Cuevas-Ramos Daniel, Gómez-Sámano Miguel A, Velasco-Espinosa Oliver A, Flores-Guerrero Zazilnait, Porras-Topete Eduardo, Ulloa-Peregrina Jonathan de J, Reyes-Flores Jacqueline I, Gómez-Pérez Francisco J
Endocrinol Diabetes Metab Case Rep. 2025 Sep 11;2025(3). doi: 10.1530/EDM-25-0019. Print 2025 Jul 1.
A 39-year-old woman presented with a 5-year history of severe intermittent headaches, rhinitis, hemoptysis, unintentional weight loss of 40 kg over a year, and unilateral vision loss. Then, she noticed polyuria, amenorrhea, muscle weakness, and cold intolerance. Diagnosis of granulomatosis with polyangiitis (GPA) was confirmed with elevated c-ANCA levels and PR3-positive antibodies. Physical examination revealed hypotension, absence of pubic and axillary hair, and classical signs of hypothyroidism. The patient reported multiple previous hospitalizations due to episodes of hypernatremia, hypotension, and hypoglycemia. The biochemical evaluation showed early signs of chronic kidney disease and central adrenal, thyroid, and gonadotropin deficiencies. Pituitary MRI revealed a heterogeneous pituitary gland with peripheral enhancement, central necrosis, and extension to adjacent structures, as well as the absence of posterior pituitary bright spot on T1-weighted imaging. A diagnosis of GPA with pituitary involvement was established. Remission therapy with corticosteroids and rituximab was started. After disease control, pituitary hormonal deficiencies persisted, requiring long-term hormone replacement therapy.
Pituitary involvement in cases with GPA is a rare manifestation frequently misdiagnosed. It is important to be aware of hypophysitis as a GPA activity complication that warrants a prompt diagnostic approach and treatment. The pathophysiology of hypophysitis may be mediated by a granulomatous lesion or due to vascular damage. Pituitary dysfunction in GPA may occur at any moment, as an initial manifestation or as a concomitant syndrome together with other organ compromise. Deficiency of arginine vasopressin and central hypogonadism are the most frequent pituitary hormonal alterations. Pituitary dysfunction usually persists despite remission of systemic activity, requiring long-term hormone replacement therapy and surveillance.
一名39岁女性，有5年严重间歇性头痛、鼻炎、咯血病史，一年来体重意外减轻40公斤，且有单侧视力丧失。随后，她出现多尿、闭经、肌肉无力和不耐寒。c-ANCA水平升高及PR3阳性抗体确诊为肉芽肿性多血管炎（GPA）。体格检查发现低血压、阴毛和腋毛缺失以及甲状腺功能减退的典型体征。患者报告此前因高钠血症、低血压和低血糖发作多次住院。生化评估显示有慢性肾病以及中枢性肾上腺、甲状腺和促性腺激素缺乏的早期迹象。垂体MRI显示垂体不均匀，周边强化，中央坏死并延伸至相邻结构，且在T1加权成像上垂体后叶亮点缺失。确诊为GPA累及垂体。开始使用皮质类固醇和利妥昔单抗进行缓解治疗。疾病得到控制后，垂体激素缺乏仍然存在，需要长期激素替代治疗。
GPA病例中垂体受累是一种罕见表现，常被误诊。重要的是要认识到垂体炎是GPA活动的一种并发症，需要及时诊断和治疗。垂体炎的病理生理学可能由肉芽肿性病变介导或由于血管损伤。GPA中的垂体功能障碍可能在任何时候出现，作为初始表现或与其他器官损害同时出现的综合征。精氨酸加压素缺乏和中枢性性腺功能减退是最常见的垂体激素改变。尽管全身活动缓解，但垂体功能障碍通常仍然存在，需要长期激素替代治疗和监测。
