Myelodysplastic syndrome (MDS) and chronic myelomonocytic leukemia (CMML) are heterogeneous clonal disorders that may be associated with immune-mediated inflammatory disorders (IMIDs), reported in up to 25% of patients.1-4 The contribution of clonal hematopoiesis to systemic inflammation is increasingly recognized, exemplified by the recently described VEXAS syndrome.5 Among recurrent genetic alterations in these myeloid neoplasms (MNs), isocitrate dehydrogenase (IDH1/2) mutations are present in 2%–12% of MDS and 4%–6% of CMML.6, 7 Beyond their leukemogenic role through 2-hydroxyglutarate–mediated epigenetic dysregulation, IDH mutations are enriched in patients with IMIDs (14%–20%), suggesting a contribution to immune dysregulation.8, 9 The clinical impact of IDH inhibition on inflammatory manifestations has never been addressed. In this retrospective multicenter study, we characterized IDH-mutated MN with IMIDs and highlighted the striking efficacy of IDH inhibitors on systemic inflammation.[...]