Objective: To explore the clinical features of rapidly progressive crytogenic organizing pneumonia (COP).
Methods: Nine cases of rapidly progressive COP with acute respiratory failure confirmed by lung biopsy at First Affiliated Hospital, Nanjing Medical University from October 2003 to March 2014 were retrospectively reviewed.
Results: There were 5 males and 4 females with an age range of (52.1 ± 11.4) years. The onset of illness was 7-20 days. The major manifestations included cough (n = 9), dyspnea (n = 9), fever (n = 7), hypodynamia (n = 6), weight loss (n = 3), night sweat (n = 2), hemoptysis (n = 1), cyanosis (n = 9), "velcro" crackles (n = 6) and moist rales (n = 3). Blood tests showed elevated levels of erythrocyte sedimentation rate (n = 8), C-reactive protein (n = 8) and serum ferritin (n = 6). The results of blood gas analysis indicated acute respiratory failure in all 9 patients (PaO2<60 mmHg, 1 mmHg = 0.133 kPa). Chest computed tomography showed bilateral and multiple distribution, patchy areas of alveolar consolidation with air bronchograms (n = 5), diffuse ground-glass opacity (n = 4), tractional bronchiectasis (n = 2), pleural effusion (n = 2) and spontaneous pneumothorax (n = 1). Six cases were cured by conventional dose corticosteroid and another 3 by large-dose methylprednisolone (240 mg/d). Two cases relapsed and then recovered after a second corticosteroid therapy.
Conclusions: The clinical manifestations of rapidly progressive COP show severe inflammatory reactions with a fast progression. Chest computed tomography depicts extensive lesions along with pleural effusion and spontaneous pneumothorax. Conventional dose of corticosteroid is the first choice therapy for rapidly progressive COP. Large-dose methylprednisolone is needed when conventional dose of corticosteroid proves ineffective.