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Bronchiolitis obliterans organizing pneumonia and scleroderma.

A J Bridges1, K C Hsu, A A Dias-Arias

  • 1Department of Medicine and Pathology, University of Missouri-Columbia School of Medicine.

The Journal of Rheumatology
|July 1, 1992
PubMed
Summary

Scleroderma patients can develop bronchiolitis obliterans organizing pneumonia (BOOP), a rare condition causing rapid lung damage. Early lung biopsy is crucial for identifying this steroid-responsive complication.

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Area of Science:

  • Pulmonology
  • Rheumatology
  • Pathology

Background:

  • Scleroderma commonly causes interstitial lung disease and pulmonary hypertension.
  • Bronchiolitis obliterans organizing pneumonia (BOOP) is rarely reported in scleroderma patients.

Observation:

  • Two scleroderma patients presented with rapidly progressing pulmonary infiltrates.
  • Histological analysis via open lung biopsy revealed BOOP in both cases.
  • Both patients exhibited severe restrictive lung disease and reduced diffusion capacity.

Findings:

  • BOOP is an uncommon but significant cause of acute respiratory deterioration in scleroderma.
  • High-dose corticosteroid therapy showed a positive response in one patient.
  • The rapid progression of pulmonary symptoms differed from typical scleroderma-related fibrosis.

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Implications:

  • Rapid pulmonary decompensation or atypical findings in scleroderma warrant consideration for lung biopsy to diagnose BOOP.
  • Identifying steroid-responsive BOOP offers therapeutic potential in scleroderma patients with lung involvement.
  • This finding highlights the importance of considering alternative diagnoses in refractory lung disease.