Department of Endocrinology, National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK), National Institutes of Health (NIH), Bethesda, MD, USA.
Section on Endocrinology & Genetics (SEGEN), Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD), National Institutes of Health (NIH), Bethesda, MD, 20892, USA.
Rev Endocr Metab Disord. 2018 Dec;19(4):335-347. doi: 10.1007/s11154-018-9480-1.
The pituitary gland and the hypothalamus can be affected by autoimmune-mediated structural and functional disruption. These autoimmune-mediated diseases occur more commonly in females and are often found during pregnancy or in the post-partum period. Autoimmune diseases can either affect parts of the pituitary or hypothalamus, or can involve both sellar and suprasellar structures. Most of these cases comprise primary hypophysitis (PRH). Over the years, there has been a tremendous increase in the number of reported PRH cases and related disorders, including hypophysitis induced by immune checkpoint inhibitors. With this increasing data, more light is being shed on the spectrum of clinical presentations, biochemical and imaging abnormalities of these disorders. Regardless, these disorders are still relatively rare. The clinical presentation can vary vastly, based on the type of pituitary cell or the area of the suprasellar region affected. The severity can range from clinically silent disease to progressive and rapid deterioration and death, likely due to unrecognized central adrenal insufficiency. Although biopsy remains a gold standard for diagnosing these disorders, the current standard of practice is biochemical assessment for hormonal deficiencies and imaging studies. In several instances, these disorders spontaneously resolve, but medical or surgical intervention might be necessary to treat symptomatic disease. Due to the subtlety and a vast spectrum of clinical manifestations which could often be asymptomatic, and the rarity of the occurrence of these diseases in clinical practice, the diagnosis can be easily missed which could potentially lead to substantial morbidity or mortality. Therefore, it is crucial to have a strong clinical suspicion and pursue timely biochemical and imaging studies to initiate prompt treatment. In this article, we review the various autoimmune conditions that affect the sellar and suprasellar structures, their diagnostic approach and management of these disorders.
垂体和下丘脑可受到自身免疫介导的结构和功能障碍的影响。这些自身免疫介导的疾病在女性中更为常见，通常发生在妊娠期间或产后。自身免疫性疾病可以影响垂体或下丘脑的一部分，也可以累及鞍内和鞍上结构。这些病例大多数为原发性垂体炎（PRH）。多年来，报告的 PRH 病例和相关疾病的数量呈指数级增长，包括免疫检查点抑制剂引起的垂体炎。随着这些数据的增加，更多的研究揭示了这些疾病的临床表现、生化和影像学异常的范围。尽管如此，这些疾病仍然相对罕见。根据受影响的垂体细胞类型或鞍上区域的不同，临床表现差异很大。严重程度从无症状疾病到进行性和快速恶化以及死亡不等，可能是由于未被识别的中枢肾上腺功能不全所致。虽然活检仍然是诊断这些疾病的金标准，但目前的标准治疗方法是进行激素缺乏的生化评估和影像学研究。在某些情况下，这些疾病会自发缓解，但可能需要医疗或手术干预来治疗有症状的疾病。由于这些疾病的临床表现微妙，范围广泛，且常常无症状，并且在临床实践中很少发生，因此容易被漏诊，这可能导致严重的发病率或死亡率。因此，强烈的临床怀疑和及时进行生化和影像学研究以启动及时治疗至关重要。在本文中，我们回顾了影响鞍内和鞍上结构的各种自身免疫性疾病，以及它们的诊断方法和治疗方法。
