title: Relapsing polychondritis with initial presentations of recurrent negative-pressure pulmonary edema and acute respiratory failure.
abstract: Relapsing 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.
authors.affiliation: Department of Respiratory Therapy.
authors.forename: Meng-Fang
authors.initials: MF
authors.lastname: Wu
authors.affiliation: Department of Respiratory Therapy.
authors.forename: Yi-Shan
authors.initials: YS
authors.lastname: Li
authors.affiliation: Department of Pulmonary and Critical Care Medicine.
authors.forename: Chen-Yiu
authors.initials: CY
authors.lastname: Hung
authors.affiliation: Department of Otolaryngology, Linkou Chang-Gung Memorial Hospital, Chang-Gung Medical Foundation, Taoyuan County, Taiwan.
authors.forename: Wei-Chieh
authors.initials: WC
authors.lastname: Chao
authors.affiliation: Department of Pulmonary and Critical Care Medicine.
authors.forename: Zhey-Ying
authors.initials: ZY
authors.lastname: Fu
authors.affiliation: Department of Respiratory Therapy Department of Pulmonary and Critical Care Medicine Department of Respiratory Therapy, Chang-Gung University, Taoyuan County, Taiwan.
authors.forename: Kuo-Chin
authors.initials: KC
authors.lastname: Kao
authors.affiliation: Department of Respiratory Therapy, Chang-Gung University, Taoyuan County, Taiwan.
authors.forename: Chung-Chi
authors.initials: CC
authors.lastname: Huang
authors.affiliation: Department of Respiratory Therapy Department of Pulmonary and Critical Care Medicine, Chiayi Chang-Gung Memorial Hospital, Chang-Gung Medical Foundation, Chiayi County, Taiwan. Department of Respiratory Therapy, Chang-Gung University, Taoyuan County, Taiwan.
authors.forename: Ying-Huang
authors.initials: YH
authors.lastname: Tsai
authors.affiliation: Department of Respiratory Therapy Department of Pulmonary and Critical Care Medicine, Chiayi Chang-Gung Memorial Hospital, Chang-Gung Medical Foundation, Chiayi County, Taiwan. Department of Respiratory Therapy, Chang-Gung University, Taoyuan County, Taiwan. mengjer@yahoo.com.
authors.forename: Meng-Jer
authors.initials: MJ
authors.lastname: Hsieh
mesh_terms: D000293:Adolescent; D003937:Diagnosis, Differential; D004423:Ear / Q000473:pathology; D005260:Female; D006801:Humans; D007830:Larynx / Q000473:pathology; D011081:Polychondritis, Relapsing / Q000150:complications* / Q000175:diagnosis; D011654:Pulmonary Edema / Q000209:etiology*; D012131:Respiratory Insufficiency / Q000209:etiology*
keywords: acute respiratory failure; barotrauma; negative-pressure pulmonary edema; relapsing polychondritis