Systemic vasculitis describes a collection of rare diseases each caused by inflammation of blood vessel walls that can cause severe systemic complications [[3, 4]](#ref-0003). The pattern of vascular inflammation and resultant damage is frequently variable and may be mimicked, or directly triggered, by infection or malignancy. Myeloid and lymphoid hematologic malignancy have long been recognized in association with vasculitis and were recognized by the 2012 Consensus Conference on the Nomenclature of Systemic Vasculitis as vasculitis associated with probable etiology (cancer-associated) [[4]](#ref-0004). We will refer to this as vasculitis with concomitant hematological malignancy (VCHM). This is a rapidly evolving field with limited but growing evidence base. Due to increased use of molecular diagnostics in hematologic clinical practice, rheumatologists should be aware of the diagnostic and treatment complexity in rare patients with VCHM, who often have refractory disease and are at risk of comorbidity from both hematologic and inflammatory disease and their treatments. Here, we discuss our collaborative approach to diagnosis and management of VCHM shared between rheumatology and hematology/oncology, in the context of relatively limited literature. We focus on myeloid neoplasia, as a clinically relevant model of the emerging recognition of overlap between our fields, with more limited discussion of associations with lymphoid forms of VCHM such as cryoglobulinemic vasculitis that have been previously discussed in the literature.
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