Swedish Pituitary Center, Department of Neuroendocrinology, Swedish Neuroscience Institute, Seattle, WA, 98122, USA.
Barrow Pituitary Center, Departments of Neuroendocrinology and Neurosurgery, Barrow Neurological Institute, University of Arizona College of Medicine, Phoenix, AZ, 85013, USA.
Pituitary. 2018 Jun;21(3):238-246. doi: 10.1007/s11102-017-0852-4.
IgG4-related hypophysitis is a rare clinical entity that forms part of an emerging group of multi-organ IgG4-related fibrosclerotic systemic diseases. The rare prevalence of the disease, presenting features that overlap with other sellar pathologies, and variable imaging features can make preoperative identification challenging.
We report three cases of isolated IgG4-related hypophysitis with atypical clinical and imaging features that mimicked those of pituitary apoplexy and other sellar lesions. Additionally, we review the literature of IgG4-related hypophysitis to provide context for individual patient data described herein.
All patients presented with symptoms that mimicked those of pituitary apoplexy and visual disturbance, and MRI findings suggestive of pituitary macroadenoma, Rathke's cleft cyst and craniopharyngioma. The clinical presentation warranted surgical decompression, resulting in rapid symptomatic improvement. Preoperative high-dose followed by postoperative low-dose glucocorticoid replacement therapy was administered in all cases. Histopathology showed dense infiltrate of IgG4 cells. Post-operative follow-up monitoring for 12-26 months revealed normal serum IgG4 levels with no other organ involvement, while endocrinological testing revealed persistent pituitary hormone deficiencies.
Our cases highlight the importance of considering IgG4-related hypophysitis in the differential diagnosis of solid and cystic sellar lesions presenting acutely with pituitary apoplexy symptoms. Existing diagnostic criteria may not be sufficiently precise to permit rapid and reliable identification, or avoidance of surgery in the acute setting. In contrast to other reports of the natural history of this condition, despite the severity of presenting features, the disease in our cases was pituitary-restricted with normal serum IgG4 levels.
IgG4 相关的垂体炎是一种罕见的临床实体，是一组新兴的多器官 IgG4 相关纤维硬化性系统性疾病的一部分。由于该病的罕见患病率、与其他鞍区病变重叠的表现特征以及可变的影像学特征，使得术前识别具有挑战性。
我们报告了三例孤立性 IgG4 相关的垂体炎，其临床表现和影像学特征不典型，类似于垂体卒中及其他鞍区病变。此外，我们还回顾了 IgG4 相关垂体炎的文献，为本文描述的个体患者数据提供背景。
所有患者均表现出类似于垂体卒中和视力障碍的症状，且 MRI 结果提示垂体大腺瘤、Rathke 裂囊肿和颅咽管瘤。临床表现需要手术减压，导致症状迅速改善。所有患者均接受了术前大剂量、术后小剂量糖皮质激素替代治疗。组织病理学显示 IgG4 细胞密集浸润。术后 12-26 个月的随访监测显示血清 IgG4 水平正常，无其他器官受累，而内分泌检查显示持续存在垂体激素缺乏。
我们的病例强调了在诊断急性表现为垂体卒中症状的实性和囊性鞍区病变时，需要考虑 IgG4 相关垂体炎的鉴别诊断。现有的诊断标准可能不够精确，无法快速可靠地识别，或避免在急性期进行手术。与该疾病自然病史的其他报告相比，尽管我们病例的表现特征严重，但疾病仅限于垂体，血清 IgG4 水平正常。
