原发性垂体炎

原发性垂体炎可分为3种类型：淋巴细胞性垂体炎、肉芽肿性垂体炎、黄瘤病性垂体炎。原发性垂体炎发病率较低，多数在手术后根据病理结果得以确诊。目前认为它是一种器官特异性自身免疫性疾病。临床表现和影像学特点与垂体肿瘤相似，但内分泌学检查和病理学特点与垂体肿瘤不同。激素冲击治疗和手术治疗是两种治疗手段。     

淋巴细胞性垂体炎的诊断与治疗

淋巴细胞性垂体炎(LYH)是一种较少见的自身免疫性内分泌疾病,以垂体组织内淋巴细胞浸润为特征,主要表现为颅内占位、垂体前叶功能减退和(或)中枢性尿崩症,需与其他类型的鞍区占位性病变鉴别.糖皮质激素治疗可以有效缩小病变并改善垂体功能.     

淋巴细胞性垂体炎三例并文献复习

目的提高临床医师对淋巴细胞性垂体炎的认识。方法分析3例淋巴细胞性垂体炎的临床特点和病理结果。结果3例患者均为青年女性,例1产后起病,例2和例3在非妊娠或产后期间发病;影像学特点:病变广泛,不仅局限于鞍区和垂体柄,并向下丘脑扩展呈“舌状”改变侵犯海绵窦;增强后病变均匀或环状强化;主要临床表现:(1)垂体功能受损和尿崩症;(2)有局部受压症状,如头痛,视交叉受压;(3)病变侵犯海绵窦,引起动眼、外展神经麻痹;2例经病理证实为淋巴细胞性垂体炎,垂体组织见大量淋巴细胞、浆细胞、组织细胞浸润;2例大剂量甲泼尼龙治疗后,症状明显好转。结论淋巴细胞性垂体炎亦可发生在非妊娠或产后期间的年轻女性,大剂量甲泼尼龙冲击治疗能有效缩小病变和改善垂体功能。     

以"垂体占位"为影像学表现并自发缓解的淋巴细胞性垂体炎一例

淋巴细胞性垂体炎(LyH)是一种少见的自身免疫病,本文报道1例影像显示"垂体占位"并自发缓解的LyH患者,经临床观察及文献复习,有理由认为对于临床症状轻微、占位效应不明显者,随访观察或生理剂量替代治疗可能是更加个体化的治疗策略。     

妊娠与淋巴细胞性垂体炎

淋巴细胞性垂体炎(lymphocytic hypophysitis,LYH)是一种较为少见的自身免疫性疾病,女性多见,且与妊娠密切相关。LYH患者的临床表现与疾病进展速度、病变范围、严重程度、疾病进展阶段有关,可表现为占位效应、部分或全垂体前叶功能减退、中枢性尿崩症等。妊娠期女性的垂体解剖结构和垂体相关激素水平发生变化...     

关于淋巴细胞性垂体炎临床思考

淋巴细胞性垂体炎是临床上比较容易见到的疾病,但临床表现却不尽相同。垂体发病的人数远远超过想象,对中国人口质量的影响巨大。教科书中的诊断标准过于苛刻,所以明确诊断也十分困难,误诊和漏诊的病人很多。其既可以单独存在,也可以与糖尿病、甲状腺功能亢进、骨病或其他自身免疫性疾病同时发生。垂体肾上腺轴、甲状腺轴和性腺轴均低下者较少,但部分异常也是重要证据。对于临床特点、实验室检查和影像学表现整合起来分析,才能怀疑为垂体炎。在除外其他可引起垂体炎的继发性原因后,如结核、梅毒、朗罕氏细胞增多症、噬伊红肉芽肿等,临床可考虑为淋巴细胞性垂体炎,有文献认为大剂量糖皮质激素可用于诊断性治疗。小剂量的环孢素A(25 mg,每日2次)可以推荐使用治疗该病,但要注意肝损害。垂体活检的病理是诊断淋巴细胞性垂体炎的金标准。     

