Am. J. Respir. Crit. Care Med., Vol 157, No. 1, Jan 1998, 199-203.
Prognostic significance of histopathologic subsets in idiopathic pulmonary fibrosis [In Process Citation]
JA Bjoraker, JH Ryu, MK Edwin, JL Myers, HD Tazelaar, DR Schroeder and KP Offord
Division of Pulmonary and Critical Care Medicine, Mayo Graduate School of Medicine, Mayo Clinic and Mayo Foundation, Rochester, Minnesota 55905, USA.
Idiopathic pulmonary fibrosis (IPF) is a generally fatal disorder with a
reported median survival of 3 to 6 yr. This has been based on relatively
few studies with diagnoses inconsistently confirmed by adequate lung
biopsy. Retrospective analysis of 104 patients with IPF who had open lung
biopsy (OLB) at Mayo Medical Center from 1976 to 1985 was performed to
establish the overall survival rate, the spectrum of histopathological
subgroups and their associated prognostic significance. The study group
consisted of 54 men and 50 women with a median age of 63 yr. Median
survival was 3.8 yr after diagnosis by OLB with an estimated 10 yr survival
of 27%. Current histopathologic review showed a heterogeneous group
including usual interstitial pneumonia (UIP), desquamative interstitial
pneumonia (DIP), nonspecific interstitial pneumonia/fibrosis (NSIP), acute
interstitial pneumonia (AIP), bronchiolitis, bronchiolitis obliterans
organizing pneumonia (BOOP), and others. Median survival of the UIP group
was 2.8 yr which is significantly worse (p < 0.001) than for other
subgroups of chronic interstitial pneumonias. IPF includes several
histopathologic subgroups with significantly different survival rates.
Patients with UIP have worse survival than patients with other types of
idiopathic chronic interstitial pneumonias including NSIP. Accurate
histopathologic classification is essential for prognostication in patients
with IPF.
