Features of Idiopathic Pulmonary Fibrosis with Organizing Pneumonia

Abstract

To characterize the clinical features of patients with idiopathic pulmonary fibrosis (IPF) having organizing pneumonia (OP), we retrospectively reviewed the clinical charts, chest X-rays, CT scans, and transbronchial lung biopsy (TBLB) specimens of patients with IPF. Patients with IPF and OP had a subacute onset of symptoms (within 2 months) (87.5%), leukocytosis ( > 10,000/ mm³) (62.5%), and a strong C-reactive protein (CRP) reaction ( > 3+) (75%). Some of these features were distinctly different from those of IPF patients without OP (subacute onset of symptoms 0%, leukocytosis 0%, strong CRP reaction 16.7%). In the patients with IPF with OP, A-aDO₂ and semiquantitative scores of chest X-ray abnormalities improved significantly after prednisolone treatment. Those abnormalities improved only slightly in the patients with IPF without OP. Diffusing capacity remained decreased and abnormal interstitial infiltration persisted, even after prednisolone treatment in the patients with IPF with or without OP. Clinical features of IPF patients with OP differed from those of patients with IPF without OP. IPF patients with OP showed good clinical response to corticosteroid therapy. These findings warrant further study of the presence of OP in TBLB specimens in predicting corticosteroid responsiveness and prognosis of patients with IPF.

Idiopathic nonspecific interstitial pneumonia/fibrosis: comparison with idiopathic pulmonary fibrosis and BOOP

“…There is one confusing report in which "IPF with organizing pneumonia" was characterized based on an approach by transbronchial lung biopsy [31]. In this report, although the mode of onset and clinical findings were similar to those of NSIP, the histology was different.…”

Management of idiopathic pulmonary fibrosis: selected case reports

“…However, the clinical course of this patient does not support the hypothesis of chronic COP as the underlying disease results in fibrotic lung disease or an initial acute exacerbation of an IPF with radiological features of COP. Moreover, the concurrent diagnosis of COP and IPF has been described previously [14]. In addition, a FVC/FEV1 of 67%, although within the lower limit of normal, is uncharacteristically low for an IPF patient without emphysema, which was not present in this patient, and might account for some bronchiolar involvement [15].…”

Bronchiolitis Obliterans Organizing Pneumonia:Clinicopathologic Review of a Series of 45 Korean Patients Including Rapidly Progressive form

“…There are some cases of idiopathic organizing DAD that show both clinical and histologic overlap with BOOP, and the distinction between the two may be somewhat arbitrary (16,17). Nagata et al (23) presumed that IPF with OP represents an incompletely resolved course of BOOP that is accompanied with fibrotic changes. However, patients with IPF and OP can not be classified as BOOP, because the honeycombing appearance on their chest radiography or CT scans is reportedly never seen in BOOP patients (1) and moreover, the presence of honeycombing as part of the interstitial pneumonia (and not an incidental finding) serves as evidence against the diagnosis of BOOP (16,17).…”