Relapsing polychondritis
ICD-10 M94.1 · ICD-11 FB82.3

Treatment of Relapsing Polychondritis in Myelodysplastic Syndrome or VEXAS Syndrome

This protocol addresses the management of relapsing polychondritis occurring in patients with myelodysplastic syndrome (MDS) or VEXAS syndrome — a setting that requires specialised, multidisciplinary care distinct from standard relapsing polychondritis management.

The initial treatment in this setting involves systemic corticosteroid therapy. The complete regimen — including sequencing, monitoring, and additional considerations specific to MDS or VEXAS — is detailed in the full protocol.

The treatment approach for patients with relapsing polychondritis associated with myelodysplastic syndrome as well as for VEXAS syndrome requires specialised management in collaboration with a team specialised in myelodysplastic syndrome.

Systemic corticosteroid therapy is used as the first line of treatment for the manifestations of relapsing polychondritis in this setting.

DOI: 10.1016/j.revmed.2023.05.005
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