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

Abstract

INTRODUCTIONRelapsing polychondritis (RP) is a rare immune-mediated condition, which is characterised by recurrent and progressive inflammation of cartilaginous structures and proteoglycan-rich structures.(1) RP particularly affects the cartilaginous areas of the ears and nose, and it is not uncommon for the eyes, joints and respiratory tract to be affected as well. RP can occur in isolation or in association with other diseases. The objective of the present study was to describe the clinical presentation, treatment and outcome of patients with RP who were seen at a large tertiarycare academic medical institution in Singapore. METHODSSingapore General Hospital is a 1,700-bed tertiary-care academic medical institution in Singapore. In this study, we retrospectively reviewed the medical records of all patients diagnosed with RP at the Department of Rheumatology and Immunology between January 2005 and December 2013. The medical records that were retrieved and reviewed included information on both inpatient and outpatient visits.The diagnosis of RP was made according to the modified McAdam criteria. The clinical presentation, laboratory results, treatment response and clinical outcome of the patients were recorded using a standardised data collection sheet; the collected data was then summarised. This study design received approval from the local institutional review board. RESULTSA total of ten patients with RP symptoms were seen at the Department of Rheumatology and Immunology, Singapore General Hospital, Singapore, during the study period. Five of these patients fulfilled the modified McAdam criteria, while the remaining five patients fulfilled only part of the diagnostic criteria and were thus classified as probable cases of RP. The average age of onset of RP was 48 (range 22-72) years and the average duration of follow-up was 3.5 years (range 17 months-7 years). The patients were all of Chinese ethnicity and the female-to-male ratio was 7:3. The clinical presentation, laboratory results, and therapy and clinical outcomes of the ten patients are summarised in Table I.

Relapsing polychondritis – analysis of symptoms and criteria

“…Elevated ESR and CRP may be detected during the acute onset of RP, but more than 10% of patients have a normal CRP level even during an RP flare [ 2 ]. In contrast to results from Chuah et al [ 28 ], who observed elevated ESR in 80% and CRP in 50% of their study, only 10% of cases from our study presented significantly increased levels of inflammatory markers.…”

Case Report: Coexistence of Multiple Myeloma and Auricular Chondritis in VEXAS Syndrome

“…Previous reports have suggested that elderly patients with RP are at risk of MDS or hematological malignancy ( 12 ). Additionally, previous studies demonstrated the co-occurrence of RP or VEXAS syndrome and B-cell or plasma cell neoplasms, but these patients were not complicated with MDS ( Table 1 ) ( 1 , 5 , 13 – 16 ).…”

Ocular Involvement in Relapsing Polychondritis

“…Scleritis/episcleritis is the most common ocular complication associated with RPC (Table 3) [10,[26][27][28][29][30][31][32][33][34]. Most cases of scleritis occur bilaterally.…”

“…Lichauco et al reported a case of RPC with an orbital mass, and a biopsy of the orbital lesion showed mucosa-associated lymphoid tissue B-cell lymphoma [74]. In addition, a high incidence of hematological malignancies, including leukemia, multiple myeloma, and lymphoma, has been reported in RPC patients [28,[75][76][77]. Therefore, biopsy of the orbital mass should be considered when the effectiveness of corticosteroid treatment is insufficient.…”