Survival of patients with biopsy-proven usual interstitial pneumonia
and nonspecific interstitial pneumonia
R.L. Riha*, E.E. Duhig#, B.E. Clarke#, R.H. Steele}, R.E. Slaughter*, P.V. Zimmerman*
Survival of patients with biopsy-proven usual interstitial pneumonia and nonspecific
interstitial pneumonia. R.L. Riha, E.E. Duhig, B.E. Clarke, R.H. Steele, R.E. Slaughter,
P.V. Zimmerman. #ERS Journals Ltd 2002.
ABSTRACT: This is the first Australian study to examine survival and clinical
characteristics in biopsy-proven idiopathic interstitial pneumonia.
A cohort of 70 patients from a single institution between January 1990 and December
1999 was reviewed.
All patients were Caucasian, 23 (33%) female. Mean age¡SD at diagnosis was
60¡12 yrs for males and 54¡14 yrs for females. A total 24% of patients had never
smoked. The histopathological diagnoses were usual interstitial pneumonia (UIP)
(n=59), nonspecific interstitial pneumonia (NSIP) (n=7), desquamative interstitial
pneumonia (n=3) and acute interstitial pneumonia (n=11). Clinical and functional
characteristics of the two main histological subgroups of UIP and NSIP showed
significantly older patients in the UIP group and a significantly lower mean forced
expiratory volume in one second (FEV1) in the NSIP group. Median survival for UIP
was 78 months compared with 178 months for NSIP. No survival difference between
treated and untreated patients with UIP was found. Multivariate analysis revealed
smoking alone to be predictive of poorer survival.
This study demonstrates the best median survival for usual interstitial pneumonia of
available series and confirms a survival difference between usual interstitial pneumonia
and nonspecific interstitial pneumonia. Furthermore, the reported results may have
implications for treatment timing using conventional protocols currently recommended.
Eur Respir J 2002; 19: 1114–1118.
*Thoracic and Radiology Dept and
#Pathology Dept, The Prince Charles
Princess Alexandra Hospital, Brisbane,
}Pathology Dept, The
Correspondence: R.L. Riha
Dept of Medicine
The University of Edinburgh
Fax: 44 1315362362
Keywords: Idiopathic interstitial pneu-
monia, idiopathic pulmonary fibrosis,
survival, usual interstitial pneumonia
Received: May 10 2001
Accepted after revision December 10
Idiopathic interstitial pneumonia is a term that
encompasses the following entities: usual interstitial
pneumonia (UIP), desquamative interstitial pneumo-
nia (DIP), acute interstitial pneumonia (AIP), and
nonspecific interstitial pneumonia (NSIP) . Several
recent studies have demonstrated variable treatment
responses and survival based on this classification
[2–8]. Formerly, lack of distinction between the
various classes had led to them being considered
together as idiopathic pulmonary fibrosis (IPF). The
American Thoracic Society, in its International
Consensus Statement  has now revised the defini-
tion of IPF as characterized solely by the histopatho-
logical pattern of UIP. This definition has now been
accepted on an international level .
The aims of this study were to examine survival
and clinical characteristics in patients identified as
having idiopathic interstitial pneumonia in the context
of their histological diagnosis.
A total 570, consecutive hospital medical records
at a single institution were examined for patients
diagnosed with pulmonary fibrosis between January
1990 and December 1999 (ICD9 codes 515 and 516.3).
Patients with connective-tissue disease, occupational-
dust exposure, other dust exposure, potential fibro-
genic or antifibrogenic drug exposure or a syndrome
associated with pulmonary fibrosis (e.g. neurofibro-
matosis) were excluded. All patients were human
immunodeficiency virus (HIV) negative. A total 112
patients had clinical, physiological and radiographical
criteria for IPF, in addition to having histological
material available (transbronchial lung biopsy, open
lung biopsy or autopsy).
Three pulmonary pathologists reviewed all histo-
pathological material by consensus, unaware of
clinical findings or previous histological diagnoses.
Classification was undertaken using standard criteria
for identifying UIP, NSIP, DIP and AIP [2, 4, 10].
UIP is characterized by temporal and spatial hetero-
geneity with patchy lung fibrosis, areas of honeycomb
change, fibroblastic foci and intervening normal lung
parenchyma. NSIP, on the other hand, is a uniform
appearing, cellular interstitial pneumonia typified
by an interstitial lymphoplasmocytic infiltrate and
accompanying amounts of interstitial fibrosis .
No distinction was made in this study between the
fibrosing and cellular patterns of NSIP according to
the criteria described by KATZENSTEM and FIORELLI
. In DIP the predominant abnormalities are within
the alveolar spaces. There is a diffuse increase in
Eur Respir J 2002; 19: 1114–1118
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Copyright#ERS Journals Ltd 2002
European Respiratory Journal