Acute exacerbation of interstitial pneumonia other than idiopathic pulmonary fibrosis.
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.
Park
I-Nae
IN
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.
Kim
Dong Soon
DS
Shim
Tae Sun
TS
Lim
Chae-Man
CM
Lee
Sang Do
SD
Koh
Younsuck
Y
Kim
Woo Sung
WS
Kim
Won Dong
WD
Jang
Se Jin
SJ
Colby
Thomas V
TV
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*
D000305:Adrenal Cortex Hormones; D000900:Anti-Bacterial Agents