Angelousi Anna, Cohen Carolina, Sosa Soledad, Danilowicz Karina, Papanastasiou Lina, Tsoli Marina, Pal Aparna, Piaditis Georgios, Grossman Ashley, Kaltsas Gregory
Department of Pathophysiology, Endocrine Oncology Unit, Laiko Hospital, National and Kapodistrian University of Athens, Greece.
Division of Endocrinology, Hospital de Clínicas, University of Buenos Aires, Argentina.
Horm Metab Res. 2018 Apr;50(4):296-302. doi: 10.1055/s-0044-101036. Epub 2018 Feb 19.
Primary hypophysitis (PH) is a rare disease with a poorly-defined natural history. Our aim was to characterise patients with PH at presentation and during prolonged follow-up. Observational retrospective study of 22 patients was conducted from 3 centres. In 14 patients, PH was confirmed histologically and in the remaining 8 clinically, after excluding secondary causes of hypophysitis. All patients had hormonal and imaging investigations before any treatment. Median follow up was 48 months (25-75%: 3-60). There was a female predominance with a female/male ratio: 3.4:1. Eight out of 22 patients had another autoimmune disease. Headaches and gonadal dysfunction were the most common symptoms. Five patients presented with panhypopituitarism; 17 patients had anterior pituitary deficiency, and 7 had diabetes insipidus. At presentation, 9 patients were treated surgically, 5 received replacement hormonal treatment, and 8 high-dose glucocorticoids from whom 5 in association with other immunosuppressive agents. Six patients showed complete recovery of pituitary hormonal deficiencies while 6 showed a partial recovery during a 5-year follow-up period. No difference was found between patients treated with surgery and those treated medically. The overall relapse rate was 18%. PH can be manifested with a broad spectrum of clinical and hormonal disturbances. Long-term follow-up is required to define the natural history of the disease and response to treatment, since pituitary hormonal recovery or relapse may appear many years after initial diagnosis. We suggest that surgery and immunosuppressive therapy be reserved for exceptional cases.
原发性垂体炎（PH）是一种罕见疾病，其自然病史尚不明确。我们的目的是对初诊时及长期随访期间的PH患者进行特征描述。对来自3个中心的22例患者进行了观察性回顾性研究。在排除垂体炎的继发原因后，14例患者经组织学确诊为PH，其余8例为临床诊断。所有患者在接受任何治疗前均进行了激素和影像学检查。中位随访时间为48个月（四分位间距：3 - 60个月）。女性占优势，男女比例为3.4:1。22例患者中有8例患有另一种自身免疫性疾病。头痛和性腺功能障碍是最常见的症状。5例患者表现为全垂体功能减退；17例患者存在垂体前叶功能减退，7例患有尿崩症。初诊时，9例患者接受了手术治疗，5例接受了激素替代治疗，8例接受了高剂量糖皮质激素治疗，其中5例联合使用了其他免疫抑制剂。在5年的随访期内，6例患者垂体激素缺乏完全恢复，6例部分恢复。手术治疗和药物治疗的患者之间未发现差异。总体复发率为18%。PH可表现为广泛的临床和激素紊乱。由于垂体激素恢复或复发可能在初始诊断多年后出现，因此需要长期随访来确定疾病的自然病史和对治疗的反应。我们建议手术和免疫抑制治疗应仅用于特殊情况。