自身免疫性胰腺炎合并淋巴细胞性垂体炎1例报道

通过1例罕见的自身免疫性胰腺炎合并淋巴细胞性垂体炎的报道，提高临床医师对此类疾病的鉴别及诊治能力。     

淋巴细胞性垂体炎的研究进展

淋巴细胞性垂体炎是一种少见的自身免疫性内分泌疾病，好发于妊娠后期或产后年轻妇女，以垂体扩大，淋巴细胞浸润和垂体功能减退为特征，临床表现多变，手术是目前确诊和治疗的主要手段，开发具有确诊价值的血清学检查指标是今后研究的重点。     

淋巴细胞性垂体炎诊断和鉴别诊断

淋巴细胞性垂体炎是一种罕见的自身免疫性疾病,以垂体淋巴细胞浸润为特征。随着散发病例的累积和影像学技术的发展,人们对该病的认识水平逐渐提高,根据患者临床表现、实验室检查和典型的影像学表现,即使没有获得病理标本,也可临床诊断。该病患病率低,临床表现多样,极易漏诊、误诊,需与垂体腺瘤、颅咽管瘤、生殖细胞瘤、垂体脓肿、Rathke囊肿、继发性垂体炎、垂体转移瘤等疾病鉴别。     

以淋巴细胞性垂体炎为主要表现的干燥综合征一例

淋巴细胞性垂体炎(lymphocytic hypophysitis,LHP)是到目前为止人类发现最晚的一种自身免疫性内分泌疾病,以垂体淋巴细胞浸润为特征,多见于妊娠后期或产后年轻妇女。临床表现类似垂体肿瘤,有头痛、视力下降和垂体功能减退。自1962年Goudie和Pinkerlon报道3例在尸检标本中证实的淋巴细胞性垂体炎,迄今已超过145例,国内报道4例,1例有病理诊断。实际上其发病率远高于文献报道,主要因为对本病的认识不足,以及缺乏可靠的诊断依据。本文     

Lymphocytic hypophysitis with lachrymal, salivary and thyroid gland involvement

We report on hypophysitis associated with a prominent lymphoid infiltration of salivary and lachrymal glands in a 35-year-old woman with a dramatic response to steroids. Four years later, overt Graves' disease developed. To our knowledge, pseudotumoral lymphocytic infiltration of both lachrymal and salivary glands has never been described in association with hypophysitis. Benign lymphocytic hypophysitis may belong to a spectrum that extends from low-grade lymphoid proliferation to autoimmune disease. Such a process may follow a regional tissue distribution including pituitary, thyroid, lachrymal and salivary glands.     

Lymphocytic Hypophysitis Masking a Suprasellar Germinoma in a 12-year-old Girl<197>A Case Report

Case history, light and electron microscopic findings of a case of a lymphocytic hypophysitis in coincidence with a suprasellar germinoma in a 12-year-old girl are reported. The girl presented with a long time case history of diabetes insipidus and subsequent panhypopituitarism. Two years after the diagnosis of diabetes insipidus magnetic resonance imaging (MRI) showed a tumorous enlargement of the sellar content and pituitary stalk. A transnasal exploration was initially performed and revealed a lymphocytic hypophysitis. Light microscopy showed a dense infiltration of mature lymphocytes and plasma cells in the interstitium of the anterior pituitary gland. The stalk area could not be exposed to exclude a germinoma. One year later the lesion relapsed despite dexamethason therapy and a second operation by another neurosurgeon had to be performed. Light microscopy showed lymphocytic infiltrates, fibrosis and necrosis. The diagnosis was a lymphocytic hypophysitis again. Though transcranially exposed only pituitary tissue was removed. No infundibular mass became visible at surgery as shown by MRI. The girl developed five months later multiple cerebral lesions, which revealed to be a germinoma. Lymphocytic hypophysitis in children is very rare and a coincedence with a germinoma has not been described from histopathological aspect until now. The origin of the pituitary infiltration is discussed.     

