Case of membranoproliferative glomerulonephritis due to essential cryoglobulinemia without hepatitis C virus infection

Abstract

An 81-year-old man was admitted to our hospital because of leg edema. Serological studies for anti-neutrophil cytoplasmic antibody (ANCA), anti-double stranded DNA antibodies, and antibodies to hepatitis C and B were negative. Severe hypocomplementemia was present and a cryoglobulin was detected with serum immunoelectrophoresis being positive for the immunoglobulin (Ig)M kappa type. The cryoglobulin was characterized by immunoelectrophoresis which showed that the protein was composed of polyclonal IgG and kappa-type monoclonal IgM. A diagnosis of essential type II cryoglobulinemia was made and the patient underwent a renal biopsy. The renal biopsy revealed endocapillary and mesangial cell proliferation with increased matrix. The resultant lobular appearance of the glomerulus and double contours of the basement membrane were indicative of membranoproliferative glomerulonephritis (MPGN). Immunofluorescence studies demonstrated granular staining in the capillary wall for IgG, IgA, IgM and C4 with little C3 deposition but no C1q. He was finally diagnosed with MPGN due to mixed cryoglobulinemia type II. MPGN with essential cryoglobulinemia type II without evidence of hepatitis C virus infection, like that found in the present case, is very rare.

Mixed cryoglobulinemic membranoproliferative glomerulonephritis due to monoclonal gammopathy of undetermined significance

“…Table 2 shows the published cases with cryoglobulinemic MPGN but without HCV infection in the literature, which just 4 cases have been reported. Our case was similar to the one reported by Okura et al, [ 15 ] who both had favorable response after steroid therapy.…”

“…Two patients showed type I cryoglobulin, and 1 patient showed type II cryoglobulin. Moreover, Okura et al [ 15 ] had reported 81-year-old male with mixed cryoglobulinemic MPGN without HCV infection, who led a favorable outcome after a steroid therapy. Table 2 shows the published cases with cryoglobulinemic MPGN but without HCV infection in the literature, which just 4 cases have been reported.…”

Detectable serum IgM monoclonal gammopathy in non-hepatitis-associated mixed cryoglobulinemic glomerulonephritis: A case report and literature review

“…Table 2 summarizes a review of the literature of the noninfectious mixed (type II) cryoglobulinemic glomerulonephritis, in which the association with serum monoclonal gammopathy was determined. 9,[13][14][15][16][17][18] Interestingly, all of the cases of the noninfectious mixed cryoglobulinemia with positive serum monoclonal gammopathy were linked with serum IgM kappa monoclonal antibody. We speculated that the IgM monoclonal gammopathy might have high autoantibody activity that allows for the formation of an immune complex, as also observed in Waldenström macroglobulinemia.…”

An unusual case of cryoglobulinemic purpura in elderly patient

“…Il nostro caso clinico presenta molte analogie con quanto descritto da autori olandesi nel 2007 e da autori giapponesi e francesi *associato a esacerbazione di crioglobulinemia nel 2009: in particolare, un paziente di 81 anni con glomerulonefrite membranoproliferativa [12], sette [13] e dieci casi con altrettanta sindrome nefrosica [14], tutti senza evidenza di HCV. Pur con il limite della mancata tipizzazzione del criocrito, abbiamo formulato l'ipotesi diagnostica di sindrome crioglobulinemica, con associata nefropatia nefrosica, da verosimile crioglobulinemia mista di tipo "essenziale" (EMC).…”