Objective: The purposes of this article are to provide a practical review of the spectrum of imaging findings in patients with systemic IgG4-related sclerosing disease and to address the differential diagnoses.
Conclusion: IgG4-related sclerosing disease is a systemic disorder that can involve almost any organ. The imaging findings consist of diffuse and focal organ infiltration and encasement by inflammatory and fibrotic tissue. Awareness of the spectrum of imaging findings in IgG4-related disease should prompt further evaluation for systemic manifestations to avoid misdiagnosis.