K O Kong1, S Vasoo, N S W T Tay, H H Chng. 1. Department of Rheumatology, Allergy and Immunology, Tan Tock Seng Hospital, 11 Jalan Tan, Tock Seng, Singapore 308433. Kok_Ooi_Kong@ttsh.com.sg
Abstract
INTRODUCTION: Relapsing polychondritis (RPC) has been described mainly in Caucasian populations. Reports from other ethnic groups are few. OBJECTIVES: To describe the clinical characteristics, management and outcome of RPC patients seen in an Oriental population in Singapore. METHODS: The case records of RPC patients treated in our department from 1989 to 2001 were reviewed. Only 12 fulfilled the McAdam-Michet-Damiani-Levine diagnostic criteria and these were studied. RESULTS: The female-to-male ratio in our series was 3:1. There were 10 ethnic Chinese and two Malay patients. The age of onset of symptoms ranges from three to 65 years, with a mean of 34 years. A diagnosis was made from two weeks to three years after onset, with a median of 4.5 months. There were 10 patients with pinna, nine articular, eight ocular, six laryngotracheal, five inner ear, four nasal and one cardiac involvement. Five presented with fever. None of them had cutaneous, renal or central nervous system involvement. Ten had raised ESR at presentation. One patient developed discoid lupus erythematosus two years later. All 12 patients received prednisolone with eight of them requiring additional immunosuppressants. Two patients had resistant disease failing to respond adequately to various immunosuppressants together with prednisolone. There was no mortality amongst the nine patients who had remained on follow-up at the time of this report. Five of the six patients with laryngotracheal involvement had tracheostomy and one of them had airway stenting as well. CONCLUSION: Our series suggests that although the clinical manifestations of RPC are similar in the Oriental and the Caucasian populations, Oriental patients may have less cutaneous, renal or nervous system involvement and more serious airway complications.
INTRODUCTION: Relapsing polychondritis (RPC) has been described mainly in Caucasian populations. Reports from other ethnic groups are few. OBJECTIVES: To describe the clinical characteristics, management and outcome of RPC patients seen in an Oriental population in Singapore. METHODS: The case records of RPC patients treated in our department from 1989 to 2001 were reviewed. Only 12 fulfilled the McAdam-Michet-Damiani-Levine diagnostic criteria and these were studied. RESULTS: The female-to-male ratio in our series was 3:1. There were 10 ethnic Chinese and two Malay patients. The age of onset of symptoms ranges from three to 65 years, with a mean of 34 years. A diagnosis was made from two weeks to three years after onset, with a median of 4.5 months. There were 10 patients with pinna, nine articular, eight ocular, six laryngotracheal, five inner ear, four nasal and one cardiac involvement. Five presented with fever. None of them had cutaneous, renal or central nervous system involvement. Ten had raised ESR at presentation. One patient developed discoid lupus erythematosus two years later. All 12 patients received prednisolone with eight of them requiring additional immunosuppressants. Two patients had resistant disease failing to respond adequately to various immunosuppressants together with prednisolone. There was no mortality amongst the nine patients who had remained on follow-up at the time of this report. Five of the six patients with laryngotracheal involvement had tracheostomy and one of them had airway stenting as well. CONCLUSION: Our series suggests that although the clinical manifestations of RPC are similar in the Oriental and the Caucasian populations, Oriental patients may have less cutaneous, renal or nervous system involvement and more serious airway complications.