[Relapsing polychondritis in childhood: report of three cases and review of the literature].

OBJECTIVE

To analyze the clinical features, diagnosis, treatment and prognosis of relapsing polychondritis (RP) in childhood.

METHODS

A retrospective analysis of three cases of childhood RP from our hospital on clinical features, diagnosis and treatment was performed, data of the other sixteen cases from MEDLINE were also reviewed. Clinical features of all nineteen children with RP were compared with adults with RP.

RESULTS

The age of the three cases at the time of diagnosis ranged from 10 years to 15 years (the minimum age was 2 years in the literature). The course from onset of clinical symptom to making definite diagnosis varied from 3 months to 9 months (from 3 months to 2 years in the literature). In the three cases, childhood RP affected multiple system/organs, and produced diverse clinical manifestations such as arthritis, auricular chondritis, nasal chondritis (leading to a saddle nose deformity), conjunctivitis and so on. Also, tracheostomy was performed because of severe difficulty in breathing for all the 3 cases. Compared with adult RP, involvement of respiratory system existed in 78.9% of childhood patients, which was more frequent and serious than that of adult cases with RP (35.2%); arthritis was the most common clinical manifestation and first symptoms; Fewer children with RP were associated with other autoimmune diseases than adults. Although both erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) blood levels were high in the three cases, anti-nuclear antibody (ANA), double stranded DNA (dsDNA) and extractable nuclear antigen (ENA) were all negative, it was difficult to define the diagnosis of RP because there were no specific laboratory indicators for diagnosis. The confirmation of diagnosis of two cases was relied on clinical criteria, while another case got pathological material for diagnostic support. CT images with three-dimensional reconstruction of the respiratory tract were helpful for early diagnosis of childhood RP. All the three patients were responsive to glucocorticoid, especially to methylprednisolone, however, recurrence of RP was frequent. Treatment with etanercept was successful in one case with refractory RP.

CONCLUSIONS

It is very important to obtain detailed medical history, complete physical examination and do necessary laboratory and imaging tests (e.g. CT images with three-dimensional reconstruction of the airways, pulmonary function tests and so on) for reducing misdiagnosis or missed diagnosis. Glucocorticoid and immunosuppressants are usually effective for childhood RP, but the effects may not be lasting. Etanercept may be a new option to treat refractory RP in childhood.