Chest
Volume 96, Issue 5, November 1989, Pages 999-1004
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Clinical Investigations
Idiopathic Bronchiolitis Obliterans Organizing Pneumonia: Definition of Characteristic Clinical Profiles in a Series of 16 Patients

https://doi.org/10.1378/chest.96.5.999Get rights and content

Bronchiolitis obliterans organizing pneumonia (BOOP) is a pathologic finding common to various injuries to the lung of either definite or idiopathic etiology. Since the presentation of patients with idiopathic BOOP varies, we studied 16 patients with BOOP on pulmonary histology to define more distinct and homogeneous clinical and imaging profiles of idiopathic BOOP. We distinguished three groups of patients: group 1 (n=4), with multiple patchy migratory pulmonary involvement of the pneumonia type. Their clinical course was subacute, with cough, fever, weight loss, mild dyspnea, and increased ESR. Chest x-ray film and CT scan showed multiple alveolar opacities. All patients completely recovered with corticosteroid therapy but relapsed when therapy was stopped too rapidly. Group 2 (n=5) had solitary pulmonary involvement of the pneumonia type occurring in a similar clinical context. Since carcinoma was suspected, they underwent surgical excision of the pneumonic area and recovered without relapse. Group 3 patients (n=7) presented with diffuse pulmonary involvement of the interstitial lung disease type. They had more progressive onset of more severe dyspnea, crackles heard over all lung surfaces, and interstitial opacities with or without alveolar opacities on chest imaging. Improvement with corticosteroid therapy was obtained in only three patients. In all three groups, lung function test results showed a restrictive pattern. The obstructive pattern characteristic of pure bronchiolitis obliterans was found in none. BAL showed a mixed pattern (increase of both lymphocytes and polymorphonuclear cells) in the patients of the first two groups. Thus, we distinguished three characteristic clinical and imaging profiles in patients with idiopathic BOOP: multiple patchy pneumonia, solitary pneumonia, and diffuse interstitial lung disease. These profiles are so different that they should be distinguished in clinical studies of idiopathic BOOP.

Section snippets

Materials and Methods

We selected from the files of the Department of Pathology all the cases of patent intraluminal fibrosis of distal airspaces observed from 1982 to August 1988 on pulmonary tissue obtained by open lung biopsy, pulmonary excision (pneumonectomy, lobectomy, segmentectomy), or autopsy.

Results

Intraluminal fibrosis of distal airspaces (BOOP) was present in 21 patients. Clinical information was insufficient for correct analysis in one case. A definite etiology was found in three cases (sarcoidosis, pulmonary vasculitis, and hyperacute radiation pneumonitis), and in one case of interstitial lung disease a reaction to amiodarone was suspected.

The remaining 16 patients with idiopathic pulmonary disease and BOOP were included in the present study. The pulmonary tissue examined had been

Discussion

The present study delineated homogeneous clinical and imaging profiles in a series of patients with idiopathic pneumonitis whose common denominator was the presence of intraluminal fibrosis of distal airspaces (BOOP).

Multiple patchy pneumonia was the most characteristic presentation of patients with BOOP in this series. The patients with “cryptogenic organizing pneumonitis” reported by Davison et al3 fell into this category, as did most patients of Epler et al.5 Nevertheless, this presentation

ACKNOWLEDGMENTS

We thank L. D. Gruer for reviewing the translation and M. C. Thévenet for secretarial assistance.

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Manuscript received November 30; revision accepted March 6

This work was supported by grant 86 MR/4 from Fonds Spécial des Comités Départementaux, Comité contre les Maladies Respiratoires et la Tuberculose.

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