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.

Relapsing polychondritis--case series from South India.

Relapsing polychondritis (RP) is a rare recurring inflammatory disorder with variable clinical course. It has been described mainly in Caucasian population. Reports from other ethnic groups are few. We report seven cases of relapsing polychondritis in south Indian population. In between 1995 and 2008, seven patients fulfilling the McAdam-Damiani-Levine criteria for diagnosis of relapsing polychondritis were identified. Records pertaining to these patients were studied and clinical presentation, course, and treatment offered were analyzed retrospectively. The female-to-male ratio in our series was 2.5:1. The age of onset of symptoms ranged from 28 to 54 years, with a mean of 40.2 years. An average of 20 months, ranging from 3 months to 6 years, elapsed before the patient presented to us seeking a diagnosis. Various structural involvement in our series were as follows: pinna in four (57%), nasal cartilage in five (71%), joints in three (43%), eyes in three (43%), laryngotracheal tree in three (43%), inner ear in one (14.3%), skin in one (14.3%), and heart in one (14.3%). Associated autoimmune diseases were present in four (57%) patients in the form of one of the following in each: vasculitis, autoimmune hemolytic anemia, hypothyroidism, and rheumatoid arthritis. All seven patients received prednisolone with three of them requiring additional immunosuppressants. There was no mortality amongst the four patients who had remained on follow-up at the time of this report. Although RP is an uncommon disorder, clinicians should be aware of the manifestations so as to initiate prompt treatment and prevent complications. Our series reports less frequent auricular cartilage and skin involvement and an exceptional case of basal cell carcinoma, although the other manifestations were similar to that seen in Caucasian and other Asian populations.