Background Relapsing polychondritis (RP) is a rare systemic autoimmune disease characterized by recurrent episodes of inflammation and progressive destruction of cartilaginous tissues. Juvenile RP (<18 years) mentioned more severe than adult as previous reports.
Objectives Our work is to study the clinical and prognostic features of Juvenile Relapsing polychondritis (RP).
Methods 7 RP cases from 1994–2013 in our hospital were reviewed and compared with 51 international juvenile cases and 158 Chinese adult case series reported from 1985–2013 including clinical features, systemic involvement, differential diagnosis and prognosis.
Results 1)Average age at the onset was 15.1 years old, the mean age at first symptoms was 15.1 months and female-male ratio was 0.4:1, 6 of 7 had been misdiagnosed. 2)The incidences of arthritis (100%: 53%), nasal chondritis (1%: 53%), laryngotracheal syndromes (86%: 68%), auricular chondritis (86%: 67%), cardiovascular involvement (14%: 10%) and autoimmune diseases (14%: 5%) were higher than in Chinese adult except skin (20%: 46%). 3) Our juvenile RP patients had more severe in respect toocular inflammation (57%: 40–47%), arthritis (100%, 71–90%), cardiovascular (14%: 3–10%) and skin involvement (20%:10–11%) than Caucasian juvenile RP.
Conclusions Our juvenile RP series were more severe than Chinese adult RP as the same as in Caucasians and has its unique characteristics in spite of sharing most clinical features with case series in literature.
Disclosure of Interest None declared
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