Cervical lymph nodes, thyroiditis and ophthalmopathy: the pleomorphic face of an immunoglobulin g4-related disease

Abstract

We report a case of immunoglobulin G4-related disease (IgG4-RD) presenting with a clinical course suggestive of fibrosing thyroiditis and endocrine ophthalmopathy associated with enlarged cervical lymph nodes. This clinical presentation should prompt a search for associated systemic disorders, including an IgG4-RD. The clue to the exact diagnosis of the underlying disease is histological examination showing an IgG4 plasmatocytic infiltrate in the inflammatory organs involved.

Keywords: Graves’-like orbitopathy; Immunoglobulin G4-related disease; Thyroiditis.

Fig. 1

MRI of the orbit performed in January 2013. Axial T1-weighted postcontrast image showing bilateral proptosis, enlarged connective tissue, orbital fat, and extraocular muscle enlargement. The infraorbital optic nerve is enlarged bilaterally. Inflammatory lesions are also seen in the maxillary sinus and in ethmoidal cells.

Fig. 2

a A high glucose uptake is present in orbital areas. b A high glucose uptake is present in bilateral tonsils and cervical lymphadenopathies. Surprisingly, there is no increased uptake in the thyroid bed.

Fig. 3

IgG4-RD in the tonsils. Immunostaining for IgG4, demonstrating ample numbers of plasma cells that stain for IgG4. Comparison with the total number of plasma cells indicates a high IgG4/IgG ratio. Plasmatocytic anti-IgG4 (50 µm).