lymphocytic hypophysitis

淋巴细胞性垂体炎
  • 文章类型: Journal Article
    垂体功能低下由于其非特异性症状而难以诊断,尤其是在存在合并症的情况下。一名77岁的厌食症和劳力性呼吸困难恶化的妇女最初被诊断为失代偿性干冷型心力衰竭。激素实验室检查表明继发性甲状腺功能减退是心力衰竭评估的一部分。此外,垂体磁共振成像显示垂体柄增厚和垂体后叶信号强度丧失,因此提示淋巴细胞性垂体炎。口服氢化可的松和左甲状腺素可改善持续性厌食症。在这种情况下,垂体功能减退偶尔表现为干冷型心力衰竭,因此,在并发心力衰竭的情况下,及时诊断具有挑战性。
    Hypopituitarism is difficult to diagnose because of its non-specific symptoms, especially in the presence of comorbidities. A 77-year-old woman with worsening anorexia and exertional dyspnea was initially diagnosed with decompensated dry cold-type heart failure. Hormonal laboratory tests indicated secondary hypothyroidism as a part of the evaluation of heart failure. Furthermore, pituitary magnetic resonance imaging revealed thickening of the pituitary stalk and a loss of signal intensity in the posterior pituitary, thus suggesting lymphocytic hypophysitis. Oral hydrocortisone and levothyroxine improved the persistent anorexia. In this case, hypopituitarism occasionally presented as dry cold-type heart failure, thus making a prompt diagnosis challenging in the setting of concurrent heart failure.
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  • 文章类型: Journal Article
    UNASSIGNED: Preoperative imaging for some unusual lesions in the sellar region can pose challenges in establishing a definitive diagnosis, impacting treatment strategies.
    UNASSIGNED: This study is a retrospective analysis of eight cases involving unusual sellar region lesions, all treated with endoscopic endonasal transsphenoidal surgery (EETS). We present the clinical, endocrine, and radiological characteristics, along with the outcomes of these cases.
    UNASSIGNED: Among the eight cases, the lesions were identified as follows: Solitary fibrous tumor (SFT) in one case, Lymphocytic hypophysitis (LYH) in one case, Cavernous sinus hemangiomas (CSH) in one case, Ossifying fibroma (OF) in two cases; Sphenoid sinus mucocele (SSM) in one case, Pituitary abscess (PA) in two cases. All patients underwent successful EETS, and their diagnoses were confirmed through pathological examination. Postoperatively, all patients had uneventful recoveries without occurrences of diabetes insipidus or visual impairment.
    UNASSIGNED: Our study retrospectively analyzed eight unusual lesions of the sellar region. Some lesions exhibit specific imaging characteristics and clinical details that can aid in preoperative diagnosis and inform treatment strategies for these unusual sellar diseases.
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  • 文章类型: Case Reports
    背景:淋巴细胞性垂体炎是一种罕见的自身免疫性疾病,通常在怀孕期间出现并引起垂体炎症。虽然病理生理学还没有很好的理解,它经常出现头痛,视觉障碍,和垂体功能减退的症状。然而,并非所有病例都可能出现垂体功能减退症,这可能使这种罕见疾病的发病率约为900万分之一,更难诊断。
    方法:我们介绍了一名35岁的G4P4女性,在产后2个月的前三个月期间,她患有进行性视力丧失和间歇性额叶头痛。她没有出现垂体功能减退的症状,她的激素组仅显示催乳素升高,可能是由于她的母乳喂养。她接受了右侧翼点开颅手术并对两个视神经进行减压治疗,鞍上肿块部分切除,和糖皮质激素治疗头痛和视力障碍。
    结论:该病例表现为淋巴细胞性垂体炎,但无垂体功能减退症状。这对门诊提供者来说很重要,特别是那些照顾怀孕患者的患者,以避免不利的结果。
    BACKGROUND: Lymphocytic hypophysitis is a rare autoimmune condition that usually presents during pregnancy and causes inflammation of the pituitary gland. Although the pathophysiology is not well understood, it often presents with headaches, visual disturbances, and symptoms of hypopituitarism. However, not all cases may present with hypopituitarism which can make this rare disease with an incidence of ~ 1 in 9 million much more difficult to diagnose.
    METHODS: We present a 35-year-old G4P4 woman with progressive vision loss and intermittent frontal headaches during her first trimester through 2 months postpartum. She presented with no symptoms of hypopituitarism and her hormone panel only showed elevated prolactin, possibly due to her breastfeeding. She was treated with a right pterional craniotomy with decompression of both optic nerves, partial resection of the suprasellar mass, and glucocorticoid therapy for headaches and visual disturbances.
