Follow-up with serum IgG4-monitoring in 8 patients with IgG4-related disease diagnosed by a lacrimal gland mass

J Clin Exp Hematop. 2021;61(1):10-21. doi: 10.3960/jslrt.20048.


The diagnostic criteria for IgG4-related disease were previously published and serum IgG4 measurement has been reimbursed by national health insurance in Japan since 2012. Eight patients diagnosed with IgG4-related disease based on lacrimal gland masses were retrospectively reviewed. The 8 patients were 3 men and 5 women ranging in age from 52 to 77 (median, 63) years at the initial visit and their follow-up period ranged from 0.25 to 11 (median, 7) years. Bilateral and unilateral involvement were noted in 4 patients each; 2 on the right side and 2 on the left side in those with unilateral involvement. Serum IgG4 was high in 5 of 8 patients at the initial visit. Five patients with no systemic signs were followed without treatment, whereas oral steroids were administered and tapered in the other 3 patients who exhibited systemic signs. One patient with a history of radiation for MALT lymphoma in bilateral lacrimal glands developed IgG4-related disease in the left lacrimal gland 10 years later and was followed without treatment. Nine years later, her serum IgG4 level increased to 1500 mg/dL and paracardiac lesions, found on positron emission tomography, were confirmed to be MALT lymphoma by needle biopsy, leading to systemic chemotherapy. The other 7 patients had neither local recurrence nor additional systemic signs. Serum IgG4 monitoring may be useful to detect systemic complications in IgG4-related ophthalmic disease and markedly high serum IgG4 levels may indicate new lymphoma at other sites.

Keywords: IgG4-related disease; extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma); lacrimal gland; prednisolone; serum IgG4.

MeSH terms

  • Aged
  • Biomarkers*
  • Biopsy
  • Female
  • Fluorodeoxyglucose F18
  • Humans
  • Immunoglobulin G / blood*
  • Immunoglobulin G4-Related Disease / blood*
  • Immunoglobulin G4-Related Disease / diagnosis*
  • Immunoglobulin G4-Related Disease / etiology
  • Immunoglobulin G4-Related Disease / therapy
  • Immunohistochemistry
  • Lacrimal Apparatus / pathology*
  • Male
  • Middle Aged
  • Positron-Emission Tomography
  • Tomography, X-Ray Computed


  • Biomarkers
  • Immunoglobulin G
  • Fluorodeoxyglucose F18