Verbeke Natacha, Pirson Nathalie, Devresse Arnaud, Furnica Raluca, Duprez Thierry, Maiter Dominique
Department of Endocrinology and Nutrition, Cliniques Universitaires Saint Luc, UCL, Avenue Hippocrate 10, 1200, Woluwé-Saint-Lambert, Belgium.
Department of Nephrology, Cliniques Universitaires Saint Luc, UCL, Avenue Hippocrate 10, 1200, Woluwé-Saint-Lambert, Belgium.
J Med Case Rep. 2018 Jun 22;12(1):174. doi: 10.1186/s13256-018-1719-7.
Amyloid infiltration of endocrine glands has been reported, mostly in the thyroid, pancreas, adrenals, and testes, but affected patients do not frequently exhibit overt endocrine insufficiency. Here we report the case of a patient with complete anterior hypopituitarism probably due to a known systemic amyloidosis.
Our male Caucasian patient was diagnosed with Crohn's disease at the age of 22 years. At the age of 37, he developed secondary renal amyloidosis, which resulted in end-stage renal failure. He received a living-donor kidney transplant at the age of 57, without initial complication. Two months later, he developed extreme fatigue, weight loss, and dyspnea. A hormonal evaluation demonstrated complete anterior pituitary insufficiency. A pituitary magnetic resonance imaging was performed and showed a diffusely hypointense anterior gland on both T1-weighted and T2-weighted images with reduced gadolinium enhancement, highly suggestive of amyloid infiltration of the pituitary. Treatment was initiated with levothyroxine, orally administered hydrocortisone, and testosterone enanthate, rapidly allowing progressive marked clinical improvement and nearly complete resolution of symptoms.
Pituitary amyloid infiltration should be considered in patients with a known systemic amyloidosis who develop symptoms of hypopituitarism and magnetic resonance imaging features compatible with protein deposits.
内分泌腺的淀粉样变浸润已有报道，主要累及甲状腺、胰腺、肾上腺和睾丸，但受累患者并不常出现明显的内分泌功能不全。在此，我们报告一例可能因已知的系统性淀粉样变导致完全性垂体前叶功能减退的患者病例。
我们的白人男性患者22岁时被诊断为克罗恩病。37岁时，他发展为继发性肾淀粉样变，导致终末期肾衰竭。57岁时，他接受了活体供肾移植，初期无并发症。两个月后，他出现极度疲劳、体重减轻和呼吸困难。激素评估显示完全性垂体前叶功能减退。进行了垂体磁共振成像检查，在T1加权和T2加权图像上均显示垂体前叶弥漫性低信号，钆增强减弱，高度提示垂体淀粉样变浸润。开始使用左甲状腺素、口服氢化可的松和庚酸睾酮进行治疗，症状迅速得到明显改善，几乎完全缓解。
对于已知患有系统性淀粉样变且出现垂体功能减退症状以及磁共振成像特征与蛋白质沉积相符的患者，应考虑垂体淀粉样变浸润。
