Relapsing polychondritis in Singapore: a case series and review of literature.

INTRODUCTION

This study aimed to describe the clinical presentation, treatment and outcome of patients with relapsing polychondritis (RP) who were seen at a large tertiary-care academic medical institution in Singapore.

METHODS

The medical records of all patients diagnosed with RP at the Department of Rheumatology and Immunology, Singapore General Hospital, Singapore, between 2005 and 2013 were reviewed. The diagnosis of RP was made using the modified McAdam criteria.

RESULTS

Ten patients were diagnosed with RP during the study period. Among these patients, five fulfilled the modified McAdam criteria and five were probable cases of RP. The most common clinical presentations were auricular chondritis (n = 9), episcleritis or scleritis (n = 5), and large airway involvement (n = 3). All of the patients received prednisolone. Five patients developed haematological disorders. Patients with both RP and haematological disorders had a longer duration of RP symptoms prior to diagnosis, compared to patients with RP who did not develop haematological disorders (average duration of symptoms 14.7 months vs. 4.2 months).

CONCLUSION

The high frequency of patients with haematological malignancies in this series was unexpected. Myelodysplastic syndrome has been reported, but other haematological malignancies are rarely associated with RP. As the association between haematological malignancies and RP is currently unclear, the threshold for haematological/lymphoproliferative screening should be lowered in patients with RP.