CRP2 A RARE CAUSE OF SEVERE UPPER AIRWAY OBSTRUCTION-A DIAGNOSTIC DILEMMA AND MANAGEMENT CHALLENGE.

Hasmiza, Mariana
Hospital Raja Perempuan Zainab II, Kelantan, Malaysia

Introduction
Relapsing Polychondritis (RPC) is a rare autoimmune disorder characterized by recurrent inflammation of cartilaginous structures, including the ears, nose, joints, eyes, and respiratory tract. Pediatric RPC is uncommon and often presents with non-specific symptoms. Although airway involvement can be life-threatening, it is rarely the initial manifestation.

Case Report
We report a previously healthy 12-year-old girl who experienced five episodes of upper airway obstruction over several months. The first episode presented with stridor and dyspnea, with neck imaging suggestive of a retropharyngeal abscess. Bronchoscopy revealed a smooth, spindle-shaped subglottic stenosis. She responded to corticosteroids and antibiotics, but symptoms recurred with steroid tapering and viral infections. During the fourth admission, she developed acute airway obstruction and experienced an apparent life-threatening event (ALTE), requiring emergency tracheostomy. Direct laryngoscopy showed severe circumferential subglottic edema. By the fifth admission, systemic features emerged—pinna pain, chest pain, conjunctivitis, and costochondritis—fulfilling four out of six diagnostic criteria for RPC. Despite aggressive immunosuppression with intravenous methylprednisolone, methotrexate, cyclophosphamide, and mycophenolate mofetil, she experienced disease progression complicated by steroid toxicity and recurrent infections. Subcutaneous adalimumab was initiated with partial response.

Discussion
This case highlights subglottic stenosis as the first presentation of RPC, a very rare occurrence, particularly in children. Early episodes mimicked infectious airway disease, delaying diagnosis. Progressive airway involvement underscores the importance of considering RPC in children with recurrent stridor or unexplained subglottic stenosis, especially when systemic features develop.

Conclusion
RPC should be considered in pediatric patients with recurrent upper airway obstruction. Early diagnosis and a multidisciplinary approach are crucial to prevent severe complications and manage disease progression effectively.