Korean J Radiol.  2016 Feb;17(1):147-150. 10.3348/kjr.2016.17.1.147.

Immunoglobulin G4-Related Sclerosing Disease Manifesting as Bilateral Tonsillar Hypertrophy on MR Images: A Case Report

• Park MH
• Woo JY
• Lee Y
• Yoon DY
• Hong HS
• Hong ME

• KMID: 2351174
• DOI: http://doi.org/10.3348/kjr.2016.17.1.147

Abstract

Immunoglobulin G4-related sclerosing disease (IgG4-SD) is currently recognized as a distinct systemic disease involving various organs. We reported the imaging findings of a case of pathologically confirmed IgG4-SD involving bilateral palatine tonsils. CT and MRI showed diffuse enlargement of both palatine tonsils with homogeneous contrast enhancement. Focal contour bulging was noted in the right palatine tonsil. Lesions appeared as isointense on T1-weighted and slightly hyperintense on T2-weighted MRI images, as compared with muscle. The T2-weighted MRI image showed a striated pattern in both tonsils. Despite its rare occurrence, IgG4-SD should be included in the differential diagnoses of patients with symptomatic bilateral tonsillar hypertrophy that is non-responsive to medication.

Keyword

IgG4-related sclerosing disease; Palatine tonsil; MRI

MeSH Terms

Diagnosis, Differential
Female
Humans
Hypertrophy/pathology
Immunoglobulin G/*immunology
Magnetic Resonance Imaging/methods
Middle Aged
Palatine Tonsil/*pathology
Retrospective Studies
Sclerosis/diagnosis/*pathology
Immunoglobulin G

Figure

• 

  Fig. 1 MRI and histologic findings of 59-year-old woman with immunoglobulin G4 (IgG4)-related sclerosing disease. A, B. Coronal T1-weighted (A) and T2-weighted (B) MR images demonstrate diffuse enlargement of both palatine tonsils (arrows). Lesion appears isointense on T1-weighted- and slightly hyperintense on T2-weighted-images, as compared with muscle. Note focal asymmetric bulging in right palatine tonsil (arrowheads). C. Axial fat-saturated T2-weighted image reveals striated pattern in bilateral tonsils (arrows). D-G. Microscopic examinations show dense lymphoplasmacytic infiltrate (D, hematoxylin and eosin [H&E], × 40) and storiform fibrosis with lymphoplasmacytic inflammatory infiltration (E, H&E, × 400). Immunochemical studies demonstrate many IgG4-positive plasma cells (> 50/high power field) (F). Ratio of IgG4- (F, × 200) to total IgG- (G, × 200) expressing cells is > 40%.

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