    CONCLUSIONS: This case is notable for a presentation of lymphocytic hypophysitis without symptoms of hypopituitarism. This is important for outpatient providers to be aware of, especially those that care for pregnant patients so that unfavorable outcomes can be avoided.
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  • 文章类型: Systematic Review
    目的:探讨聚焦放疗在治疗药物治疗和手术难治性淋巴细胞性垂体炎(LH)中的潜在作用。
    方法:根据PRISMA指南进行了系统的文献综述,以确定对垂体炎的放射治疗的研究。以及我们机构的经验。
    结果:该研究包括8名患者,三个来自我们的机构,五个来自现有文献。演讲年龄从37岁到75岁不等,平均年龄为58岁。出现的症状包括7例患者的头痛和2例患者的复视。在四名患者中发现了放射前视野缺损。所有患者在放射前都表现出不同程度的垂体功能减退症,口服皮质类固醇是最初的药物治疗。在放疗前对两名患者进行了免疫抑制治疗。7例患者有经蝶窦手术史,经组织学证实为LH。三名患者接受了立体定向放射外科(SRS),而其余的收到FSRT,平均辐照体积为2.2cm3。在SRS组中施用12-15Gy的单次总剂量。在FSRT组中,剂量范围为24至30Gy,中位剂量为25Gy,以2Gy分数交付。四名患者获得了视野缺陷的分辨率,而另外两名患者则表现出相关的局灶性神经功能缺损的改善。放射后未显示先前存在的内分泌状态的变化,除了一个病人。在单疗程放疗后,七名患者获得了临床反应,而一名患者需要第二疗程。6例患者在至少12个月的随访期内,低剂量糖皮质激素保持稳定,一个人完全停止了它,没有复发。三名患者表现出完全的放射学反应,其余显示部分放射学反应。
    结论:聚焦辐射,包括FSRT,可以起到缓解症状的作用,有效质量收缩,并尽量减少对难治性LH患者关键周围结构的辐射暴露。然而,需要进一步的研究努力,以更好地阐明其效果和最佳剂量计划。
    OBJECTIVE: To explore the potential role of focused radiotherapy in managing the lymphocytic hypophysitis (LH) refractory to medical therapy and surgery.
    METHODS: A systematic literature review was conducted following PRISMA guidelines to identify the studies on radiation treatment for hypophysitis, along with the experience in our institution.
    RESULTS: The study included eight patients, three from our institution and five from existing literature. The age at presentation ranged from 37 to 75 years old, with a median age of 58. The presenting symptoms involved headache in seven patients and diplopia in two patients. Pre-radiation visual field defects were noticed in four patients. All patients exhibited variable degrees of hypopituitarism before radiation, with oral corticosteroids being the initial medical treatment. Immunosuppressive therapy was attempted in two patients prior to radiation. Seven patients had a history of transsphenoidal surgery with a histologically confirmed LH. Three patients underwent stereotactic radiosurgery (SRS), while the remaining received FSRT, with a mean irradiation volume of 2.2 cm3. A single-session total dose of 12 -15 Gy was administered in the SRS group. In the FSRT group, doses ranged from 24 to 30 Gy with a median dose of 25 Gy, delivered in 2 Gy fractions. Four patients achieved a resolution of visual field defects, while another two patients demonstrated improvement in their associated focal neurologic deficits. No change in pre-existing endocrine status was shown after radiation, except in one patient. Clinical response was achieved in seven patients after a single course of radiation, while one patient required the second course. Six patients remained stable on low-dose glucocorticoid during at least a 12-month follow-up period, and one discontinued it entirely without experiencing relapse. Three patients demonstrated a complete radiologic response, while the remaining showed a partial radiologic response.
    CONCLUSIONS: Focused radiation, including FSRT, can play a role in symptomatic relief, effective mass shrinkage, and minimizing radiation exposure to critical surrounding structures in patients with refractory LH. However, further research efforts are necessary to better clarify its effects and optimal dose planning.
