Cryptogenic organizing pneumonia history and symptoms

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Template:Cryptogenic organizing pneumonitis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Symptoms

  • Cough
  • Dyspnea
  • Influenza-like symptoms
  • Febrile illness
  • Widespread crackles
  • Mild resting hypoxemia

On examination, crackles are common, but clubbing is not. Laboratory findings are nonspecific. On HRCT, airspace consolidation with air bronchograms is present in more than 90% of patients, often with a lower zone predominance. A subpleural or peribronchiolar distribution is noted in up to 50% of patients. Ground glass or hazy opacities associated with the consolidation are detected in most patients. Pulmonary physiology is restrictive with a reduced DLCO. Airflow limitation is uncommon; gas exchange is usually abnormal and mild hypoxemia is common. Bronchoscopy with BAL reveals up to 40% lymphocytes, along with more subtle increases in neutrophils and eosinophils. In patients with typical clinical and radiographic features, a transbronchial biopsy that shows the pathologic pattern of organizing pneumonia and lacks features of an alternative diagnosis is adequate to make a tentative diagnosis and start therapy. On surgical lung biopsy, the histopathologic pattern is organizing pneumonia with preserved lung architecture; this pattern is not exclusive to BOOP and must be interpreted in the clinical context. Most patients recover with corticosteroid therapy. A standardized approach to dosing starting at 0.75 mg/kg and weaning over 24 weeks has been shown to reduce total corticosteroid exposure without affecting outcome.

References