Immune-Complex Mediated Mesangial Proliferative Glomerulonephritis with Full-House Pattern Observed During Treatment of Immune Thrombocytopenic Purpura

Abstract

This study reports a rare case of immune-complex mediated mesangial proliferative glomerulonephritis (ICGN) with a full-house pattern in a 56-year-old Japanese man, observed during the treatment of immune thrombocytopenic purpura (ITP). Because of persistent complement deficiency and worsening of kidney function, he was treated with prednisolone, and his urinary findings improved markedly. However, as the complement titers were still low, mycophenolate mofetil was also prescribed, which normalized complement levels. Production of anti-platelet antibodies is considered to be involved in the etiology of ITP. Although little is known about the mechanism by which ITP causes glomerulonephritis, including ICGN, glomerular deposition of circulating immune complexes synthesized by antiplatelet antibodies may be involved. This case shows full-house nephropathy, suggesting the involvement of immune complexes, which in turn, suggested an association between ITP and glomerulonephritis.

Keywords: Immune-complex mediated mesangial proliferative glomerulonephritis; full-house nephropathy; immune thrombocytopenic purpura; membranoproliferative glomerulonephritis; systemic lupus erythematosus.

Figure 1.

Representative light microscopy and electron microscopy on kidney biopsy specimen: (a) mesangial hypercellularity (arrow) by light microscope (periodic acid-Schiff staining, ×400), (b) total circumferential thickening of glomerular basement membrane (arrow) by light microscope (periodic acid-Schiff staining, ×400), (c) electron-dense deposits (EDD) in the mesangial region (arrow) by electron microscope (×1500), and (d) EDD in the subendothelial region (arrow) by electron microscope (×1000). Granular IgG, IgA, IgM, C3, C4, and C1q deposit in the mesangial region. Fibrinogen is negative.

Figure 2.

Immunohistochemistry of the glomerulus (immunofluorescence staining, ×400). Granular IgG, IgA, IgM, C3, C4, and C1q deposit in the mesangial region. Fibrinogen is negative.

Figure 3.

Clinical course of the patient after the initiation of treatment for membranoproliferative glomerulonephritis. Abbreviations: Cr, creatinine; PSL, prednisolone.