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  • 文章类型: Case Reports
    淋巴细胞性垂体炎是一种被低估的疾病,其发病机制尚不清楚。冠状病毒后淋巴细胞性垂体炎是一种新兴的实体。
    方法:一名16岁以前健康的女孩出现垂体肿瘤综合征。她患有额叶头痛,多尿-多相综合征,视力受损。三周前,她被诊断出患有COVID-19感染。对比增强的磁共振成像(MRI)显示,在T1加权图像上,垂体肿大并具有强烈的同质增强后钆。排除其他鉴别诊断后,诊断为淋巴细胞性垂体炎。她开始服用甲基强的松龙。在第5天观察到临床症状的改善,头痛强度显著降低。
    本文总结了文献中报道的病例和我们的病例的数据,以强调冠状病毒是淋巴细胞性垂体炎的新触发因素。尽管这种并发症很少,近期COVID-19感染后怀疑有垂体炎的患者应仔细评估.
    结论:COVID-19感染可引起淋巴细胞性垂体炎。然而,就COVID-19与内分泌疾病之间的因果关系得出结论似乎为时过早。需要对更大样本进行进一步研究,以了解COVID-19感染后自身免疫性内分泌病的发病机理。
    UNASSIGNED: Lymphocytic hypophysitis is an underestimated disease and the pathogenesis is still poorly elucidated. Post-coronavirus lymphocytic hypophysitis is a new emerging entity.
    METHODS: A 16-year-old previously healthy girl presented with pituitary tumor syndrome. She suffered from frontal headaches, polyuria-polydipsic syndrome, and impaired visual acuity. She was diagnosed with COVID-19 infection three weeks before. Contrast-enhanced magnetic resonance imaging (MRI) revealed pituitary enlargement with intense homogenous enhancement postgadolinium on T1 weighted images. The diagnosis of lymphocytic hypophysitis was made after ruling out other differential diagnosis. She was started on methylprednisolone. Improvement of clinical symptoms was seen on day 5 with a significant decrease in headache intensity.
    UNASSIGNED: The article summarizes data from cases reported in the literature and our case to highlight coronavirus as a new trigger of lymphocytic hypophysitis. Despite the rarity of this complication, patients with a suspicion of hypophysitis after a recent COVID-19 infection should be carefully evaluated.
    CONCLUSIONS: COVID-19 infection can cause lymphocytic hypophysitis. However, it seems premature to conclude on the causal link between COVID-19 and endocrine diseases. Further studies on larger samples are needed to comprehend the pathogenesis of autoimmune endocrinopathies after COVID-19 infection.
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  • 文章类型: Case Reports
    一名75岁的妇女出现恶心和呕吐。磁共振成像(MRI)显示她有垂体肿块。活检显示淋巴细胞性垂体炎(LYH)。激素替代疗法改善了症状。虽然她没有症状,随访MRI显示肿块大小增加.静脉注射甲基强的松龙(IVMP)减少了肿块的大小;然而,右眼痛和动眼神经麻痹发展。MRI显示垂体肿块扩大到海绵窦右动眼神经和右颈内动脉(ICA),导致ICA狭窄。IVMP给药后,症状明显改善,但ICA狭窄持续存在。
    A 75-year-old woman presented with nausea and vomiting. Magnetic resonance imaging (MRI) revealed that she had a pituitary mass. A biopsy revealed lymphocytic hypophysitis (LYH). Symptoms were improved by hormone replacement therapy. Although she was asymptomatic, follow-up MRI revealed an increase in the size of the mass. Intravenous methylprednisolone (IVMP) reduced the size of the mass; however, right ophthalmalgia and oculomotor nerve palsy developed. MRI showed that the pituitary mass had enlarged to the right oculomotor nerve in the cavernous sinus and to the right internal carotid artery (ICA), causing stenosis of the ICA. After IVMP administration, the symptoms dramatically improved, but ICA stenosis persisted.