Pituitary and systemic autoimmunity in a case of intrasellar germinoma

Germinomas arising in the sella turcica are difficult to differentiate from autoimmune hypophysitis because of similar clinical and pathological features. This differentiation, nevertheless, is critical for patient care due to different treatments of the two diseases. We report the case of an 11-year-old girl who presented with diabetes insipidus and growth retardation, and was found to have an intra- and supra-sellar mass. Initial examination of the pituitary biopsy showed diffuse lymphocytic infiltration of the adenohypophysis and absent placental alkaline phosphatase expression, leading to a diagnosis of hypophysitis and glucocorticoid treatment. Because of the lack of clinical and radiological response, the pituitary specimen was re-examined, revealing this time the presence of scattered c-kit and Oct4 positive germinoma cells. The revised diagnosis prompted the initiation of radiotherapy, which induced disappearance of the pituitary mass. Immunological studies showed that the patient’s serum recognized antigens expressed by the patient’s own germinoma cells, as well as pituitary antigens like growth hormone and systemic antigens like the Sjögren syndrome antigen B and alpha-enolase. The study first reports the presence of pituitary and systemic antibodies in a patient with intrasellar germinoma, and reminds us that diffuse lymphocytic infiltration of the pituitary gland and pituitary antibodies does not always indicate a diagnosis of autoimmune hypophysitis.     

Lymphocytic panhypophysitis in a young man with involvement of the cavernous sinus and clivus

Lymphocytic hypophysitis is an unusual inflammatory lesion that is caused by autoimmune destruction of the pituitary gland. We report a case of 42-year-old man who presented with a 6-month history of severe headache, blurred vision in the right eye, hearing loss, polyuria, polydipsia, and impotence. Medical history showed that he and his mother had osteopetrosis. The results of the physical examination and laboratory tests showed that secondary hypothyroidism, hypogonadism, and hypocortisolism had developed. Central diabetes insipidus was diagnosed by water deprivation test. MRI of the sella showed pituitary enlargement with symmetrical suprasellar expansion, compression of the chiasma, thickened infundibulum, and involvement of both bilateral cavernous sinuses and clivus. Hormonal substitution with hydrocortisone, levothyroxine, and DDAVP resulted in rapid improvement of all symptoms and signs. Transsphenoidal biopsy was diagnostic of lymphocytic hypophysitis. In spite of extensive literature reviewing, we have not been aware of any case of lymphocytic hypophysitis with clivus involvement. The present case represents a variant of lymphocytic hypophysitis which has progressed to involve bilateral cavernous sinuses and the clivus.     

A case of cystic lymphocytic hypophysitis with cacosmia and hypopituitarism

Lymphocytic hypophysitis is a rare inflammatory disease of the pituitary gland that is being increasingly recognized as a cause of hypopituitarism. This condition may be due to an autoimmune pituitary destruction which usually occurs in young women during pregnancy or in the immediate postpartum period. We describe a case of cystic pituitary mass in a thirty-eight year-old woman presenting with nausea, vomiting, cold intolerance, blurring of vision and the presence of disagreeable odors for a one-month period. She had secondary amenorrhea and galactorrhea for three months. Combined anterior pituitary stimulation test confirmed the diagnosis of hypopituitarism. Magnetic resonance imaging scan with enhancement showed a huge cystic sellar mass with suprasellar extension and thickening of the pituitary stalk. Transsphenoidal exploration was performed with preoperative diagnosis of pituitary macroadenoma with cystic necrosis. Histological examination revealed lymphocytic hypophysitis characteristic of diffuse, dense lymphocytes and plasma cells infiltration with surrounding interstitial reactive fibrosis. Postoperatively, the patient's olfactory function returned to normal but combined anterior pituitary stimulation test showed persistence of hypopituitarism with mild hyperprolactinemia. Prednisolone, thyroxine and estrogen replacements were started and clinical symptoms were much improved. In summary, we report an extremely rare case of a woman with cystic lymphocytic hypophysitis with cacosmia and hypopituitarism, confirmed by histological examination.     

Lymphocytic hypophysitis in a patient with systemic lupus erythematosus

A case of lymphocytic hypophysitis in a patient with systemic lupus erythematosus is described. A 20-year-old woman was admitted to our hospital with generalized myalgia and facial rash in May 1998. The patient had a medical history, physical examination, and laboratory findings compatible with systemic lupus erythematosus (SLE). Headache and nausea had developed 3 months previously and worsened over the following months. Hormonal investigation showed hypopituitarism except for prolactin. A magnetic resonance image of the brain showed a mass lesion in the pituitary fossa. A trans-sphenoidal surgical procedure was performed which revealed a dark-yellowish hematoma. Microscopic examination showed diffuse infiltration of lymphocytes and plasma cells with fibrosis in the anterior pituitary. Post-operatively the patient's headaches and nausea resolved. This indicates that lymphocytic hypophysitis may be associated with SLE.     

