GOOD COP OR BAD COP: USE OF MACROLIDES TO ARREST THE INFLAMMATORY TURMOIL OF CRYPTOGENIC ORGANIZING PNEUMONIA

Abstract

TOPIC: Diffuse Lung Disease TYPE: Medical Student/Resident Case Reports INTRODUCTION: Cryptogenic organizing pneumonia (COP) represents a rare type of idiopathic interstitial pneumonia that is characterized by the recruitment of inflammatory cells and deposition of fibrinous material in the form of intraluminal plugs within alveolar ducts and surrounding alveoli. Clinical symptoms include subacute shortness of breath, cough, and fever, and radiological findings include peripheral, patchy, and peribronchovascular regions of ground-glass opacities (GGOs), consolidation on chest CT. CASE PRESENTATION: A 67-year-old man presented to the Pulmonology clinic with 3 months history of worsening shortness of breath(SOB) associated with a cough with white sputum. CT chest revealed widespread GGOs suspicious for a diffuse interstitial lung process. The physical exam was unremarkable except for some dry inspiratory crackles with no wheezing. Serum studies, including inflammatory markers such as sedimentation rate and C-reactive protein, were normal. Autoimmune assays and the Quantiferon Gold test for tuberculosis were negative. Pulmonary function testing was consistent with a restrictive pattern. Bronchoscopy with transbronchial biopsies was performed for further evaluation and negative for any malignant process or infectious etiologies. The patient was started empirically on prednisone with little symptomatic and radiologic improvement at a 2-month interval. Hypersensitivity pneumonitis or a nonspecific interstitial pneumonitis variant of interstitial lung disease were considered and, hence, mycophenolate was added to the patient's steroid regimen. Despite this intensive immunosuppressive therapy, the patient returned to the clinic with a worsening SOB and cough with yellowish, mucopurulent sputum. His immunosuppressive agents were stopped and a repeat bronchoscopy with transbronchial biopsies was obtained that was, again, inconclusive. A multidisciplinary discussion with Infectious Disease and Thoracic Surgery ensued and a surgical biopsy was obtained that revealed findings suggestive of chronic COP. Considering the patient's poor clinical response to steroid therapy alone, a trial of azithromycin was initiated following consultation with Pulmonologists at other US facilities. Patient-reported a remarkable improvement in symptoms at 3 months follow-up with a near resolution of GGOs on chest CT. DISCUSSION: COP is remarkably responsive to steroid therapy, but relapse is common. Steroid-sparing agents such as cyclophosphamide and azathioprine have been used as alternatives with inconsistent benefits and poor tolerability. The anti-inflammatory properties of macrolide antibiotics are well known and have been explored in recent times with few case reports showing promising results. CONCLUSIONS: This case report demonstrates the clinical utility of macrolide antibiotics in the management of COP and conveys the importance of exploring this approach further to improve clinical outcomes in COP. REFERENCE #1: Pathak V, Kuhn JM, Durham C, Funkhouser WK, Henke DC. Macrolide use leads to clinical and radiological improvement in patients with cryptogenic organizing pneumonia. Ann Am Thorac Soc. 2014 Jan;11(1):87-91. doi: 10.1513/AnnalsATS.201308-261CR. PMID: 24460438. REFERENCE #2: Stover DE, Mangino D. Macrolides: a treatment alternative for bronchiolitis obliterans organizing pneumonia? Chest. 2005 Nov;128(5):3611-7. doi: 10.1378/chest.128.5.3611. PMID: 16304320. DISCLOSURES: No relevant relationships by Tania Dhawan, source=Web Response No relevant relationships by Faisal Siddiqui, source=Web Response No relevant relationships by Naresh Veerabattini, source=Web Response