[Restatement. Relapsing polychondritis].

Abstract

RELAPSING POLYCHONDRITIS. Relapsing polychondritis (RP) is a systemic disease which diagnosis relies on the existence of typical chondritis present at the beginning of the disease only in 1/3 of cases. Three phenotypes of RP have been described, each one characterized by specific manifestations and the need of a specific therapeutic management and follow-up. Screening for tracheo-bronchial manifestations must be systematic if RP is suspected, as it is responsible for most of the morbi-mortality of the disease. Screening for the presence of UBA1 mutations for VEXAS syndrome (Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) is key in male patients over 50 years-old presenting with macrocytic anemia, especially also in case of dermatologic or pulmonary manifestations or thrombo-embolic complications. Initial screening allow to rule-out the main differential diagnosis (ANCA-associates vasculitis) and to look for associated auto-immune or inflammatory diseases which are present in 30% of cases. Therapeutic management of RP is yet to be codified and depends on the severity of the disease.