An uncommon association of dry eye syndrome with relapsing polychondritis and necrotizing scleritis

Introduction Relapsing polychondritis (RP) is a rare disease evolving with recurrent cartilage inflammation, but also with ocular, respiratory, cardiac and vascular involvement. Associations with various autoimmune disorders and with hematological diseases, mainly leukemia, lymphoma and myelodysplastic syndrome and rarely with hemolytic anemia, have been described. We report a 63-year patient with thyroiditis and pernicious anemia in whom a left eye necrotizing scleritis led to the diagnosis of RP and common variable immune deficiency (CVID). The necrotizing scleritis was successfully operated with scleral graft. However, the disease control was difficult to be achieved with glucocorticoids and various immune suppression regimens tried (including cyclophosphamide, cyclosporine, azathioprine, leflunomide and infliximab) along with immunoglobulin substitution. The association of RP and CVID or CVID-like diseases is rare, another 4 cases having been reported. We review the literature and discuss the diagnostic and management difficulties. A multidisciplinary team approach is necessary in this setting.