Basic Research and Clinical Reports Associated with Low Serum IgG4 Concentrations

Elevated IgG4 concentrations in serum have received a great deal of attention recently, whereas the significance of decreased IgG4 levels was frequently neglected in spite of its close relation with infectious and noninfectious inflammations. In this review, based on the structural and functional characteristics of IgG4, we bring together case reports and research related to low levels of IgG4 and try to scratch the importance of decreased IgG4 concentrations in serum. As with elevated IgG4 levels, low serum IgG4-related diseases can be involved in multiple systems such as infection in the respiratory system, stroke in the circulatory system, and glomerulonephritis in the urinary system. Both genetic and immune dysregulation can contribute to decreased IgG4 levels. In the light of animal experiments, we believe that the mystery of low IgG4 can be revealed as long as enough attention is acquired.