IgG4-related Disease of the Head and Neck: CT and MR Imaging Manifestations

Abstract

Immunoglobulin G4 (IgG4)-related disease is a recently established systemic disease that commonly involves the head and neck, including the salivary glands, lacrimal glands, orbits, thyroid gland, lymph nodes, sinonasal cavities, pituitary gland, and larynx. Although the definitive diagnosis of IgG4-related disease requires histopathologic analysis, elevated serum IgG4 levels are helpful in making the diagnosis. Because of the proposed clinical diagnostic criteria for this disease, cross-sectional imaging modalities such as computed tomography (CT) and magnetic resonance (MR) imaging play an important diagnostic role. CT and MR imaging findings of IgG4-related disease are usually nonspecific. At CT, involved organs may demonstrate enlargement or decreased attenuation; at T2-weighted MR imaging, they may have relatively low signal intensity owing to their increased cellularity and amount of fibrosis. Some pathologic entities involving the head and neck are now considered to be part of the IgG4-related disease spectrum, including idiopathic orbital inflammatory syndrome (inflammatory pseudotumor), orbital lymphoid hyperplasia, Mikulicz disease, Küttner tumor, Hashimoto thyroiditis, Riedel thyroiditis, and pituitary hypophysitis. Because involvement of multiple sites is common in IgG4-related disease, radiologists should be familiar with manifestations of this systemic process outside the head and neck, in organs such as the pancreas, bile ducts, gallbladder, kidneys, retroperitoneum, mesentery, lungs, gastrointestinal tract, and blood vessels. Moreover, IgG4-related disease usually demonstrates a dramatic response to corticosteroid therapy, and radiologists should be familiar with its clinical and imaging manifestations to avoid a delay in diagnosis or unnecessary invasive interventions.