title: Acute exacerbation of interstitial pneumonia other than idiopathic pulmonary fibrosis.
abstract: BACKGROUND
  Acute exacerbation (AE) in idiopathic pulmonary fibrosis (IPF) is increasingly recognized as a relatively common and highly morbid clinical event. However, clinical data on AE in non-IPF interstitial pneumonia are sparse. This study was performed to find the frequency, clinical features, and outcome of AE in non-IPF interstitial pneumonia.
  
  
  METHODS
  Retrospective analysis of 10 patients who satisfied the modified Akira criteria for AE during follow-up of 74 patients with surgical lung biopsy-confirmed idiopathic nonspecific interstitial pneumonia (I-NSIP) and 93 patients with biopsy-confirmed interstitial pneumonia associated with collagen vascular disease (CVD-IP).
  
  
  RESULTS
  AE occurred in six patients with I-NSIP (1-year frequency, 4.2%) and in four patients with CVD-IP (rheumatoid arthritis [RA], n = 3; scleroderma, n = 1), with 1-year frequency of 3.3%. Median age was 58 years (range, 47 to 75); six patients were female. AE occurred in two patients immediately after surgical biopsy. Median duration of acute symptom before hospital admission was 10 days (range, 1 to 30). Median ratio of Pao(2) to the fraction of inspired oxygen (Fio(2)) was 172 (range, 107 to 273), and Pao(2)/Fio(2) ratio was < 200 in six patients. Surgical lung biopsy performed at the time of AE in two patients revealed diffuse alveolar damage superimposed on nonspecific interstitial pneumonia pattern. Four patients with I-NSIP survived to discharge and were followed up for 24 months (range, 6 to 121).
  
  
  CONCLUSIONS
  AE occurred in the patients with I-NSIP with apparently better prognosis. In patients with CVD-IP, AE occurred mostly with RA-usual interstitial pneumonia in our small series with poor outcome.
authors.affiliation: Division of Pulmonary and Critical Care Medicine, University of Ulsan College of Medicine, Asan Medical Center, 388-1 Pungnap-2dong, Songpa-gu, Seoul 138-736, Korea.
authors.forename: I-Nae
authors.initials: IN
authors.lastname: Park
authors.forename: Dong Soon
authors.initials: DS
authors.lastname: Kim
authors.forename: Tae Sun
authors.initials: TS
authors.lastname: Shim
authors.forename: Chae-Man
authors.initials: CM
authors.lastname: Lim
authors.forename: Sang Do
authors.initials: SD
authors.lastname: Lee
authors.forename: Younsuck
authors.initials: Y
authors.lastname: Koh
authors.forename: Woo Sung
authors.initials: WS
authors.lastname: Kim
authors.forename: Won Dong
authors.initials: WD
authors.lastname: Kim
authors.forename: Se Jin
authors.initials: SJ
authors.lastname: Jang
authors.forename: Thomas V
authors.initials: TV
authors.lastname: Colby
mesh_terms: D000208:Acute Disease; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000368:Aged; D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D001172:Arthritis, Rheumatoid / Q000175:diagnosis / Q000209:etiology*; D001706:Biopsy; D015331:Cohort Studies; D005260:Female; D006801:Humans; D008168:Lung / Q000473:pathology / Q000503:physiopathology; D017563:Lung Diseases, Interstitial / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D011658:Pulmonary Fibrosis / Q000175:diagnosis / Q000209:etiology*; D012121:Respiration, Artificial; D012129:Respiratory Function Tests; D012189:Retrospective Studies; D016896:Treatment Outcome; D014652:Vascular Diseases / Q000175:diagnosis / Q000209:etiology*
chemical_list: D000305:Adrenal Cortex Hormones; D000900:Anti-Bacterial Agents