View Abstract

A6544 - Fatal Acute on Chronic Respiratory Failure Due to Cryptogenic Organizing Pneumonia in the Setting of Systemic Lupus Erythematosus
Author Block: P. Sankhyan1, A. Mahajan2, M. F. Khalid2, S. Bhogal1, G. Falasca2; 1Internal Medicine, East Tennessee State University, Johnson City, TN, United States, 2Internal Medicine, East Tennessee State University, Johnson city, TN, United States.
INTRODUCTION : Cryptogenic Organizing Pneumonia (COP) is an idiopathic interstitial lung disease that has a similar presentation as infectious pneumonia with fever, dyspnea and non productive cough but does not respond to antibiotic therapy. We came across a rare case of COP in the setting of steroid dependent Systemic Lupus Erythematosus (SLE) that lead to respiratory failure, recurrent pulmonary infections and prolonged intensive care unit stay that ultimately turned fatal.
CASE : A 43 year old lady with a history of COP, steroid dependent SLE, rheumatoid arthritis, diabetes mellitus, venous thromboembolism, paraplegia with chronic indwelling foley catheter, presented with acute on chronic respiratory failure requiring endotracheal intubation followed by tracheostomy. She was suspected to have a recurrence of COP and was not able to be weaned off an FiO2 of 60%. During the hospital course she developed ventilator associated pneumonia, Staphylococcus hominis bacteremia and multi drug resistant urinary tract infection leading to multi organ failure and ultimately died after being placed on comfort care. She was diagnosed with COP 2 years prior to this hospitalization when she was thought to have bilateral pneumonia but failed to respond to antibiotic therapy. A biopsy of the left lower lobe of the lung showed bronchiolitis. She was treated with high dose steroids followed by chronic steroid therapy and improved clinically at the time.
DISCUSSION/ CONCLUSION : COP ( previously known as Bronchiolitis Obliterans Organizing Pneumonia or BOOP) is an interstitial lung disease that primarily affects the distal and respiratory bronchioles. It occurs due to injury to the alveolar walls, leading to formation of granulation tissue. The etiology of COP is unclear and it has been postulated to be associated with toxin inhalation, drugs and malignancy. However, its association with SLE, as seen in our case is relatively rare. The diagnostic modality of choice is lung biopsy. Timely initiation of steroid therapy is crucial and patients with early administration of steroids have a much better prognosis than those in whom steroid therapy is delayed. In patients who fail to respond to high dose steroid therapy, another immunosuppressive agent may lead to improvement. It is crucial to think of COP as a differential in case of suspected pulmonary inflammatory disease, especially in non-smokers with no prior history of chronic obstructive pulmonary disease, asthma or other interstitial lung diseases. Our case highlights an unusual complication of COP in the setting of SLE.