Increased Numbers of IgG4-Positive Plasma Cells May Rarely Be Seen in Lymph Nodes of Patients Without IgG4-Related Sclerosing Disease

IgG4-related sclerosing disease (IRSD) is a steroid-responsive fibroinflammatory disorder characterized by increased IgG4+ cells. Nodal involvement usually lacks the dense sclerosis seen in extranodal sites, with histologic patterns overlapping with other reactive processes. Twenty-six lymph nodes showing IRSD-related histologic patterns were evaluated for IgG and IgG4 positive cells by immunohistochemistry and correlated with the clinical features. Cases included 7 Castleman disease-like cases (type I pattern), 10 follicular hyperplasia (type II), and 9 plasmacytosis (type III). The mean numbers of IgG4+ cells per high-power field (HPF) were 4.8 (I), 8.4 (II), and 26.6(III), and the mean IgG4/IgG ratios were 0.05 (I), 0.04 (II), and 0.08 (III). Using > 50 IgG4+cells/HPF and IgG4/IgG ratio of > 0.4 for absolute and relative increases, only 1 case fulfilled both criteria for increased IgG4+ cells, a patient with Hashimoto's thyroiditis without clinical evidence of IRSD. The results suggest that increased IgG4+ cells may rarely be seen in non-IRSD lymph nodes.