Clinical and prognostic characteristics of 158 cases of relapsing polychondritis in China and review of the literature

This work is aimed to study the clinical and prognostic features of relapsing polychondritis (RP) in China. A total of 158 RP cases from 1985 to 2013 in China were included and compared with international case series in terms of clinical features, systemic involvement, differential diagnosis and prognosis. (1) The average age at the onset was 45.3 years old, the average age for initial symptoms was 14.4 months, female/male ratio was 0.7:1 and misdiagnosis rate was 47 %. (2) The incidence of arthritis was lower than that in Caucasians. The incidences of auricular chondritis (68 %: 84–95 %), ocular inflammation (44 %: 49–65 %) and renal involvement (3 %: 7–26 %) were lower, and laryngotracheal symptoms (69 %: 31–67 %), skin (46 %: 4–38 %) and neurological involvement (12 %: 2–8 %) were higher during the follow-up period. The proportion of associated autoimmune disease and systemic vasculitis were 5 and 3 %, respectively, similar to that in Japanese (4 and 2 %), but less than that in Caucasians (12–31 and 8–18 %) except the Francès’s study (7 and 3 %). The primary death cause is respiratory failure due to RP, followed by lung infections and cardiovascular events. (3) Juvenile RP (onset ≤18 years) was more severe than adults, similar to results from the Caucasians. However, Chinese juvenile RP had more severe ocular inflammation (57 %: 40–47 %), arthritis (100 %: 71–90 %), cardiovascular (14 %: 3–10 %) and skin involvement (20 %: 10–11 %) than Caucasian juvenile RP. Although sharing most of the clinical features with case series in previous literature, Chinese patients with RP have its unique characteristics.