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IgG4-related systemic disease is a rare autoimmune disorder that has been increasingly recognized and seen in many organ systems. It is characterized by elevated serum IgG4 and diffuse lymphoplasmacytic infiltrates, which may be multisystem and life threatening. Hypophysitis is an inflammatory disorder involving the pituitary gland, and may or may not be accompanied by serological alterations of IgG4. This disease remains under-recognized in the clinical setting. We report the case of a 42-year-old woman with diabetes and autoimmune thyroid disease who presented with progressive visual field defects. Imaging studies revealed swelling of the optic chiasm; she was treated with corticosteroids and followed up. Eight years later she developed visual loss in all 4 quadrants of her vision with thickening of the optic nerve on imaging. She was found to have an IgG4-related systemic disorder and received treatment with corticosteroids. She showed dramatic visual improvement and has not been treated with corticosteroids since. We present the case of a patient with autoimmune hypophysitis who developed symptoms of pituitary mass and systemic disorder. Diagnosis was reached by clinical and laboratory studies, and was confirmed with imaging. Extensive workup including endocrinology, infectious, and imaging studies were performed to exclude other causes of pituitary dysfunction. IgG4-related disease was successfully treated with high-dose corticosteroids. Given the rare presentation of autoimmune hypophysitis, clinicians should be aware that IgG4-related hypophysitis may present with systemic symptoms and pituitary mass, thus mimicking the presentation of other pituitary masses, such as pituitary adenomas.—
title: “search::instance::sourceRecords (Method)”
ms.date: “03/30/2017”
ms.assetid: 90f61935-0f2a-4d97-b731-c45dd9afa31f

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