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  • 文章类型: Journal Article
    儿童期发作的淋巴细胞性漏斗型神经垂体炎(LINH)很少有报道。通过垂体活检进行病理评估对于明确诊断LINH是必要的。然而,垂体活检是一种高度侵入性的手术。最近,抗rabphilin-3A抗体(RPH3A-Ab)已被报道为成人LINH的有希望的诊断标记;然而,在儿科人群中很少有这样的报道。我们报告了一名8岁男孩患有中央性尿崩症(CDI)的病例,该男孩根据RPH3A-Ab阳性临床诊断为LINH。使用水剥夺试验结合去氨加压素给药,他被诊断为CDI。血清和脑脊液肿瘤标志物均为阴性,和T1加权磁共振成像(MRI)显示垂体后叶没有高信号强度和垂体柄增大。垂体前叶功能测试未发现异常。由于其侵袭性,没有进行垂体活检,并开始去氨加压素治疗。CDI发病三个月后,患者RPH3A-Ab检测呈阳性.CDI发病后9个月进行的MRI显示垂体柄增大改善,临床过程证实了我们对LINH的诊断。RPH3A-Ab可用作儿科人群中LINH的早期诊断工具。
    Childhood-onset lymphocytic infundibuloneurohypophysitis (LINH) has rarely been reported. Pathological evaluation via pituitary biopsy is necessary for a definitive diagnosis of LINH. However, pituitary biopsy is a highly invasive procedure. Recently, anti-rabphilin-3A antibody (RPH3A-Ab) has been reported as a promising diagnostic marker for LINH in adults; however, there are few such reports in the pediatric population. We report the case of an 8-year-old boy with central diabetes insipidus (CDI) who was clinically diagnosed with LINH based on RPH3A-Ab positivity. He was diagnosed with CDI using a water deprivation test combined with desmopressin administration. Serum and cerebrospinal fluid tumor markers were negative, and T1-weighted magnetic resonance imaging (MRI) revealed the absence of high signal intensity in the posterior pituitary gland and an enlarged pituitary stalk. Anterior pituitary function tests revealed no abnormalities. No pituitary biopsy was performed because of its invasive nature, and desmopressin treatment was initiated. Three months after CDI onset, the patient tested positive for RPH3A-Ab. MRI performed 9 months after CDI onset revealed amelioration of the pituitary stalk enlargement, and the clinical course corroborated our diagnosis of LINH. RPH3A-Ab may be useful as an early diagnostic tool for LINH in the pediatric population.
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  • 文章类型: Journal Article
    这项荟萃分析是为了分析临床表现,磁共振成像(MRI)特征,和淋巴细胞性垂体炎(LYH)的管理。
    从2010年1月至2020年12月检索了四个不同的数据库,两名研究人员独立进行了文献筛选,数据提取,和质量评估。我们使用随机效应荟萃分析以95%CI计算总相对风险。
    这项荟萃分析显示,LYH患者中女性的百分比为78%。15%的女性患者LYH与妊娠有关,头痛(49%)和中枢神经性尿崩症(CDI)的症状(45%)是最常见的表现。在24%的LYH患者中,与另一种自身免疫性疾病有关.继发性性腺功能减退的发生率,继发性肾上腺功能减退,继发性甲状腺功能减退,生长激素缺乏是54%,49%,43%,22%,分别。垂体对比增强(63%),对称垂体肿大(60%),垂体柄增厚(58%),鞍区包块或鞍上延伸(58%),垂体后叶高强度丢失(50%)是典型的MRI表现。关于LYH治疗,观察或激素替代的患者百分比,类固醇治疗,手术率是43%,36%,34%,分别。
    充分了解淋巴细胞性垂体炎的临床特征具有重要意义,减少漏诊和误诊,避免不必要的手术,维持正常的垂体功能。
    UNASSIGNED: This meta-analysis was performed to analyze the clinical presentation, magnetic resonance imaging (MRI) characteristics, and the management of lymphocytic hypophysitis (LYH).
    UNASSIGNED: Four different databases were searched from January 2010 to December 2020, two researchers independently conducted literature screening, data extraction, and quality evaluation. We used a random effects meta-analysis to calculate summary relative risks with 95% CI.
    UNASSIGNED: This meta-analysis showed that the percentage of women among LYH patients was 78%. LYH was associated with pregnancy in 15% of female patients, with headache (49%) and symptoms of central diabetes insipidus (CDI) (45%) being the most frequent presentation. In 24% of LYH patients, there was an association with another autoimmune disease. The incidence of secondary hypogonadism, secondary hypoadrenalism, secondary hypothyroidism, and growth hormone deficit was 54%, 49%, 43%, and 22%, respectively. Pituitary contrast enhancement (63%), symmetrical pituitary enlargement (60%), thickening of the pituitary stalk (58%), sella mass or suprasellar extension (58%), and loss of posterior pituitary hyperintensity (50%) were typical MRI findings. Regarding LYH treatment, the percentage of patients who had observation or hormone replacement, steroid therapy, and surgery was 43%, 36%, and 34%, respectively.
    UNASSIGNED: It is of great significance to fully understand the clinical characteristics of lymphocytic hypophysitis, reduce missed diagnosis and misdiagnosis, avoid unnecessary surgery and maintain normal pituitary function.
