ECE2019 Poster Presentations Pituitary and Neuroendocrinology 1 (72 abstracts)
IgG4-releated hypophysitis: A case report
Fatih Kilicli 1 , Hatice Sebile Dokmetas 1 , Gunes Cavusoglu 2 , Meric Dokmetas 2 , Kubra Karaipek 2 , Bugra Erol 2 & Aysenur Cila 3
1İstanbul Medipol University, Department of Endocrinology, İstanbul, Turkey; 2İstanbul Medipol University, Department of Internal Medicine, İstanbul, Turkey; 3İstanbul Medipol University, Department of Radiology, İstanbul, Turkey.
Introduction: IgG4-dependent disease is a newly defined fibroinflammatory disease. This disease has been shown to affect almost all organs in the body, especially the pancreas, salivary gland, orbital tissue, lymph node, lung and kidney. IgG4 hypophysitis is a rare and inflammatory process that mimics pituitary tumors. The diagnosis of IgG4 releated hypophysitis can be done in many ways. The definitive diagnosis is biopsy; however, it is not necessary in most cases. If the other organs are diagnosed with IgG4-releated disease with biopsy, the appearance of pituitary mass is also diagnostic.
Case: A 28-year-old woman was admitted to our clinic with blurred vision and double vision during her pregnancy. Our patient was receiving replacement therapy due to total hypophyseal insufficiency. In June 2018, pituitary biopsy was performed and the pathology report was revealed as IgG4 pituitary. In November 2018, outward shifting started in the right eye. The patient presented with a Gamma-knife recommendation. TSH: 0.026 (N: 0.27–4.2 IU/ml), freee T4: 0.836 (N: 0.93–1.7 ng/dl), FSH: 1.24 (N: 3.5–12.5 mIU/ml), LH 0.4 (2.5–11.2 IU/ml), E2 (3 pg/ml), Prolactin: 0.466 (N: 4.79–23.3 ng/ml), IgG4: 340 (N: 80–140 mg/dl). Pituitary MRI revealed bilateral cavernous sinus, optic chiasm and infundibulum infiltration, and T2 hypointense hypophysitis. The patient was diagnosed with IgG4-releated hypophysitis and pulse steroid treatment was started, but because there was no clinical and radiological improvement, rituximab1000 mg was given twice daily for 15 days.
Conclusion: There is still no consensus about the treatment of IgG4-releated hypophysitis, and in patients with steroid resistance, rituximab therapy may be required.
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Endocrine Abstracts
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