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Rapidly progressive bronchiolitis obliterans with organizing pneumonia
A J Cohen1, T E King, G P Downey
1Department of Medicine, National Jewish Center for Immunology and Respiratory Medicine, Denver, CO 80206.
Summary
Rapidly progressive Bronchiolitis obliterans with organizing pneumonia (BOOP) can cause severe respiratory failure and death. This severe form of BOOP may present as early lung injury with rapid progression to fibrosis.
Area of Science:
- Pulmonology
- Pathology
- Internal Medicine
Background:
- Bronchiolitis obliterans with organizing pneumonia (BOOP) is a distinct clinicopathologic syndrome.
- BOOP typically shows a good response to therapy, with uncommon fatalities.
- A rapidly progressive form of BOOP, however, can lead to severe outcomes.
Observation:
- This study details 10 patients with rapidly progressive BOOP and severe respiratory failure.
- Clinical symptoms included dyspnea, cough, fever, crackles, and resting hypoxemia.
- Associated factors included connective-tissue disease, avian exposure, and nitrofurantoin use.
Findings:
- All patients exhibited characteristic BOOP histopathology.
- Autopsies in 6 patients revealed predominant alveolar septal inflammation and fibrotic honeycombing.
- Seven patients died, and three survivors had persistent pulmonary dysfunction; two showed disease progression.
Implications:
- A subset of BOOP patients presents with a fulminant course, resulting in mortality or chronic, severe lung fibrosis.
- BOOP's histologic pattern may represent early lung injury with potential for rapid progression to end-stage fibrosis.