  :"  keyword"  ,"  $"  :{"  id"  :"  kwrd0045"  },"  $$"  :[{"  #name"  :"  text"  ,"  _"  :"  acute exacerbation of idiopathic pulmonary fibrosis  CI"  },{"  #name"  :"  keyword"  ,"  $"  :{"  id"  :"  kwrd0055"  },"  $$"  :[{"  #name"  :"  text"  ,"  _"  :"  confidence interval  CRP"  },{"  #name"  :"  keyword"  ,"  $"  :{"  id"  :"  kwrd0065"  },"  $$"  :[{"  #name"  :"  text"  ,"  _"  :"  C-reactive protein  DLco"  },{"  #name"  :"  keyword"  ,"  $"  :{"  id"  :"  kwrd0075"  },"  $$"  :[{"  #name"  :"  text"  ,"  _"  :"  diffusing capacity of the lungs for carbon monoxide  forced expiratory volume in 1 s  fraction of inspiratory oxygen  FVC"  },{"  #name"  :"  keyword"  ,"  $"  :{"  id"  :"  kwrd0105"  },"  $$"  :[{"  #name"  :"  text"  ,"  _"  :"  forced vital capacity  HRCT"  },{"  #name"  :"  keyword"  ,"  $"  :{"  id"  :"  kwrd0115"  },"  $$"  :[{"  #name"  :"  text"  ,"  _"  :"  high-resolution computed tomography  IPF"  },{"  #name"  :"  keyword"  ,"  $"  :{"  id"  :"  kwrd0125"  },"  $$"  :[{"  #name"  :"  text"  ,"  _"  :"  idiopathic pulmonary fibrosis  KL-6"  },{"  #name"  :"  keyword"  ,"  $"  :{"  id"  :"  kwrd0135"  },"  $$"  :[{"  #name"  :"  text"  ,"  _"  :"  Krebs von der Lungen-6  LDH"  },{"  #name"  :"  keyword"  ,"  $"  :{"  id"  :"  kwrd0145"  },"  $$"  :[{"  #name"  :"  text"  ,"  _"  :"  lactate dehydrogenase  partial pressure of arterial carbon dioxide  partial pressure of arterial oxygen/fraction of inspiratory oxygen  PFT"  },{"  #name"  :"  keyword"  ,"  $"  :{"  id"  :"  kwrd0175"  },"  $$"  :[{"  #name"  :"  text"  ,"  _"  :"  pulmonary function test  PMX"  },{"  #name"  :"  keyword"  ,"  $"  :{"  id"  :"  kwrd0185"  },"  $$"  :[{"  #name"  :"  text"  ,"  _"  :"  polymyxin B-immobilized fiber column  rhTM"  },{"  #name"  :"  keyword"  ,"  $"  :{"  id"  :"  kwrd0195"  },"  $$"  :[{"  #name"  :"  text"  ,"  _"  :"  recombinant human soluble thrombomodulin  SD"  },{"  #name"  :"  keyword"  ,"  $"  :{"  id"  :"  kwrd0205"  },"  $$"  :[{"  #name"  :"  text"  ,"  _"  :"  standard deviation  SLB"  },{"  #name"  :"  keyword"  ,"  $"  :{"  id"  :"  kwrd0215"  },"  $$"  :[{"  #name"  :"  text"  ,"  _"  :"  surgical lung biopsy  UIP"  },{"  #name"  :"  keyword"  ,"  $"  :{"  id"  :"  kwrd0225"  },"  $$"  :[{"  #name"  :"  text"  ,"  _"  :"  usual interstitial pneumonia  WBC"  },{"  #name"  :"  keyword"  ,"  $"  :{"  id"  :"  kwrd0235"  },"  $$"  :[{"  #name"  :"  text"  ,"  _"  :"  white blood cell
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