title: Clinical features and outcome of acute exacerbation of interstitial pneumonia: collagen vascular diseases-related versus idiopathic.
abstract: BACKGROUND
  Relatively little is known about acute exacerbation (AE) of interstitial pneumonia associated with collagen vascular diseases (CVD-IPs).
  
  
  OBJECTIVE
  This study was aimed at clarifying clinical characteristics and outcome in AE of CVD-IPs, compared with those of idiopathic interstitial pneumonias (IIPs).
  
  
  METHODS
  We retrospectively reviewed 112 admission cases with suspected AE of CVD-IPs or IIPs during 2003-2009. IIPs were diagnosed with idiopathic pulmonary fibrosis (IPF) or non-IPF, mostly based on radiologic findings. Of these, 15 AEs of CVD-IPs (6 rheumatoid arthritis, 6 dermatomyositis and 3 systemic sclerosis) and 47 AEs of IIPs (13 IPF and 34 non-IPF) were included.
  
  
  RESULTS
  The clinical characteristics in AE of CVD-IPs were similar to those of IIPs, except for younger age (63.3 ± 6.8 vs. 73.8 ± 9.1 years; p = 0.0001) and higher PaO(2)/FiO(2) at the onset of AE (205 ± 81.2 vs. 145 ± 53.8 mm Hg; p = 0.002) in the former. Dermatomyositis-related interstitial pneumonia (IP) showed a relatively indolent onset and was often associated with worsening control of the underlying disease, whereas AE of other CVD-IPs resembled that of IIPs. 90-day mortality of 33% in AE of CVD-IPs was similar to that of IIPs (44%; p = 0.44) or non-IPF (34%; p = 0.94), but was significantly better than that of IPF (69%; p = 0.04).
  
  
  CONCLUSIONS
  Clinical features and outcome in AE of CVD-IPs were similar, if not identical, to those of IIPs, having a significant impact on the clinical course. AE of advanced IPF with typical radiologic features seems to have higher mortality compared with other forms of IP.
authors.affiliation: Department of Respiratory Medicine, Kobe City Medical Center General Hospital, Kobe, Japan. ryotkw@kcho.jp
authors.forename: Ryo
authors.initials: R
authors.lastname: Tachikawa
authors.forename: Keisuke
authors.initials: K
authors.lastname: Tomii
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Ueda
authors.forename: Kazuma
authors.initials: K
authors.lastname: Nagata
authors.forename: Shigeki
authors.initials: S
authors.lastname: Nanjo
authors.forename: Ayako
authors.initials: A
authors.lastname: Sakurai
authors.forename: Kyoko
authors.initials: K
authors.lastname: Otsuka
authors.forename: Reiko
authors.initials: R
authors.lastname: Kaji
authors.forename: Michio
authors.initials: M
authors.lastname: Hayashi
authors.forename: Nobuyuki
authors.initials: N
authors.lastname: Katakami
authors.forename: Yukihiro
authors.initials: Y
authors.lastname: Imai
mesh_terms: D000208:Acute Disease; D000368:Aged; D001706:Biopsy; D018893:Bronchoalveolar Lavage; D003095:Collagen Diseases / Q000150:complications* / Q000175:diagnosis / Q000401:mortality; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000150:complications* / Q000175:diagnosis / Q000401:mortality; D007564:Japan / Q000453:epidemiology; D008168:Lung / Q000000981:diagnostic imaging / Q000473:pathology; D017563:Lung Diseases, Interstitial / Q000175:diagnosis / Q000209:etiology* / Q000401:mortality; D008297:Male; D008875:Middle Aged; D013902:Radiography, Thoracic; D012008:Recurrence; D012189:Retrospective Studies; D012307:Risk Factors; D015996:Survival Rate / Q000639:trends; D014057:Tomography, X-Ray Computed; D014652:Vascular Diseases / Q000150:complications* / Q000175:diagnosis / Q000401:mortality