Immunoglobulin G4-Related Disease: An Update
Journal Title: Oman Medical Journal - Year 2018, Vol 33, Issue 2
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.
Authors and Affiliations
Abdullah Al-Mujaini, Murtadha Al-Khabori, Kashinatha Shenoy, Upender Wali
Keywords
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- EP ID EP278017
- DOI 10.5001/omj.2018.20
- Views 102
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