Keys Phillip Howard, Hunt Patrick J, Anozie Clement, Cayenne Samir, Davila-Siliezar Pamela, Laylani Noor, Lee Andrew G
John Sealy School of Medicine, University of Texas Medical Branch, Galveston, TX, USA.
Medical Scientist Training Program, Baylor College of Medicine, Houston, TX, USA.
Case Rep Ophthalmol. 2023 Jul 12;14(1):319-325. doi: 10.1159/000531445. eCollection 2023 Jan-Dec.
Lymphocytic hypophysitis (LH) is a primary inflammatory disorder of the pituitary gland and infundibulum that commonly manifests in both mass effect and endocrinologic symptoms. Although the exact pathophysiology remains unclear, it has been increasingly linked to an autoimmune process. Complications arise by two separate mechanisms. Inflammation in the sella can lead to headaches and visual field defects. Pituitary inflammation and, chronically, fibrosis interfere with the gland's hormone-secreting capacity, often resulting in various endocrinopathies such as polyuria, polydipsia, amenorrhea, and others. While final histologic classification requires pathologic evaluation, diagnosis can often be made clinically with appropriate imaging. Treatment often consists of conservative management but can also include glucocorticoids or surgical resection. We present a case of biopsy-proven LH involving the entire pituitary, dubbed lymphocytic panhypophysitis (LPH) that was misdiagnosed for years as glaucoma due to the lack of endocrinopathy as well as delay in magnetic resonance imaging. After imaging revealed the sellar mass, the patient responded symptomatically to surgical resection and glucocorticoid treatment. LPH may present without endocrinologic symptoms and therefore mimic alternate diagnoses such as glaucoma. Clinicians should be suspicious of a diagnosis of glaucoma in the setting of a temporal field defect and lack of response to traditional therapy. A personal or family history of autoimmune disease in such patients should prompt further imaging and investigation. Therefore, endocrinopathy is supportive but not present in every case of LPH.
淋巴细胞性垂体炎(LH)是一种累及垂体和漏斗部的原发性炎症性疾病,通常表现为占位效应和内分泌症状。虽然确切的病理生理机制尚不清楚,但越来越多地与自身免疫过程相关。并发症通过两种不同机制产生。蝶鞍内的炎症可导致头痛和视野缺损。垂体炎症以及长期的纤维化会干扰腺体的激素分泌能力,常导致各种内分泌疾病,如多尿、烦渴、闭经等。虽然最终的组织学分类需要病理评估,但通过适当的影像学检查通常可在临床上做出诊断。治疗通常包括保守治疗,但也可能包括糖皮质激素或手术切除。我们报告一例经活检证实的累及整个垂体的LH,称为淋巴细胞性全垂体炎(LPH),由于缺乏内分泌病以及磁共振成像延迟,多年来被误诊为青光眼。影像学检查发现蝶鞍肿块后,患者对手术切除和糖皮质激素治疗有症状性反应。LPH可能无内分泌症状,因此可类似青光眼等其他诊断。临床医生在出现颞侧视野缺损且对传统治疗无反应的情况下应怀疑青光眼的诊断。此类患者的个人或家族自身免疫病史应促使进一步的影像学检查和调查。因此,内分泌病对LPH有支持作用,但并非在每例LPH中都存在。