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Renal-limited Cryoglobulinemic Vasculitis: Two Case Reports #MMPMID29962417
Toriu N; Sawa N; Oguro M; Mizuno H; Oshima Y; Hasegawa E; Sumida K; Suwabe T; Kawada M; Ueno T; Hayami N; Sekine A; Hiramatsu R; Yamanouchi M; Hoshino J; Takaichi K; Ohashi K; Fujii T; Yanagita M; Ubara Y
Intern Med 2018[Jul]; 57 (13): 1879-86 PMID29962417show ga
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.