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Review
. 2018 May-Jun;115(3):253-256.

IgG-4 Related Disease: An Introduction

Orwah M Al-Khalili  1 , Alan R Erickson  2
Affiliations
Review

IgG-4 Related Disease: An Introduction

Orwah M Al-Khalili et al. Mo Med. 2018 May-Jun.

Abstract

IgG4-related disease (IgG-RD) describes a group of fibroinflammatory diseases that affect a variety of tissues resulting in tumor-like effect and/or organ dysfunction. Clinical presentation varies according to the tissue(s) involved, and diagnosis relies on tissue findings of dense infiltration of IgG4-positive plasma cells and a characteristic storiform fibrosis. Treatment is mainly with glucocorticoids, while multiple immunosuppressive medications can be used as adjuvant agents. Rituximab has showed promising results, but further studies are needed.

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Figures

Figure (1)
Figure (1)
Suggested flow chart for IgG4-RD diagnostic work up. HPF: high-power field. FNA: Fine needle aspiration.
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