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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.

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