ABSTRACT
Autoimmune hypophysitis is a rare disorder characterized by autoimmune-mediated inflammation of the pituitary, among which lymphocytic hypophysitis is the most common type that occurs mainly in young women of reproductive ages. Its common clinical manifestation includes headache, visual dysfunction, hypopituitarism and etc. Typical magnetic resonance imaging shows diffuse homogenous enlargement of the pituitary gland with gadolinium enhancement and stalk thickening. Pituitary biopsy with histopathological examination is the gold standard for the diagnosis of autoimmune hypophysitis, but it should be performed with extra care in that transsphenoidal surgery is invasive and may cause pituitary insufficiency. Closely relating to clinical manifestation of the patients and the radiological features is advised for diagnosis. Good response to glucocorticoid therapy also help confirm the diagnosis. Full course of pharmacological glucocorticoid treatment can effectively control the clinical symptoms, reduce the mass effects, and promote the resumption of pituitary functions. Surgery is usually necessary for xanthomatous and granulomatous hypophysitis. Pathophysiological mechanism, diagnostic biomarkers, and need studying further.
ABSTRACT
Lymphocytic hypophysitis(LYH) is a rare autoimmune disease, which is more common in women and is closely associated with pregnancy. For patients with lymphocytic hypophysitis, the clinical manifestations mainly depend on the speed of disease progression, the extent of lesions, the severity and stage of disease, which can present as mass effect, decreased anterior pituitary hormone function, and central diabetes insipidus, etc. Due to physical changes in pituitary anatomy and hormone levels, as well as restriction of imaging examinations during pregnancy, it poses greater challenge to establish diagnosis in perinatal patients, which demands a comprehensive assessment of the patient′s clinical symptoms and biochemical tests. For treatment, patients with hypopituitarism during pregnancy should be given hormone replacement therapy. Moreover, patients need to adopt optimized tailored therapy according to gestational age and various clinical manifestations. At present, there is no report on the adverse effects of LYH on pregnancy outcome and re-pregnancy. This review discusses the diagnosis, treatment and prognosis of patients with LYH during pregnancy, and provides reference for the clinical management of this condition.
ABSTRACT
Objective: To investigate the clinical characteristics, imaging features and therapy of lymphocytic hypophysitis (LYH), and to clarify the importance of definitive diagnosis and hormonal therapy. Methods: The clinical materials of a LYH patient diagnosed by the post-operative pathology were collected; combined with the relative literatures, the clinical performance, imageological features and diagnosis and treatment methods were analyzed. Results: The patient was a 28 years old woman with intermittent headache for 2 months and went to hospital. There was no abnormal signs on physical examination. The cephalic MRI results showed the pituitary space-occupying lesions. Relevant examinations including thyroid color Dopplar ultrasound, pituitary thyroid hormone, Gonarelin test and so on were performed. The clinical manifestations, imaging examinations and laboratory examinations suggested pituitary adenomas. The patient underwent transsphenoidal saddle area surgery. The post-operative pathology suggested LYH. After operation, the headache symptom was improved obviously, but the hypopituitarism presented, and the patient was given hormone replacement therapy. It took 3 months for the patient to recover the normal levels of sexual hormones and menstruation. But intermittent headache and hypothyroidism still prestented. Conclusion: It is difficult to distinguish LYH from pituitary adenoma. Pathological biopsy is the gold standard for the diagnosis of LYH. Hypopituitarism may exist after surgical treatment, and long-term hormone replacement therapy should be initiated as needed.
ABSTRACT
To improve the differential diagnosis of sellar region mass,4 cases with sellar mass and misdiagnosed as lymphocytic hypophysitis (LYH) were reviewed retrospectively.The 4 patients (2 male and 2 female) aged 20-60 years old were all presented with symptoms of headache,polydipsia and polyuria.Biochemical studies confirmed the diagnoses of central diabetes insipidus and hypopituitarism.Head MRI scans showed LYH like image for all the cases,and,thus,high dose methylprednisolone pulse therapy (HDMPT) was applied to the patients.Their symptoms deteriorated and the sellar mass enlarged after a short period of partial improvement.Operations were performed in all the patients.Histology study showed craniopharyngioma with abscess,primary abscess,secondary hypophysitis caused by Wegener's granulomatosis,and germinoma with secondary hypophysitis,respectively.In conclusion,surgery or biopsy is necessary for those who presented with sellar region mass and was suspected to be with LYH,but with poor response or even worse after HDMPT.
