Recurrent polychondritis with childhood onset

  Recurrent polychondritis can develop at any age. We have encountered patients in the age range of 6 to 86 years. We have experience with more than 270 patients. Several years ago, we summarized the clinical data of 11 patients with recurrent polychondritis of childhood onset and found that: the proportion of boys and girls with recurrent polychondritis was similar; the average age of onset was about 11 years; the average delay in diagnosis was about 2 years; laryngotracheal involvement was the first symptom in more children (63.6%), followed by rhinitis, ocular involvement and arthritis; 90.9% of patients had laryngeal involvement at the time of diagnosis At diagnosis, 90.9% of patients had laryngeal involvement, 54.5% had tracheotomy, followed by ocular and nasal involvement and saddle nose deformity (45.5%, respectively), followed by external ear, vestibular function and joint involvement (36.4%, respectively); 18.2% had positive serum autoantibodies; 36.4% had increased ESR or CRP; most patients were treated with hormones and immunosuppressive drugs. At 27±15 months of follow-up, 2 cases were discontinued, 2 cases were aggravated, and the rest were stable or improving.  Hint: Recurrent polychondritis with childhood onset is more common with laryngotracheal involvement, easy to be misdiagnosed, and has a poor prognosis; therefore, patients who have been untreated for a long time and for whom no clear cause can be identified, with symptoms of eye, ear, nose, and throat (especially hoarseness, cough, shortness of breath, etc.), should pay attention to this disease; timely laryngeal and tracheal examination can provide an important basis for early diagnosis; active treatment can help improve the prognosis.