Relapsing polychondritis in childhood: A case report

Relapsing polychondritis (RP) is a rare immune-mediated disease that leads to progressive cartilage destruction, notably affecting the ears, nose, and airways. Timely diagnosis is essential to prevent irreversible airway damage and life-threatening complications. This study presents a case of a 12-year-old child diagnosed with RP, emphasizing the critical need for timely identification of RP in children.

A 12-year-old girl was admitted with worsening hoarseness and cough, leading to respiratory distress and severe bradycardia, requiring transfer to the pediatric intensive care unit. After successful resuscitation and tracheal intubation, imaging showed significant subglottic swelling. She received antibiotics, corticosteroids, and nebulization. Despite initial stabilization, she required a tracheostomy 2 months post-discharge due to recurrent dyspnea. Six months later, she developed joint pain and a saddle nose deformity.

The patient was clinically diagnosed with RP in accordance with the diagnostic standards set forth by Micheet et al.

During the initial phase of treatment, the patient was administered antibiotics, corticosteroids, and nebulization therapy. Due to severe respiratory distress, an emergency tracheostomy was performed by ear, nose, and throat surgeons. After a definitive diagnosis, the patient was treated with tocilizumab, methotrexate, and corticosteroid therapy. Additionally, supportive measures including calcium supplementation, gastric protection, and immune support were provided.

Three years post-diagnosis, the patient's condition is stable.

Due to RP low prevalence, diagnosis in children is frequently delayed or overlooked. Notably, involvement of the major airways is more common and severe in pediatric patients compared to adults. It is crucial for Otolaryngologists to have a comprehensive understanding of this condition to effectively diagnose and manage complications associated with RP.