Renal-limited Cryoglobulinemic Vasculitis: Two Case Reports

Intern Med. 2018;57(13):1879-1886. doi: 10.2169/internalmedicine.0131-17. Epub 2018 Jul 1.

Abstract

Cryoglobulinemic vasculitis (CV) presents with systemic manifestations, including renal disease, arthritis, peripheral neuropathy, and muscle weakness. We encountered two patients who developed severe nephrotic range proteinuria; however, extrarenal manifestations were not noted during the clinical course. A renal biopsy revealed typical membranoproliferative glomerulonephritis (MPGN) with huge thrombus-like endothelial deposits and predominant IgM positivity, but electron microscopy did not reveal any definite microtubules. Immunosuppressive therapy and plasmapheresis were only partially effective, and the improvement was not durable. Biological therapy with rituximab (RTX) had no effect. Renal-limited CV should be recognized as a subset of essential CV.

Keywords: cryoglobulinemic vasculitis; nephrotic syndrome.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Asian Continental Ancestry Group
  • Cryoglobulinemia / etiology*
  • Cryoglobulinemia / physiopathology
  • Cryoglobulinemia / therapy*
  • Glomerulonephritis, Membranoproliferative / complications*
  • Glomerulonephritis, Membranoproliferative / physiopathology
  • Humans
  • Immunosuppressive Agents / therapeutic use*
  • Male
  • Middle Aged
  • Rituximab / therapeutic use*
  • Treatment Outcome
  • Vasculitis / etiology*
  • Vasculitis / physiopathology*

Substances

  • Immunosuppressive Agents
  • Rituximab