IgG4-Related Disease: Beyond Glucocorticoids

Mitsuhiro Akiyama, Tsutomu Takeuchi

Research output: Contribution to journalReview articlepeer-review

45 Citations (Scopus)


IgG4-related disease is a heterogeneous immune-mediated fibroinflammatory condition that can affect every single organ. This disease is more prevalent in the elderly (the mean age of patients is above 60 years) and the prevalence rate is estimated to be over 4.6 per 100,000 population. Before making a diagnosis, the exclusion of malignancies, lymphoma, anti-neutrophil cytoplasmic antibody-associated vasculitis, multicentric Castleman disease, and other mimickers is crucial for appropriate treatment. Broad management guidelines have been published emphasizing the need for prompt treatment and the use of glucocorticoids as first-line drug therapy for induction of remission. However, the toxic effects of glucocorticoids are problematic because IgG4-related disease is more prevalent in patients above 60 years of age, a population with frequent comorbid conditions and polypharmacy. Immunosuppressants (cyclophosphamide, methotrexate, leflunomide, and tacrolimus) and targeted immunomodulators (rituximab, XmAb5871, and abatacept) are appealing to overcome potential toxic effects of glucocorticoids and as emerging glucocorticoid-sparing and/or maintenance treatments. In this review, we provide an overview of our understanding of the pathophysiology of the disease (T follicular helper cells, CD4+ cytotoxic T cells, plasmablasts, and alternatively activated M2 macrophages) and clinical characteristics, and highlight the potential targets for treatment intervention.

Original languageEnglish
Pages (from-to)275-287
Number of pages13
JournalDrugs and Aging
Issue number4
Publication statusPublished - 2018 Apr 1

ASJC Scopus subject areas

  • Geriatrics and Gerontology
  • Pharmacology (medical)


Dive into the research topics of 'IgG4-Related Disease: Beyond Glucocorticoids'. Together they form a unique fingerprint.

Cite this