Limited auricular relapsing polychondritis in a child treated successfully with infliximab

Abstract

Relapsing polychondritis (RP) is a rare progressive and destructive multisystem disorder characterised by recurrent inflammation of cartilaginous structures. It is a rare disease in paediatrics compared with adults. In children, the diagnosis is either delayed or overlooked due to low incidence. Auricular chondritis has been described in more than half of paediatric cases with RP. However, isolated auricular chondritis has not been reported as the only presentation of pediatric-onset RP. We described a lad who presented with isolated auricular chondritis, which is refractory to conventional treatment, including glucocorticoids and methotrexate as steroid-sparing agent. Remission of his disease’s relapses was sustained with infliximab. Limited auricular involvement as a presenting feature of RP in the absence of systemic association is very rare in children. We describe a case of successful use of infliximab on limited auricular chondritis disease.

Clinical patterns and the evolution of relapsing polychondritis based on organ involvement: a Chinese retrospective cohort study

“…Meanwhile, partial RPC has been reported by Mathew et al [11] as well as in our previous study [6]. Emerging data suggest that limited RPC are not uncommon in clinical practice [6,[17][18][19][20][21][22][23][24].…”

“…Patients with laryngo-tracheal involvement or auricular in ammation as the only initial feature of the disease [13], sometimes as the sole feature [8,[17][18][19][20][21][22][23][24], have been de ned as limited form of RPC. In our cohort, 52.3% of the patients presented as limited RPC initially and 40.5% of them remained limited form during follow-up, suggesting that limited RPC is not uncommon among Chinese patients.…”

“…Patients that underwent recurrent chondritis associated with deformity, vestibular dysfunction, ocular in ammation, or in ammatory arthritis were diagnosed as partial RPC as suggested by Mathew et al [11]. Limited RPC can also be diagnosed if patients presented with recurrent in ammatory episodes at isolated cartilaginous sites after exclusion of other possible causes and are responsive to glucocorticoids, based on the observations by other groups and our own [6,8,13,[17][18][19][20][21][22][23][24] . The diagnostic criteria used in present study were listed in table 4.…”

Clinical patterns and the evolution of relapsing polychondritis based on organ involvement: analysis of 126 Chinese patients

“…Meanwhile, partial RPC has been reported by Mathew et al [11] and our previous study [6] , and limited RPC is not uncommon in clinical settings as demonstrated by our previous study [6] and case reports [17][18][19][20][21][22][23][24]. As a result, these patients do not meet with the current criteria.…”

“…Patients that underwent recurrent chondritis associated with deformity, in accompany with vestibular dysfunction, ocular inflammation, or inflammatory arthritis were diagnosed as partial RPC as suggested by Mathew et al [11]. Limited RPC can also be diagnosed if patients presented with recurrent inflammatory episodes at isolated cartilaginous sites after exclusion of other possible causes and are responsive to glucocorticoids, based on the observations by other groups and our own [8,13,[17][18][19][20][21][22][23][24] and our own experience. Patients younger than 18 years or without complete electronic case files were excluded.…”