IgG4-related disease: review of the histopathologic features, differential diagnosis, and therapeutic approach

APMIS. 2018 Jun;126(6):459-476. doi: 10.1111/apm.12845.


Immunoglobulin G4-related disease (IgG4-RD) is an uncommon disorder that demonstrates characteristic clinicopathologic features including sclerosing lesions with storiform fibrosis, increased IgG4+ plasma cells with an increased IgG4+/IgG+ plasma cell ratio, obliterative phlebitis, and often an increased serum IgG4 level. This review summarizes the characteristic histopathologic and clinical features of IgG4-RD with detailed discussion of the histopathologic characteristics of the most commonly involved anatomic sites. We also present recent advances in our understanding of the pathophysiologic mechanisms of IgG4-RD and discuss updates on the treatment, prognosis, and outcomes of this rare disease, including discussion of the possible association between IgG4-RD and malignancy.

Keywords: IgG4; IgG4-RD; IgG4-related disease; pathology.

Publication types

  • Review

MeSH terms

  • Autoimmune Diseases / blood*
  • Autoimmune Diseases / diagnosis*
  • Cholangitis, Sclerosing / blood
  • Cholangitis, Sclerosing / diagnosis
  • Dacryocystitis / blood
  • Dacryocystitis / diagnosis
  • Diagnosis, Differential
  • Humans
  • Immunoglobulin G / blood*
  • Kidney Diseases / blood
  • Kidney Diseases / diagnosis
  • Lung Diseases / blood
  • Lung Diseases / diagnosis
  • Lymphadenopathy / blood
  • Lymphadenopathy / diagnosis
  • Pancreatitis / blood
  • Pancreatitis / diagnosis
  • Plasma Cells / cytology*


  • Immunoglobulin G