Clinical manifestation of child-onset relapsing polychondritis / 中华风湿病学杂志

ABSTRACT

Objective To investigate the clinical features of child-onset relapsing polychondritis (CORP). Methods The clinical manifestations of 11 CORP cases were analyzed retrospectively. Results Eleven cases of CORP were collected with Male to Female ratio of 5 ∶6; the mean age of disease onset was (11±4)(3-15) years; and the mean age of final diagnosis was(16±5)(6-22) years-old. The mean time of delay of diagnosis was(55±46)(2-144) month. Nine cases were simple CORP and 2 cases were concurred with SLE and pSS respectively. Laryngotracheal symptoms as initial presentation was seen in 7/11(64%) of cases, followed by rhinitis, eye involvement and arthritis. Laryngotracheal involvement occurred in 10/11 (91%) cases and 6/11(55%) underwent trachectomy when the diagnosis was made, followed by eye and nose involvement (saddle nose)(46% each), involvement of auricular cartilage,vestibule disorder and arthritis (36% each). Elevation of ESR or CRP(36%) were observed and positive autoantibody(18%) were detected in serology study; 9/11 (82%) were treated with steroid combined immunosuppressant. All cases were improvedor stable except 2 cases with exacerbation after upper respiratory infection during the follow-up of mean(27±15)(4-48) month. Conclusion Laryngopharyngeal involvement is the most common phenomenon in CORP and is associated with poor prognosis due to diagnosis delay. Imaging investigation may provide useful evidence for early diagnosis. Aggressive treatment is helpful in improving outcome.