Type 1 Cryoglobulinemic Vasculitis Due to Monoclonal Gammopathy of Undetermined Significance Successfully Treated by Bortezomib Plus Dexamethasone

Intern Med. 2024 Feb 15;63(4):541-545. doi: 10.2169/internalmedicine.2053-23. Epub 2023 Jun 14.

Abstract

Cryoglobulins are immunoglobulins that precipitate in cold conditions. Type I cryoglobulinemic vasculitis is associated with hematological malignancies. We herein report a case of steroid-resistant type 1 cryoglobulinemic vasculitis associated with monoclonal gammopathy of undetermined significance (MGUS) in a 47-year-old woman. By immunofixation of cryoglobulin, we found that the main component of cryoglobulin was the M protein due to MGUS, so treatment of MGUS was needed. Bortezomib+dexamethasone therapy resulted in a rapid decrease in cryoglobulin and improvement in the symptoms of cryoglobulinemic vasculitis. In refractory type I cryoglobulinemic vasculitis, treatment of the underlying gammaglobulinopathy should be considered.

Keywords: MGUS; bortezomib; cryoglobulin.

Publication types

  • Case Reports

MeSH terms

  • Bortezomib / therapeutic use
  • Cryoglobulinemia* / complications
  • Cryoglobulinemia* / drug therapy
  • Cryoglobulins
  • Dexamethasone / therapeutic use
  • Female
  • Humans
  • Middle Aged
  • Monoclonal Gammopathy of Undetermined Significance* / complications
  • Monoclonal Gammopathy of Undetermined Significance* / diagnosis
  • Monoclonal Gammopathy of Undetermined Significance* / drug therapy
  • Paraproteinemias* / complications
  • Vasculitis* / complications
  • Vasculitis* / drug therapy

Substances

  • Bortezomib
  • Cryoglobulins
  • Dexamethasone