Successful treatment with bortezomib in type-1 cryoglobulinemic vasculitis patient after rituximab failure: a case report and literature review.

Type-1 cryoglobulinemic vasculitis (CV) and mixed CV differ in their pathophysiology, clinical expression and treatment response. We report one patient with type-1 cryoglobulinemic vasculitis and skin ulcers that had remained active despite treatment with a variety of immunomodulating drugs including rituximab. The patient had a past medical history of non-Hodgkin lymphoma 10 years after CV onset for which she went into complete remission. The patient developed subsequent hematological anomalies in the serum and in the bone marrow without a definite diagnosis of myeloma. The patient finally went into clinical remission of her cryoglobulinemic vasculitis after treatment with bortezomib and dexamethasone. But she did not achieve an immunological remission and still had positive cryoglobulinemia and serum kappa-type free light chains. This suggests that bortezomib, a proteasome inhibitor that inhibits angiogenesis and production of paraproteins, is a promising treatment in type-1 cryoglobulinemic vasculitis.