Immunoglobulin G4-related disease: An update

Abdullah Al-Mujaini*, Murtadha Al-Khabori, Kashinatha Shenoy, Upender Wali

*Corresponding author for this work

Research output: Contribution to journalReview articlepeer-review

14 Citations (Scopus)


Immunoglobulin G4-related disease (IgG4-RD) is an increasingly recognized immune-mediated condition comprised of a collection of disorders that share specific pathological, serological, and clinical features. IgG4-RD is a fibroinflammatory condition with a tendency to form tumors with inflammatory infiltrate with IgG4 rich plasma cells and elevation of serum IgG4, which may affect virtually every organ and tissue. IgG4-related ophthalmic disease may present as dacryoadenitis, myositis, or involvement of other orbital tissue. Hypophysitis or pachymeningitis may manifest as cranial neuropathies. The diagnosis of IgG4-RD is based on a typical clinical scenario, supportive laboratory test, expected radiological characteristics, and distinct histopathological and immunohistochemical features. Corticosteroids and immunosuppressives form the mainline treatment.

Original languageEnglish
Pages (from-to)97-103
Number of pages7
JournalOman Medical Journal
Issue number2
Publication statusPublished - Mar 2018


  • Granuloma
  • Immunoglobulin G
  • Immunosuppressants
  • Inflammatory pseudotumor
  • Orbital
  • Plasma cells

ASJC Scopus subject areas

  • General Medicine


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