Relapsing polychondritis in childhood: three case reports, comparison with adulthood disease and literature review

Abstract

Relapsing polychondritis (RP) is a rare autoimmune systemic disease, especially in childhood. To report three new pediatric RP cases, to provide a literature review and to compare with adulthood disease, retrospective data collection from three childhood RP cases was observed in a Brazilian Pediatric Rheumatology Division. A literature review based on a MEDLINE database search was performed. Arthritis and auricular chondritis were present in our three patients. Two cases presented with early and severe laryngotracheal chondritis, besides initial and symptomatic costochondritis. The other case developed prominent epiphyseal plate involvement. Two patients were refractory to corticosteroids and immunosuppressants and required the use of TNF-alpha inhibitors to improve the symptoms, while corticosteroids plus methotrexate induced remission in the other patient. The literature review showed 44 cases of pediatric-onset disease in English language. Arthritis and ear chondritis are the most common initial and cumulative manifestations of RP in children and adults. Nasal and laryngotracheobronchial chondritis are also common manifestations observed during follow-up in childhood. There is also an early severity of respiratory chondritis in childhood, requiring aggressive treatment with corticosteroids, immunosuppressants and biologic agents. The data presented by those 3 children, considered in conjunction with the data from the 44 published cases, may reflect some distinguishing childhood RP features, such as more severe and frequent respiratory tract involvement, symptomatic costochondritis and the atypical pattern of persistent and destructive arthritis with epiphyseal plate involvement. Response to immunosuppressants and biologic agents is anecdotal, but steroids remain the main drug during the flares.

Relapsing polychondritis: Clinical presentations, disease activity and outcomes

“…The youngest patient in this series was 14 years old. Childhood RP has been described and its clinical presentations are similar to adult RP [13][14][15]. A family history of autoimmune disease is often noted in these patients although they infrequently have coexistent autoimmune diseases themselves [15].…”

Multisystem Inflammatory Disease with Respiratory Failure in a 7-Year-Old Boy

“…With lower respiratory tract involvement, mortality in RP increases to nearly 50%. Literature review found increased utilization of tracheostomy in pediatric patients with respiratory involvement [2]. Respiratory chondritis (nasal or laryngotracheobronchial) is often asymptomatic earlier in disease.…”

Three Cases of Relapsing Polycondritis with Isolated Laryngotracheal Stenosis

“…While the auricle (83%) is classically affected most commonly in adults, the larynx and trachea (50%), nasal, costal cartilage, joints, eye (20-60%), skin (36%), and heart (<%10) are other anatomical structures that can be affected (4). Joints (36%), auricles (27%), and airway (25%) are more commonly affected in children (9). Although the pathogenesis of RP remains uncertain, the response that emerges with the deterioration of the immune balance and the association with HLA-DR4 are believed to be at least partially responsible (5).…”