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[Clinical characteristics of 6 cases of relapsing polychondritis in children with airway involvement].
Zhonghua 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.
To investigate the pathogenesis, clinical signs and diagnosing procedures of relapsing polychondritis(RP) in children with airway involvement. The medical history, clinical symptoms, physical examination, electronic laryngoscopy and imaging findings of six patients were retrospectively analyzed. The patients diagnosed as relapsing polychondritis with involving the airway from January 2018 to December 2021 were in our hospital.
View Article and Find Full Text PDFRelapsing polychondritis in childhood: A case report.
Medicine (Baltimore)
October 2024
Department of Otorhinolaryngology, Linyi People's Hospital, Linyi, China.
Article Synopsis
- - Relapsing polychondritis (RP) is a rare autoimmune disease that harms cartilage, particularly in the ears, nose, and airways, making early diagnosis vital to avoid severe airway damage and complications, as highlighted by a case study of a 12-year-old girl.
- - The patient faced severe respiratory distress and underwent tracheal intubation and later a tracheostomy, with symptoms evolving to joint pain and a saddle nose deformity, leading to a clinical diagnosis of RP.
- - Treatment included antibiotics, corticosteroids, and the biologic drugs tocilizumab and methotrexate, and three years after diagnosis, the girl is stable, underscoring the importance of prompt recognition of
Systemic amyloidosis: an aggressive evolution in a patient with relapsing polychondritis and monoclonal gammopathy of undetermined significance (MGUS) undergoing peritoneal dialysis.
Autops Case Rep
September 2024
Universidade de São Paulo (USP), Faculdade de Medicina, Hospital das Clínicas, Serviço de Reumatologia, São Paulo, SP, Brasil.
Article Synopsis
- A case study of a female patient in her 50s with primary amyloidosis affecting multiple organs, alongside a history of relapsing polychondritis and chronic kidney disease.
- Initial tests were negative for amyloid but a second biopsy confirmed amyloid protein presence as AL-lambda amyloidosis.
- Despite medical interventions, the patient experienced worsening symptoms, eventually leading to ICU admission and death due to pulseless ventricular tachycardia, emphasizing the importance of accurate diagnosis for treatment decisions.
Diagnostic Challenges and Management of Relapsing Polychondritis with Large-Airway Involvement: A Case Series and Literature Review.
Life (Basel)
September 2024
Division of Pediatric Otolaryngology, Aerodigestive and Esophageal Center, Cincinnati Children's Hospital Medical Center, Cincinnati, OH 45229, USA.
Article Synopsis
- The study investigates the challenges in diagnosing and managing relapsing polychondritis (RP) with airway involvement, emphasizing the importance of accurate diagnosis to avoid serious complications.
- Among 34 patients diagnosed with RP, 4 showed severe airway issues after being misdiagnosed with asthma, highlighting the need for better diagnostic practices.
- Key interventions included steroid treatment, rituximab, and various surgical procedures; early identification of RP and a multidisciplinary approach are crucial for improving patient outcomes and reducing the risk of complications.
Comprehensive Interventional treatment for severe central airway collapse caused by Relapsing Polychondritis: A case report.
Heliyon
September 2024
Respiratory Department, Dongzhimen Hospital Affiliated to Beijing University of Chinese Medicine, Beijing, 100700, China.
Article Synopsis
- Relapsing Polychondritis (RP) is a rare and serious inflammatory disease that can lead to life-threatening airway collapse.
- A patient who was misdiagnosed with refractory asthma experienced severe airway collapse, requiring advanced treatments like bronchoscopic laser tracheobronchoplasty and stent placement.
- After successful stabilization and discharge, the patient managed their symptoms with a tapering glucocorticoid regimen and did not require rehospitalization.
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