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  • 文章类型: Case Reports
    淋巴细胞性垂体炎(LH)是垂体和漏斗的原发性炎症性疾病,通常表现为肿块效应和内分泌症状。尽管确切的病理生理学尚不清楚,它越来越多地与自身免疫过程联系在一起。并发症由两个单独的机制产生。蝶鞍中的炎症可导致头痛和视野缺陷。垂体炎症和,长期而言,纤维化干扰腺体的激素分泌能力,经常导致各种内分泌疾病,如多尿,多饮,闭经,和其他人。虽然最终的组织学分类需要病理评估,通常可以通过适当的影像学检查在临床上做出诊断。治疗通常包括保守治疗,但也可以包括糖皮质激素或手术切除。我们介绍了一例活检证实的LH涉及整个垂体,被称为淋巴细胞性垂体炎(LPH),由于缺乏内分泌病以及磁共振成像延迟而被误诊为青光眼。成像后发现了鞍状肿块,患者对手术切除和糖皮质激素治疗有对症反应.LPH可能表现为无内分泌症状,因此类似于青光眼等替代诊断。在颞叶视野缺损和对传统治疗缺乏反应的情况下,临床医生应该怀疑青光眼的诊断。此类患者的自身免疫性疾病的个人或家族史应提示进一步的影像学检查和调查。因此,内分泌病是支持性的,但并非在所有LPH病例中都存在。
    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.
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  • 文章类型: Review
    已知视神经炎(神经病变)或垂体功能减退症在COVID-19疫苗接种后单独发生。在这份报告中,我们描述了COVID-19疫苗接种后发生的罕见的垂体炎和视神经炎组合。一名74岁的妇女开始口渴,多饮,和多尿,并在第四次COVID-19mRNA疫苗接种后1个月被诊断为中枢性尿崩症。头部磁共振成像(MRI)显示,在T1加权图像上,垂体后叶中垂体茎增厚,垂体腺体增大,对比度增强,并且没有高强度信号。导致淋巴细胞性垂体炎的诊断。她对去氨加压素鼻腔喷雾剂很好,直到两个月后,当她患上双侧视神经炎时,连同步态障碍,上肢的意向震颤,尿潴留,便秘,下肢远端感觉异常,左侧中度偏瘫.自身抗体,包括抗水通道蛋白4(AQP4)和抗髓磷脂少突胶质细胞糖蛋白(MOG),都是负面的。她在MRI上显示多灶性脊髓病变,并通过脊髓穿刺获得脑脊液中的寡克隆带,因此在多发性硬化症的初步诊断中接受了甲基强的松龙的类固醇脉冲疗法,导致视力恢复和神经症状的缓解。在文献综述中,视神经炎和垂体炎的组合,主要是尿崩症,在COVID-19大流行之前,有15例患者报告为病例报告。COVID-19疫苗接种会引发该患者垂体炎和视神经炎的发作。
    Either optic neuritis (neuropathy) or hypopituitarism has been known to occur separately after COVID-19 vaccination. In this report, we describe the rare combination of hypophysitis and optic neuritis which occurred after COVID-19 vaccination. A 74-year-old woman began to have thirst, polydipsia, and polyuria, and was diagnosed as central diabetes insipidus 1 month after the fourth COVID-19 mRNA vaccine. Head magnetic resonance imaging (MRI) disclosed the thickened pituitary stalk and enlarged pituitary gland with high contrast enhancement as well as the absence of high-intensity signals in the posterior pituitary lobe on the T1-weighted image, leading to the diagnosis of lymphocytic hypophysitis. She was well with desmopressin nasal spray until further 2 months later, when she developed bilateral optic neuritis, together with gait disturbance, intention tremor of the upper extremities, urinary retention, constipation, abnormal sensation in the distal part of the lower extremities, and moderate hemiplegia on the left side. Autoantibodies, including anti-aquaporin 4 (AQP4) and anti-myelin oligodendrocyte glycoprotein (MOG), were all negative. She showed multifocal spinal cord lesions on MRI and oligoclonal bands in the cerebrospinal fluid obtained by spinal tap, and so underwent steroid pulse therapy with methylprednisolone in the tentative diagnosis of multiple sclerosis, resulting in visual acuity recovery and alleviation of neurological symptoms. In the literature review, the combination of optic neuritis and hypophysitis, mostly with diabetes insipidus, was reported in 15 patients as case reports before the years of COVID-19 pandemic. The COVID-19 vaccination would trigger the onset of hypophysitis and optic neuritis in this patient.
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