Lymphadenopathy associated with IgG4-related disease: Diagnosis & differential diagnosis

Semin Diagn Pathol. 2018 Jan;35(1):61-66. doi: 10.1053/j.semdp.2017.11.006. Epub 2017 Nov 11.


IgG4-related sclerosing disease, which now encompasses diverse organ-related disorders with various prior eponymic designations, may also present with solitary or multifocal lymph node enlargement. This review considers the histopathologic features of IgG4 lymphadenopathy (IgG4LAD), which has been subdivided by Cheuk & Chan into 5 microscopic subtypes. Those include variants that are typified by multicentric Castleman disease (MCD)-like changes, follicular hyperplasia, interfollicular lymphoplasmacytic proliferation, progressive transformation of germinal centers, and formation of inflammatory pseudotumor (IPT)-like lesions. All of them demonstrate an excess of IgG4-immunoreactive plasma cells in the inflammatory cell population. Differential diagnostic considerations for IgG4LAD include true MCD, true IPT, luetic lymphadenitis, Rosai-Dorfman disease, and inflammatory myofibroblastic tumor, among others. An interpretative distinction between malignant lymphoma and IgG4LAD is also crucial.

Keywords: Autoimmune disorders; Benign lymphadenopathies; Castleman disease; IgG4-related sclerosing disease; Inflammatory pseudotumor; Progressive transformation of germinal centers.

Publication types

  • Review

MeSH terms

  • Autoimmune Diseases / pathology*
  • Humans
  • Immunoglobulin G*
  • Lymphadenopathy / pathology*


  • Immunoglobulin G