Renal monoclonal immunoglobulin deposition disease—mainly proliferative glomerulonephritis with monoclonal IgG deposits (PGNMID)

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  • Komatsuda Atsushi
    Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine

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  • 単クローン性免疫グロブリン関連腎症―特にproliferative glomerulonephritis with monoclonal IgG deposits について

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Abstract

Glomerular diseases associated with monoclonal gammopathy are divided into two subgroups. The first group is characterized by organized deposits, like fibrils (mainly in amyloidosis) or microtubules (cryoglobulinemia and immunotactoid glomerulopathy). The second group represents granular electron dense deposits, and defines entities named monoclonal immunoglobulin deposition disease (MIDD) and proliferative glomerulonephritis with monoclonal IgG deposits (PGNMID). MIDD is characterized by the presence of nodular sclerosing glomerulopathy and other patterns including membranoproliferative glomerulonephritis (MPGN) by light microscopy, monoclonal linear staining along the glomerular basement membranes (GBM) and the tubular basement membranes (TBM) by immunofluorescence, and continuous linear deposits of finely granular electron dense material along the inner aspect of the GBM and along the TBM by electron microscopy. On the other hand, PGNMID is characterized by endocapillary proliferative, membranoproliferative, or membranous features by light microscopy and immune complex type deposits by electron microscopy. The biopsy incidence of PGNMID is about 0.2%. The age was ranged from 20 to 81 years until 2012. Since two pediatric patients with PGNMID have been reported in Japan since 2013, the recognition of PGNMID may be widespread. Recently, cases with PGN with monoclonal other immunoglobulins, including IgA and IgM, have been reported, therefore, we need to reconsider the disease spectrum of PGNMID. It is important for nephrologists to confirm the diagnosis of PGNMID fully using light, immunofluorescence including kappa and lambda light chains, and electron microscopies.

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