Khare Shruti, Jagtap Varsha S, Budyal Sweta R, Kasaliwal Rajeev, Kakade Harshal R, Bukan Amol, Sankhe Shilpa, Lila Anurag R, Bandgar Tushar, Menon Padmavathy S, Shah Nalini S
Department of Endocrinology, Seth G S Medical College, KEM Hospital, Parel, Mumbai, 400012, India,
Pituitary. 2015 Feb;18(1):16-22. doi: 10.1007/s11102-013-0550-9.
Autoimmune hypophysitis (AH) is a rare autoimmune inflammatory disorder of pituitary gland.
To analyse clinical, hormonal, radiological features and management outcomes of AH.
Retrospective analysis of patients with primary hypophysitis (where secondary causes of hypophysitis were ruled out) was carried out from 2006 to 2012. AH emerged as the most plausible aetiology and the diagnosis of exclusion.
Twenty-four patients with AH (21 females and 3 males) were evaluated. They presented with symptoms of expanding sellar mass (83.3%), symptoms of anterior pituitary hormone deficiencies (58.3%), and diabetes insipidus (16.7%). The anterior pituitary hormonal axes affected were cortisol (75%), thyroid (58.33%) and gonadotropin (50%). All had sellar mass on magnetic resonance imaging, which was symmetrical (91.7%) and homogenously enhancing (91.7%). Stalk thickening, suprasellar extension, loss of posterior pituitary hyperintensity and parasellar T2 dark sign were seen in 87.5, 87.5, 71.5, and 50% respectively. In addition to hormone replacement, five (20.83%) patients underwent trans-sphenoidal surgery, fifteen (62.5%) were watchfully monitored, while four cases (16.67%) received steroid pulse therapy. On follow up imaging, the sellar mass regressed in all, while, stalk thickening was persistent in 13/19 (68.4%) non-operated patients at median follow up of 1 year. Pituitary hormone axis recovery was seen in 10 (41.67%) and was seen in cortisol 10/18 (55.5%) followed by gonadotropin 5/12 (41.67%) axis.
Characteristic radiology helps in diagnosis of AH even without tissue diagnosis. Non-operative treatment is the preferred treatment modality. Steroid pulse therapy potentially improves pituitary axis recovery.
自身免疫性垂体炎（AH）是一种罕见的垂体自身免疫性炎症性疾病。
分析AH的临床、激素、影像学特征及治疗结果。
对2006年至2012年原发性垂体炎（排除垂体炎的继发性病因）患者进行回顾性分析。AH被认为是最合理的病因，且为排除性诊断。
对24例AH患者（21例女性，3例男性）进行了评估。他们表现出蝶鞍肿块扩大症状（83.3%）、垂体前叶激素缺乏症状（58.3%）和尿崩症（16.7%）。受影响的垂体前叶激素轴为皮质醇（75%）、甲状腺（58.33%）和促性腺激素（50%）。所有患者磁共振成像均显示蝶鞍肿块，肿块对称（91.7%）且均匀强化（91.7%）。分别有87.5%、87.5%、71.5%和50%的患者出现垂体柄增粗、鞍上延伸、垂体后叶高信号消失和鞍旁T2低信号征。除激素替代治疗外，5例（20.83%）患者接受了经蝶窦手术，15例（62.5%）接受密切监测，4例（16.67%）接受类固醇脉冲治疗。随访影像学检查显示，所有患者的蝶鞍肿块均缩小，但在中位随访1年时，19例未手术患者中有13例（68.4%）垂体柄增粗持续存在。10例（41.67%）患者垂体激素轴恢复，其中皮质醇轴恢复10/18（55.5%），其次是促性腺激素轴恢复5/12（41.67%）。
即使没有组织学诊断，特征性影像学检查也有助于AH的诊断。非手术治疗是首选的治疗方式。类固醇脉冲治疗可能会改善垂体轴的恢复。
