Clínica de Neuroendocrinología, Departamento de Endocrinología y Metabolismo, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Vasco de Quiroga 15, Sección XVI, Tlalpan, 14000, Mexico City, Mexico.
Departamento de Inmunología y Reumatología, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.
Clin Rheumatol. 2020 Feb;39(2):595-606. doi: 10.1007/s10067-019-04735-7. Epub 2019 Aug 24.
Granulomatosis with polyangiitis (GPA) is a necrotizing granulomatous vasculitis of small vessels that affect the pituitary gland in less than 1% of cases being exceptionally rare. To describe the clinical, biochemical, radiological findings, treatment, and outcomes of 4 patients with GPA-related hypophysitis. A systematic review of published cases with the same diagnosis is presented as well. A cross-sectional case series of patients with hypophysitis due to GPA from 1981 to 2018 at a third level specialty center. Literature review was performed searching in seven different digital databases for terms "granulomatosis with polyangiitis" and "pituitary gland" or "hypophysitis," including in the analysis all published cases between 1950 and 2019 with a minimum follow-up of 6 months. We found 197 patients with GPA in our institution of whom 4 patients (2.0%) had pituitary involvement. Clinical characteristics and outcomes are described. We also reviewed 7 case series, and 36 case reports describing pituitary dysfunction related to GPA from 1953 to 2019, including the clinical picture of an additional 74 patients. Pituitary dysfunction due to GPA is rare. Treatment is targeted to control systemic manifestations; nevertheless, the outcome of the pituitary function is poor. Central diabetes insipidus, particularly in younger women with other systemic features, should raise suspicion of GPA.Key Points• Involvement of the pituitary gland is an uncommon manifestation in GPA patients. The presence of central diabetes insipidus in the setting of systemic symptoms should prompt its suspicion.• In patients with pituitary involvement due to GPA, affection of other endocrine glands is rare, neither concomitant nor in different times during the disease course. This may arise the hypothesis of a local or regional pathogenesis affection of the gland.• There is no consensus on the best therapy strategy for GPA hypophysitis. Although the use of glucocorticoids with CYC is the most common drug combination, no differences in the outcome of the pituitary function and GPA disease course are seen with other immunosuppressants.• Poor prognosis regarding pituitary function is expected due to possible permanent pituitary tissue damage that results in the need of permanent hormonal replacement.
显微镜下多血管炎（GPA）是一种罕见的小血管坏死性肉芽肿性血管炎，在不到 1%的病例中会影响脑垂体。本文描述了 4 例 GPA 相关垂体炎患者的临床、生化、影像学表现、治疗和结局，并对相同诊断的已发表病例进行了系统回顾。这是一项横断面病例系列研究，纳入了 1981 年至 2018 年在三级专业中心因 GPA 导致垂体炎的患者。文献检索在七个不同的数字数据库中检索了术语“显微镜下多血管炎”和“垂体”或“垂体炎”，并对 1950 年至 2019 年发表的所有病例进行了分析，这些病例的随访时间至少为 6 个月。在我们的机构中发现了 197 例 GPA 患者，其中 4 例（2.0%）存在垂体受累。描述了临床特征和结局。我们还回顾了 7 个病例系列和 36 个病例报告，这些报告描述了 1953 年至 2019 年与 GPA 相关的垂体功能障碍，包括另外 74 例患者的临床表现。GPA 引起的垂体功能障碍很少见。治疗针对控制全身表现；然而，垂体功能的结果很差。中枢性尿崩症，尤其是年轻女性出现其他全身症状时，应怀疑 GPA。
GPA 患者中，垂体受累并不常见。如果存在中枢性尿崩症且伴有全身症状，应怀疑 GPA。
在因 GPA 而出现垂体受累的患者中，其他内分泌腺受累很少见，无论是在疾病过程中同时出现，还是不同时出现。这可能会引起局部或区域性发病机制影响腺体的假说。
GPA 垂体炎的最佳治疗策略尚未达成共识。虽然最常见的药物联合是使用糖皮质激素加 CYC，但其他免疫抑制剂在垂体功能和 GPA 病程方面的结果没有差异。
由于可能导致永久性垂体组织损伤，需要永久性激素替代，因此预计垂体功能预后不佳。
