Immunoglobulin G4-related disease: An update

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

Research output: Contribution to journalReview article

7 Citations (Scopus)

Abstract

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
Volume33
Issue number2
DOIs
Publication statusPublished - Mar 1 2018

Fingerprint

Immunoglobulins
Immunoglobulin G
Dacryocystitis
Cranial Nerve Diseases
Myositis
Eye Diseases
Immunosuppressive Agents
Plasma Cells
Meningitis
Adrenal Cortex Hormones
Serum
Neoplasms
Hypophysitis

Keywords

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

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Immunoglobulin G4-related disease : An update. / Al-Mujaini, Abdullah; Al-Khabori, Murtadha; Shenoy, Kashinatha; Wali, Upender.

In: Oman Medical Journal, Vol. 33, No. 2, 01.03.2018, p. 97-103.

Research output: Contribution to journalReview article

Al-Mujaini, Abdullah ; Al-Khabori, Murtadha ; Shenoy, Kashinatha ; Wali, Upender. / Immunoglobulin G4-related disease : An update. In: Oman Medical Journal. 2018 ; Vol. 33, No. 2. pp. 97-103.
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