Internal Medicine
Online ISSN : 1349-7235
Print ISSN : 0918-2918
ISSN-L : 0918-2918
CASE REPORTS
Renal-limited Cryoglobulinemic Vasculitis: Two Case Reports
Naoya Toriu, Naoki Sawa, Masahiko Oguro, Hiroki Mizuno, Yoichi Oshima, Eiko Hasegawa, Keiichi Sumida, Tatsuya Suwabe, Masahiro Kawada, Toshiharu Ueno, Noriko Hayami, Akinari Sekine, Rikako Hiramatsu, Masayuki Yamanouchi, Junichi Hoshino, Kenmei Takaichi, Kenichi Ohashi, Takeshi Fujii, Motoko Yanagita, Yoshifumi Ubara
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Keywords: cryoglobulinemic vasculitis, nephrotic syndrome
JOURNAL OPEN ACCESS

2018 Volume 57 Issue 13 Pages 1879-1886

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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.

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© 2018 by The Japanese Society of Internal Medicine
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