Treatment of Relapsing Polychondritis in Myelodysplastic Syndrome or VEXAS Syndrome

When relapsing polychondritis occurs in a patient with myelodysplastic syndrome (MDS) or VEXAS syndrome, the underlying haematological condition substantially shapes the management strategy. Standard approaches may be insufficient, and specialised guidance is required.

Clinical Scenario

This protocol applies to relapsing polychondritis arising in the setting of myelodysplastic syndrome or VEXAS syndrome. These associations call for specialised management developed in collaboration with a team experienced in myelodysplastic syndrome.

Treatment Approach

In difficult-to-control cases within this population, a hypomethylating agent is among the therapeutic options to consider for disease control. The complete protocol — including the full treatment algorithm, sequencing, and clinical criteria — is available below.

Full regimen details available in the structured protocol →

Instant Access to Structured Evidence-Based Regimens

References

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.

Early treatment with a hypomethylating agent (azacitidine) is therefore an option to consider in order to control relapsing polychondritis in difficult cases.

DOI: 10.1016/j.revmed.2023.05.005

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