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

Renal-limited Cryoglobulinemic Vasculitis: Two Case Reports.

Author information

1
Nephrology Center, Toranomon Hospital, Japan.
2
Okinaka Memorial Institute for Medical Research, Japan.
3
Department of Pathology, Toranomon Hospital, Japan.
4
Department of Pathology, Yokohama City Hospital, Japan.
5
Department of Nephrology, Kyoto University Graduate School of Medicine, Japan.

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

PMID:
29962417
PMCID:
PMC6064702
DOI:
10.2169/internalmedicine.0131-17
[Indexed for MEDLINE]
Free PMC Article

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