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IgG-4 Related Disease: An Introduction
Orwah M Al-Khalili1, Alan R Erickson2
1Orwah M. Al-Khalili, MD, is a Rheumatology Fellow, University of Nebraska Medical Center, Omaha, Neb.
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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