Treatment of Relapsing Polychondritis in Myelodysplastic Syndrome or VEXAS Syndrome
Relapsing polychondritis occurring in the setting of myelodysplastic syndrome (MDS) or VEXAS syndrome represents a clinically distinct and complex presentation that requires a specialised management approach.
Clinical scenario: This protocol applies to patients with relapsing polychondritis who have an underlying myelodysplastic syndrome or VEXAS syndrome. The coexistence of these conditions necessitates multidisciplinary collaboration with a team experienced in myelodysplastic syndrome.
Treatment approach
A structured multidisciplinary approach is required. In selected patients, the treatment strategy may include consideration of
allogeneic stem cell transplantation — the full evaluation criteria and decision pathway are detailed in the complete protocol.
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.
Allogeneic stem cell transplantation should be discussed in a multidisciplinary consultation meeting taking into account age and IPSS-R.
DOI: 10.1016/j.revmed.2023.05.005
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