Ma Wanlu, Wang Xi, Nie Min, Fu Junling, Mao Jiangfeng, Wu Xueyan
Department of Endocrinology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China.
Department of Endocrinology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, NO.1 Shuaifuyuan, Dongcheng District, Beijing 100730, China.
Ther Adv Endocrinol Metab. 2020 Jun 27;11:2042018820924556. doi: 10.1177/2042018820924556. eCollection 2020.
Immunoglobulin G4-related hypophysitis (IgG4-RH) is a rare disease, diagnosis of which typically depends on histopathology following an invasive pituitary biopsy, possibly leading to permanent hypopituitarism. Herein, we report two cases of IgG4-RH with favorable responses to glucocorticoids. One of them was multiple organs involved and treated with glucocorticoids and methotrexate.
We retrospectively review clinical features, radiological images, and treatment of two cases with IgG4-RH. In addition, literature on IgG4-RH was comprehensively reviewed and a new therapeutic strategy for IgG4-RH was provided.
A 45-year-old man presented with diabetes insipidus for 6 months. Pituitary magnetic resonance imaging (MRI) indicated thickening of pituitary stalk. His serum IgG4 was 13,500 mg/l and hormonal evaluation revealed isolated growth hormone deficiency. Pituitary biopsy was denied by the patient due to fears of permanent pituitary damage. Treatment with prednisone and methotrexate (MTX) for 1 week led to improvement in sellar images and reduction in IgG4 level. His IGF1 (insulin-like growth factor-1) recovered after a 4-month treatment. The second case is a 43-year-old woman presenting with diabetes insipidus and amenorrhea for 20 months. Her pituitary MRI was similar to the patient above. Her serum IgG4 level was 5980 mg/l and hormonal measurement confirmed isolated hypogonadotropic hypogonadism. After 2 weeks of prednisone, the sellar images improved. After 3 months of treatment, her pituitary MRI was normal, IgG4 level had decreased to near normal range, and menstruation resumed. Literature review found additional patients with IgG4-RH, who were treated successfully without invasive pituitary biopsy in a manner similar to our cases. Therefore, we discuss the necessity of invasive pituitary biopsy for IgG4-RH.
For suspected IgG4-RH with pituitary hormone deficiency, biopsy-induced hypopituitarism may be avoided by using diagnostic glucocorticoid treatment. Impaired pituitary hormone secretion may be recovered in response to steroid therapy. Improved pituitary MRI after 1-2 weeks of glucocorticoid treatment may provide diagnostic evidence of IgG4-RH.
免疫球蛋白G4相关性垂体炎（IgG4-RH）是一种罕见疾病，其诊断通常依赖于侵袭性垂体活检后的组织病理学检查，这可能导致永久性垂体功能减退。在此，我们报告两例对糖皮质激素反应良好的IgG4-RH病例。其中一例累及多个器官，接受了糖皮质激素和甲氨蝶呤治疗。
我们回顾性分析了两例IgG4-RH患者的临床特征、影像学表现及治疗情况。此外，全面复习了关于IgG4-RH的文献，并提出了一种新的IgG4-RH治疗策略。
一名45岁男性出现尿崩症6个月。垂体磁共振成像（MRI）显示垂体柄增粗。其血清IgG4为13500mg/l，激素评估显示孤立性生长激素缺乏。患者因担心永久性垂体损伤而拒绝垂体活检。泼尼松和甲氨蝶呤（MTX）治疗1周后，蝶鞍影像学表现改善，IgG4水平降低。治疗4个月后，其胰岛素样生长因子1（IGF1）恢复正常。第二例是一名43岁女性，出现尿崩症和闭经20个月。她的垂体MRI表现与上述患者相似。其血清IgG4水平为5980mg/l，激素检测证实为孤立性低促性腺激素性性腺功能减退。泼尼松治疗2周后，蝶鞍影像学表现改善。治疗3个月后，她的垂体MRI恢复正常，IgG4水平降至接近正常范围，月经恢复。文献复习发现其他IgG4-RH患者，他们以与我们病例相似的方式在未进行侵袭性垂体活检的情况下成功接受了治疗。因此，我们讨论了IgG4-RH进行侵袭性垂体活检的必要性。
对于疑似IgG4-RH且存在垂体激素缺乏的患者，使用诊断性糖皮质激素治疗可避免活检引起的垂体功能减退。垂体激素分泌受损可能会对类固醇治疗产生反应而恢复。糖皮质激素治疗1-2周后垂体MRI改善可能为IgG4-RH提供诊断依据。