ABSTRACT
Autoimmune hypophysitis is an inflammation of the pituitary gland resulting from autoimmune response, which can be classified as primary hypophysitis and secondary hypophysitis according to the etiology.The clinical presentation includes hypopituitarism and a pituitary mass.The diagnosis is based on clinical findings and radiology, while the pathology serves as the golden standard.The treatment is aimed at reducing the size of the pituitary mass, replacing the defective hormones and decreasing hyperprolactinemia.This review is about the epidemiology, pathogenesis, diagnosis, and treatment of lymphocytic hypophysitis, hypophysitis secondary to cytotoxic T-4 blockade, and IgG4-related hypophysitis.
ABSTRACT
La hipofisitis linfocitaria (HL) es un proceso autoinmune caracterizado por infiltración linfocitaria difusa de la glándula pituitaria con grados variables de destrucción. Los niveles elevados de prolactina y la caída de los estrógenos en el posparto son factores predisponentes para su desarrollo por lo que esta patología se presenta principalmente en mujeres jóvenes durante el embarazo o el puerperio. Se desconoce el antígeno implicado en su patogenia. En los últimos años se ha investigado la existencia de anticuerpos antipituitarios (APA) que permitan realizar un diagnóstico certero, de manera sencilla, con una sensibilidad y especificidad aceptables, que faciliten una adecuada interpretación clínica y un tratamiento acorde. Los autores declaran no poseer conflictos de interés.
Lymphocytic hypophysitis (HL) is an autoimmune condition characterized by diffuse lymphocytic infiltration of the pituitary gland with varying degrees of destruction. It occurs mainly in young women during pregnancy or the postpartum period secondary to elevated levels of prolactin (PRL) associated with a drop in estrogen levels in the postpartum period. The antigen involved in its pathogenesis is still unknown. In recent years, the existence of antipituitary antibodies (APA) has been investigated to allow a simple, accurate diagnosis, with acceptable sensitivity and specificity that enables an adequate clinical interpretation and treatment.
ABSTRACT
All three cases of recurrent lymphocytic hypophysitis were female, one of them being 70-yearold postmenopausal woman.Adenohypophysis, cavernous sinus, and optic chiasm were involved in case 1,hypothalamus and neurohypophysis were invaded in case 2, and adenohypophysis and hypophyseal stalk were involved in case 3.Relapse occured 4 months after operation in case 1, then high dosage methylprednisolone pulse therapy (HDMPT) brought about a remission lasting for 14 months before second relapse set in.Relapse occurred in case 2 at 16 months after prednisone treatment was discontinued, and case 3 recurred during the period of dose reduction.All three patients were then treated with glucocorticoid plus azathioprine for 16 weeks, and good response was seen in 3 cases.During follow-up, the symptoms were relieved and significant reduction of lesion was revealed by MRI in all thee patients, and the pituitary function was resumed in case1and 3.
ABSTRACT
Lymphocytic hypophysitis (LH) is an uncommon inflammatory disease of the hypophysis. It's female to male ratio of appearance is 9:1. Pregnant women are more affected during the third trimester of pregnancy or postpartum. Clinical and radiological presentation can simulate a hypophyseal adenoma. We report a nonpregnant 13 years old adolescent, with a trisomy 12p, with panhypopituitarism, diabetes insipidus and a selar tumor. It was necessary to differentiate between a germinoma and a LH. The latter was confirmed with the hypophyseal biopsy.
Subject(s)
Humans , Female , Pregnancy , Adolescent , Diabetes Insipidus/etiology , Pituitary Diseases/surgery , Pituitary Diseases/complications , Hypopituitarism/etiology , Trisomy , Diabetes Insipidus/surgery , Pituitary Diseases/diagnosis , Hypopituitarism/surgery , Inflammation , Lymphocytes/pathologyABSTRACT
Lymphocytic hypophysitis is a rare inflammatory disease of the pituitary gland that is one of the cause of hypopituitarism. The majority of cases occur in women during the peripartum period, and it is rare in men. Testicular feminization syndrome is a genetic disorder presenting a 46XY karyotype, but a normal female phenotype. We report a case of lymphocytic hypophysitis in a 23-year-old woman with testicular feminization syndrome.
