PDF(Pubmed)
Abstract：
CONCLUSIONS: Lymphocytic hypophysitis is a rare neuroendocrine disease characterised by an autoimmune inflammatory disorder of the pituitary gland. We report a 50-year-old woman who presented with headaches and bilateral sixth cranial nerve palsies. MRI of the pituitary revealed extensive fibrosis involving the sellar and extending into both cavernous sinuses causing bilateral occlusion of the internal carotid arteries (ICA). Transphenoidal biopsy confirmed the diagnosis of infiltrative fibrotic lymphocytic hypophysitis. Symptoms resolved with high dose of oral steroids but relapsed on tapering, requiring several treatments of i.v. pulse steroids over 8 months. Rituximab combined with mycophenolate mofetil was required to achieve long-term symptom relief. Serial MRI pituitary imaging showed stabilisation of her disease without reduction in sellar mass or regression of ICA occlusion. The patient\'s brain remained perfused solely by her posterior circulation. This case demonstrates an unusual presentation of a rare disease and highlights a successful steroid-sparing regimen in a refractory setting.
CONCLUSIONS: Lymphocytic hypophysitis is a rare inflammatory disorder of the pituitary gland. In exceptional cases, there is infiltration of the cavernous sinus with subsequent occlusion of the internal carotid arteries. First-line treatment of lymphocytic hypophysitis is high-dose glucocorticoids. Relapse after tapering or discontinuation is common and its use is limited by long-term adverse effects. There is a paucity of data for treatment of refractory lymphocytic hypophysitis. Goals of treatment should include improvement in symptoms, correction of hormonal insufficiencies, reduction in lesion size and prevention of recurrence. Steroid-sparing immunosuppressive drugs such as rituximab and mycophenolate mofetil have been successful in case reports. This therapeutic combination represents a viable alternative treatment for refractory disease.
CONCLUSIONS: Lymphocytic hypophysitis is a rare inflammatory disorder of the pituitary gland. In exceptional cases, there is infiltration of the cavernous sinus with subsequent occlusion of the internal carotid arteries. First-line treatment of lymphocytic hypophysitis is high-dose glucocorticoids. Relapse after tapering or discontinuation is common and its use is limited by long-term adverse effects. There is a paucity of data for treatment of refractory lymphocytic hypophysitis. Goals of treatment should include improvement in symptoms, correction of hormonal insufficiencies, reduction in lesion size and prevention of recurrence. Steroid-sparing immunosuppressive drugs such as rituximab and mycophenolate mofetil have been successful in case reports. This therapeutic combination represents a viable alternative treatment for refractory disease.
摘要：
结论：淋巴细胞性垂体炎是一种罕见的神经内分泌疾病，以脑垂体的自身免疫性炎症为特征。我们报告了一名50岁的女性，她出现了头痛和双侧第六颅神经麻痹。垂体的MRI显示广泛的纤维化涉及鞍区，并延伸到两个海绵状窦，导致颈内动脉（ICA）双侧闭塞。经蝶活检证实诊断为浸润性纤维化淋巴细胞性垂体炎。高剂量口服类固醇可缓解症状，但逐渐减少后复发，需要多次治疗静脉内脉冲类固醇超过8个月。需要利妥昔单抗联合霉酚酸酯才能实现长期症状缓解。连续MRI垂体成像显示她的疾病稳定，而鞍区质量不减少或ICA闭塞消退。患者的大脑仅通过后循环维持灌注。此病例表明罕见疾病的异常表现，并强调了在难治性环境中成功的类固醇保留方案。
结论：淋巴细胞性垂体炎是一种罕见的垂体炎性疾病。在特殊情况下,海绵窦浸润，随后颈内动脉闭塞。淋巴细胞性垂体炎的一线治疗是大剂量糖皮质激素。逐渐变细或停药后复发是常见的，其使用受到长期不利影响的限制。治疗难治性淋巴细胞性垂体炎的数据很少。治疗的目标应该包括改善症状，纠正荷尔蒙不足，减少病变大小和预防复发。在病例报告中，保留类固醇的免疫抑制药物如利妥昔单抗和霉酚酸酯已成功。该治疗组合代表难治性疾病的可行替代治疗。
结论：淋巴细胞性垂体炎是一种罕见的垂体炎性疾病。在特殊情况下,海绵窦浸润，随后颈内动脉闭塞。淋巴细胞性垂体炎的一线治疗是大剂量糖皮质激素。逐渐变细或停药后复发是常见的，其使用受到长期不利影响的限制。治疗难治性淋巴细胞性垂体炎的数据很少。治疗的目标应该包括改善症状，纠正荷尔蒙不足，减少病变大小和预防复发。在病例报告中，保留类固醇的免疫抑制药物如利妥昔单抗和霉酚酸酯已成功。该治疗组合代表难治性疾病的可行替代治疗。
