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A6486 - Rare Case of Severe Cryptogenic Organizing Pneumonia Resistant to Steroids Plus Azathioprine
Author Block: H. S. Bukamur1, H. Mezughi2, Y. Shweihat3; 1Pulmonary, Joan C. Edwards School of Medicine, Marshall University, Huntington, WV, United States, 2Internal medicine, Joan C. Edwards School of Medicine, Marshall University, Huntington, WV, United States, 3Pulmonary Medicine, Joan C. Edwards School of Medicine, Marshall University, Huntington, WV, United States.
Introduction: Bronchiolitis Obliterans Organizing Pneumonia BOOP is a rare disorder of unknown etiology that can be divided into cryptogenic organizing pneumonia (COP) of unknown cause, and secondary organizing pneumonia associated with many recognized conditions. COP usually respond well to corticosteroid therapy with a good outcome. However, there are reports of patients with COP resistant to steroids with poorer out comes. We present a case of COP resistant to steroids that was treated with Azathioprine with no success. Case report:A 60-year-old female presented with 1-week history of progressive dyspnea with non-productive cough. On examination oxygen saturation 98 on 4 L of oxygen, had mild respiratory distress, diffuse bilateral rhonchi. CT chest with IV contrast demonstrated Multi lobar pneumonia with associated reactive lymphadenopathy in both hila and mediastinum. Serological tests for viruses, atypical organisms, hepatitis, Human immunodeficiency virus (HIV), Collagen vascular and autoimmune disease were negative. Open lung biopsy specimen showed an Organizing fibrinous pneumonia superimposed on background lung with smoking related interstitial fibrosis. The patient was started on IV Methylprednisolone 40 mg Q8H, IV Ceftriaxone and Doxycycline initially before result of biopsy for suspected COPD exacerbation and community acquired pneumonia, patient was switched to Prednisone 250 mg orally Q6H without any improvement. Azathioprine 50 mg daily was added. Repeat CT scan of the chest 19 days interval showed worsening of bilateral airspace disease. The patient had prolonged hospital admission with intubation and difficult weaning of MV and tracheostomy and she eventually died. Discussion: COP is a characteristic clinic-pathologic syndrome that is in general responds dramatically to steroids with a good outcome. However, with increasing experience of the clinical spectrum of COP, it is recognized that some patients exhibit rapidly progressive disease that is often refractory to steroids and is associated with a poor outcome. In our case COP was diagnosed by characteristic features of CT scan and Biopsy without any obvious cause. The patient was treated with high dose of steroids with no improvement and was switched to Azathioprine with deterioration of CT scan of the chest. Conclusion:To date there has been few case reports of patients treated successfully with non-steroidal agents (a combination Cyclosporine or Azathioprine plus steroids or cyclosporine A and Pirfenidone), thus there is little information currently available to guide treating this condition.We urge development of controlled studies to define the utility of these treatments and development of guidelines for treatment of COP resistant to steroids.