• Meng-Fang Wu, Yi-Shan Li, Chen-Yiu Hung, Wei-Chieh Chao, Zhey-Ying Fu, Kuo-Chin Kao, Chung-Chi Huang, Ying-Huang Tsai, and Meng-Jer Hsieh.
• Department of Respiratory Therapy.
• Respir Care. 2015 May 1;60(5):e101-4.

AbstractRelapsing polychondritis is a rare autoimmune disease causing inflammation in cartilaginous structures and other tissues throughout the body. Negative-pressure pulmonary edema (NPPE) due to laryngeal swelling from relapsing polychondritis is rare and has not been reported. Here, we report a case of relapsing polychondritis in an 18-y-old female who presented with recurrent NPPE and acute respiratory failure, which was diagnosed initially as ARDS during the influenza season. She underwent an emergent tracheotomy to relieve the upper airway obstruction resulting from severe laryngeal edema. A chest radiograph showed diffuse infiltrations, pneumothorax, and pneumomediastinum. The pulmonary infiltrations resolved rapidly in 2 d, and NPPE was diagnosed. Left ear swelling with erythematous change and saddle nose developed during the course of hospitalization, and an ear biopsy demonstrated severe cartilage necrosis. Relapsing polychondritis was diagnosed based on clinical images and pathological findings.Copyright © 2015 by Daedalus Enterprises.

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