P50 A case of juvenile relapsing polychondritis with rapidly developing respiratory complications
Authors: Dawson Pamela , Al-Abadi Eslam 
Published: October 2019

Background

Relapsing polychondritis in childhood is a rare multi-system disease characterised by inflammation of cartilage and can be life-threatening with complications of the larynx, trachea and cardiovascular system. It is often a delayed diagnosis with a more severe course than in adults; there is higher incidence of laryngotrachobronchial involvement, which can occur early and is often unresponsive to conventional therapy.

Methods

A previously healthy 14-year-old of African descent presented to a children’s hospital with unilateral ear pain and swelling, lower anterior rib pain and red, painful eyes. Initial work-up showed microcytic anaemia and raised inflammatory markers. Initially treated with broad-spectrum antibiotics, markers of inflammation continued to rise and she became febrile. After discussion with tertiary paediatric rheumatology, polychondritis was suspected and high dose oral steroids were commenced, improving all symptoms.

She was seen in tertiary paediatric rheumatology clinic and steroids were weaned. She quickly relapsed with fever, chest pain, cough, sore throat, dizziness and vomiting. On examination there was pain and swelling of the nasal bridge and cricoid tenderness. The left ear was swollen, warm and tender. She had tinnitus in the right ear. Further investigation showed high inflammatory markers, anterior uveitis and right sensorineural hearing loss. Management was pulsed intravenous methylprednisolone followed by oral Prednisolone as induction, with plans for methotrexate commencement. Parental concerns and missed appointments prevented methotrexate initiation; the family wished to withdraw steroids over two months before further treatment decision.

On weaning steroids she developed stridor, barking cough, hoarse voice and dyspnoea. Saturations remained normal despite moderate work of breathing. Flexible nasendoscopy showed interarytenoid and subglottic oedema and likely trachobronchitis. She was commenced on CPAP, rituximab and a six-month course of cyclophosphamide. Due to parental concern and stress, relationships with healthcare professionals became increasingly challenging.

Five months after initial presentation, she underwent semi-elective intubation owing to biphasic stridor and increased work of breathing. Subsequently extubation proved difficult and she was transferred to a specialist centre for ongoing management.

Results

The patient was subsequently extubated to BiPAP and completed cyclophosphamide infusions. Multi-speciality national teleconference agreed upon methotrexate and anti-TNF maintenance treatment with gradual wean of steroids. She has both saddle-nose and ear deformities, in addition to BiPAP-dependant airway stenosis. Discussions regarding airway reconstruction are ongoing with a national specialist team.

Conclusion

Relapsing polychondritis in childhood and adolescence is rare and more aggressive than in adults. This case demonstrates the speed destructive features can develop despite early treatment with steroids. Without aggressive and prompt treatment, life-threatening complications can develop rapidly and outcomes are poor. Also highlighted is potential airway reconstruction in collaboration with specialist centres.

Excellent communication between healthcare professionals and with families is essential, especially when care of a complex and rare disease is shared between multiple centres.

Conflicts of Interest

The authors declare no conflicts of interest.