Relapsing polychondritis is an immune disorder of unknown etiology involving multiple systems that is characterized by persistent inflammation and destruction of cartilage, including the ears, nose, costal, joint, and airways. Airway involvement caused by relapsing polychondritis is common, and tracheobronchomalacia is the most serious complication, which is life-threatening. Currently, the exact mechanism of relapsing polychondritis with tracheobronchomalacia is unknown. Although glucocorticoids and immunosuppressive agents are administered, failures often occur. Currently, bronchoscopy-guided intervention therapy used in tracheobronchomalacia caused by chronic obstructive pulmonary disease or other etiology has gradually increased, but bronchoscopy-guided intervention therapy with extracorporeal membrane oxygenation assist used in tracheobronchomalacia caused by relapsing polychondritis has not been reported. Here, we report a case of relapsing polychondritis with severe tracheobronchomalacia. Although drug therapy was provided and airway stent implantation was performed, the tracheal stenosis was further aggravated. Because conventional anesthesia and mechanical ventilation cannot meet the needs of bronchoscopy-guided intervention therapy or guarantee sufficient safety. The intervention treatment was performed with the support of extracorporeal membrane oxygenation, which was successfully completed without obvious complications. The symptoms were significantly improved, and the patient was discharged uneventfully.
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http://dx.doi.org/10.3389/fmed.2021.695505 | DOI Listing |
Immunotherapy
December 2024
Department of Clinical Immunology and Allergy, Flinders Medical Centre, Bedford Park, South Australia.
Relapsing polychondritis is rare and affects non-synovial fibrocartilage. Currently, there is a paucity of treatment algorithms, especially for those with refractory disease. A middle-aged man presented with polychondritis affecting the nose, ears, joints, and larynx.
View Article and Find Full Text PDFRelapsing polychondritis (RP) is a rare immune-mediated systemic inflammatory disease with diverse clinical manifestations. Independent involvement of the respiratory system in RP is uncommon. In the event of respiratory involvement as the initial airway-only manifestation, the diagnosis of RP is challenging and might be delayed, and patients with respiratory involvement exhibit a poor prognosis.
View Article and Find Full Text PDFZhonghua Er Bi Yan Hou Tou Jing Wai Ke Za Zhi
November 2024
Department of Otorhinolaryngology Head and Neck Surgery, Beijing Children's Hospital Affiliated to Capital Medical University, Beijing Key Laboratory for Pediatric Diseases of Otolaryngology Head and Neck Surgery, National Center for Children's Health(NCCH), Beijing100045, China.
Rev Prat
November 2024
Service de médecine interne 2, centre de référence des maladies auto-immunes et autoinflammatoires systémiques rares de l'adulte d'Île-de-France, Centre et Martinique, hôpital de La Pitié-Salpêtrière, AP-HP, Paris, France. Association francophone contre la polychondrite chronique atrophiante (AFPCA).
Int J Rheum Dis
November 2024
Department of Allergy and Rheumatology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan.
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