IgG4相关性腹膜后纤维化和结节漏斗部垂体炎病例中梗阻后利尿与隐匿性尿崩症的重叠：一例报告及文献复习

Sasaki Yatabe Midori, Watanabe Kimio, Hayashi Yoshimitsu, Yatabe Junichi, Morimoto Satoshi, Ichihara Atsuhiro, Nakayama Masaaki, Watanabe Tsuyoshi

Department of Nephrology, Hypertension, Diabetology, Crinology and Metabolism, Fukushima Medical University School of Medicine, Japan.

Intern Med. 2017;56(1):47-53. doi: 10.2169/internalmedicine.56.6648. Epub 2017 Jan 1.

The clinical picture of IgG4-related disease (IgG4-RD) is diverse because various organs can be affected. We describe the case of a 56-year-old man with acute renal failure and tuberoinfundibular hypophysitis due to IgG4-RD. Steroid therapy lowered the serum IgG4 level and ameliorated renal dysfunction, bilateral hydronephrosis and retroperitoneal fibrosis. However, polyuria from post-obstructive diuresis and unmasked central diabetes insipidus ensued. The patient's polyuria continued despite the administration of a therapeutic dose of glucocorticoid; the patient's pituitary swelling and anterior pituitary dysfunction were partially ameliorated. The pituitary swelling recurred seven months later. In patients with IgG4-RD, the manifestation of polyuria after steroid therapy should prompt suspicion of post-obstructive diuresis and the unmasking of central diabetes insipidus.

IgG4相关疾病（IgG4-RD）的临床表现多样，因为各个器官均可受累。我们描述了一例56岁男性因IgG4-RD导致急性肾衰竭和结节漏斗部垂体炎的病例。类固醇治疗降低了血清IgG4水平，并改善了肾功能不全、双侧肾积水和腹膜后纤维化。然而，随后出现了梗阻后利尿引起的多尿以及隐匿性中枢性尿崩症。尽管给予了治疗剂量的糖皮质激素，患者的多尿仍持续存在；患者的垂体肿大和垂体前叶功能障碍得到部分改善。七个月后垂体肿大复发。在IgG4-RD患者中，类固醇治疗后出现多尿应警惕梗阻后利尿和隐匿性中枢性尿崩症的发生。