Treatment of Relapsing Polychondritis in Myelodysplastic Syndrome or VEXAS Syndrome

When relapsing polychondritis occurs in the context of myelodysplastic syndrome (MDS) or VEXAS syndrome, it presents a clinically distinct situation that requires a substantially different management approach from standard relapsing polychondritis.

This scenario — relapsing polychondritis associated with myelodysplastic syndrome or VEXAS syndrome — requires specialised management in close collaboration with a team experienced in myelodysplastic syndrome. The haematological background directly shapes which therapeutic options are feasible.

Therapeutic Direction

In this setting, targeted therapies form part of the treatment approach. The complete protocol — including selection, sequencing, and the specific agents considered — is available via the link below.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1016/j.revmed.2023.05.005

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.

As corticosteroid dependence is common and immunosuppressants are poorly tolerated, targeted therapies are often used with very inconsistent efficacy.

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