Lymphocytic hypophysitis accompanied by aseptic meningitis mimics subacute meningoencephalitis

A 66-year-old man was admitted to our hospital with muscle pain, fatigue and appetite loss that had lasted for a month. The patient was somnolent and had nuchal stiffness with a high fever. Cerebrospinal fluid (CSF) tests demonstrated lymphocytic pleocytosis with a decreased CSF-blood glucose ratio. The tests for tuberculous, fungal and carcinomatous meningitis and herpetic meningoencephalitis were negative. Endocrinological exams showed hypopituitarism and diabetes insipidus. A pituitary MRI showed an enlargement of the pituitary stalk. A diagnosis of lymphocytic hypophysitis with aseptic meningitis was made after the exclusion of secondary hypophysitis. Lymphocytic hypophysitis with aseptic meningitis can mimic subacute meningoencephalitis.     

Granulomatous hypophysitis causing compression of the internal carotid arteries reversible with azathioprine and rituximab treatment

Introduction

Compression of the internal carotid artery (ICA) in the cavernous sinus area is a rare event and is mostly associated with pituitary adenomas and meningiomas. Other causes of ICA compression are less well known. We present a rare case of granulomatous hypophysitis causing compression of the ICA, which was treated successfully with immune-suppressive agents.

Methods

The electronic database MEDLINE (PubMed) was searched systematically and other cases with ICA compression were identified and analyzed.

Results

A female patient with a history of two previous transsphenoidal operations for suspected pituitary adenoma and post-operative complete pituitary insufficiency presented with severe headaches, nausea, fatigue, and diplopia. Pituitary MRI scan suggested relapse of the pituitary lesion with atypical bilateral infiltration of cavernous sinuses and compression of ICAs. After histological reevaluation of her previous pituitary operations, granulomatous hypophysitis was diagnosed. Treatment was started with high doses of prednisolone. With decreasing doses of prednisolone, symptoms recurred, and azathioprine was started, followed by administration of rituximab resulting in clinical recovery and regression of ICA compression. Literature analysis disclosed 36 case reports with ICA compression in the cavernous sinus region (12 pituitary adenoma, 6 meningioma, 7 hypophysitis, 5 other tumors, and 4 other etiologies). Two cases of hypophysitis recovered completely; five cases improved only partly.

Conclusion

In the case of ICA compression, clinical signs, onset of symptoms, radiological findings and pituitary insufficiencies should be thoroughly evaluated, and hypophysitis should be considered as a possible cause. In our patient, treatment with azathioprine and, finally, rituximab was successful.

     

A case of sparsely granulated growth hormone cell adenoma associated with lymphocytic hypophysitis.

Lymphocytic hypophysitis is in itself rare and usually occurs in the postpartum period or the last trimester of pregnancy. It has not been described in combination with a pituitary tumor. A twenty-two year old woman, who had never been pregnant, presented with a history of nine months amenorrhea and spontaneous galactorrhea. She was not taking any medication and had never used oral contraceptives. Physical examination was unremarkable except that whitish fluid could be expressed from both breasts. Her visual fields were normal. Her serum PRL levels was high at 105.7 micrograms/l and increased to 138.4 micrograms/l at 60 minutes in a triple bolus test. GH values were normal and there was no evidence of overproduction of other pituitary hormones. CT scan showed an intrasellar mass with suprasellar extension. A tumor was selectively removed transsphenoidally. Morphologic examination revealed a clinically silent sparsely granulated growth hormone cell adenoma with lymphocytic infiltration of the adjacent pituitary tissue. Postoperatively her menstrual periods resumed and she conceived despite a slightly elevated PRL level. Three months after an uneventful pregnancy and full term delivery her PRL level was 69.9 micrograms/l and increased to 102.2 micrograms/l at 60 min. Basal GH and cortisol levels were normal. She remains well without replacement fourteen months after delivery. This case is of interest because it is the first reported simultaneous occurrence of a pituitary adenoma and lymphocytic hypophysitis and also because the hypophysitis preceded her first pregnancy.