Background Relapsing polychondritis (RP) is a rare immune-mediated condition resulting in recurrent, progressive inflammation of cartilaginous and proteoglycan-rich structures. Haematological disorders were observed in several patients with RP.
Objectives To determine the clinical presentation and the frequency of haematological disorder in our RP cohort and to determine if there are any predictive factors for this complication.
Methods The medical records of all patients diagnosed with RP by the Department of Rheumatology and Immunology, Singapore General Hospital between 2003-2013 were reviewed. The diagnosis of RP was made using the modified McAdam criteria.
Results Overall, there were ten patients diagnosed with RP, of whom, five fulfilled the modified McAdam criteria and five were probable cases. All patients were ethnic Chinese with a male:female ratio of 3:7. The median age at presentation was 46.5 year-old (range: 22-72 years), and the average duration of follow up was 3.5 years (range: 6 months-7 years). The commonest clinical presentations were: auricular chondritis (n=9), episcleritis or scleritis (n=5), large airway involvement (n=3). All but one patient received prednisolone and second-line agents. Five patients developed haematologic disorders, including myelodysplastic syndrome (n=2, diagnosed 10 and 60 months after RP, respectively), monoclonal gammopathy of unknown significance (n=1, diagnosed 12 years after RP), and chronic myelomonocytic leukemia (n=1, diagnosed 30 months after RP). All three male patients, as opposed to one in seven female patients, developed haematological disorder after diagnosis of RP. One patient had a history of diffuse large B-cell lymphoma diagnosed 11 years prior to RP.
Conclusions The high frequency of patients with haematologic malignancies in this series is unexpected. Myelodysplastic syndrome has been reported in up to 27% of patients with RP . Other haematological malignancies associated with RP are rare. A MEDLINE review of literature revealed only six other cases of RP associated with non-myelodysplastic haematological malignancies: Hodgkin's lymphoma (n=2), non-Hodgkin's lymphoma (n=2), chronic lymphocytic leukaemia (n=1). The correlation between haematologic malignancies and RP is currently unclear. Threshold for haematological/lymphoproliferative screening should be lower in patients with RP. Even though our numbers are too small to make any clinically meaningful statistical analysis, the apparent higher ESR and CRP levels in RP patients with haematological malignancies in this series provides an early indication of its potential use to identify at risk patients. Larger studies are required to confirm this observation.
Hebbar M, Brouillard M, Wattel E, et al. Association of myelodysplastic syndrome and relapsing polychondritis: further evidence. Leukemia 1995;9:731-3.
Disclosure of Interest : None declared