Subject(s)
Female , Humans , Male , Young Adult , Androgen-Insensitivity Syndrome , Hypopituitarism , Karyotype , Peripartum Period , Phenotype , Pituitary GlandABSTRACT
Lymphocytic hypophysitis (LH) is characterized by lymphocytic pituitary infiltration, which occurs mostly during or after pregnancy. Its involvement in male is very rare. The authors report herein a LH mimicking pituitary macroadenoma clinically and radiologically in male patient who presented with visual disturbance and hypogonadism.
Subject(s)
Humans , Male , Pregnancy , Hypogonadism , Hypopituitarism , Pituitary NeoplasmsABSTRACT
Lymphocytic hypophysitis is a clinically rare disease, and it has been known to be an autoimmune disease which mainly affects pregnant women at the end of gestation or right after delivery. The authors experienced a case of lymphocytic hypophysitis in a 29-year-old pregnant woman with rapid progressing visual disturbance. Sella MRI showed a mass-like lesion of hypophysis and hypertrophy of pituitary stalk with evidences of hypopituitarism. Cesarean section was done and then TSA was performed. The pathologic diagnosis was lymphocytic hypophysitis. After TSA, visual acuity was improved and visual field defect was recovered. She was given thyroid hormone replacement therapy because of transient partial hypopituitarism for 6 months after surgery. One must consider the probability of lymphocytic hypophysitis, if there are alteration of visual acuity and visual field defect which aggravate rapidly during pregnancy due to mass effect, decreased serum hormonal levels shown in hypopituitarism and sella MRI findings of hypertrophy of pituitary stalk and enlargement of pituitary gland.
Subject(s)
Adult , Female , Humans , Pregnancy , Autoimmune Diseases , Cesarean Section , Diagnosis , Hormone Replacement Therapy , Hypertrophy , Hypopituitarism , Magnetic Resonance Imaging , Pituitary Gland , Pituitary Gland, Posterior , Pregnant Women , Rare Diseases , Thyroid Gland , Visual Acuity , Visual FieldsABSTRACT
We report here on a case of a 22-year-old girl with a suprasellar mass that was originally diagnosed as lymphocytic hypophysitis, but it eventually turned out to be a germinoma. She initially underwent partial tumor removal and the tissue diagnosis was suggestive of lymphocytic hypophysitis. 46 months later, she presented with a lateral visual field defect and decreased visual acuity of her right eye. The serum and cerebrospinal beta-hCG and alpha-fetoprotein levels were measured. The beta-hCG level was elevated in both, whereas the alpha-fetoprotein level was detectable in neither. The serum anti-pituitary antibody-1 level was negative. She was reoperated and the permanent section biopsies were compatible with pure germinoma. There was no evidence of meningeal metastasis on her whole spine MRI. She was treated with chemotherapy.
Subject(s)
Female , Humans , Young Adult , alpha-Fetoproteins , Biopsy , Diagnosis , Drug Therapy , Germinoma , Magnetic Resonance Imaging , Neoplasm Metastasis , Spine , Visual Acuity , Visual FieldsABSTRACT
PURPOSE: To report a rare case of lymphocytic hypophysitis in a 31-year-old woman who presented with gradually progressive bilateral visual loss during the third trimester of pregnancy. METHODS: Ophthalmologic examination revealed best corrected visual acuity of 0.02 OD and counting fingers at 20 cm OS. Pupil examination revealed no relative afferent pupillary defect in either eye and intraocular pressure was normal in both eyes. A visual field test revealed nearly total visual defect sparing superotemporal area OD and total defect OS. An MRI of the head was performed. RESULTS: At the time of transsphenoidal surgery following the patient's delivery, a frozen biopsy of the lesion revealed diffuse lymphocytic infilteration and fibrosis of the pituitary gland consistent with the diagnosis of lymphocytic hypophysitis. Postoperatively the patient was treated for hypopituitarism. At 5 weeks postoperative, her best corrected visual acuity was 1.0 OU, and visual field defects resolved in both eyes. On follow-up by telephone for postoperative 7 months, the patient remained visually asymptomatic. CONCLUSIONS: The clinical presentation of lymphocytic hypophysitis may mimic pituitary adenoma, lymphoma, germinoma, and histiocytosis. The diagnosis should be suspected in any pregnant or postpartum patient with an intrasellar or suprasellar mass.
Subject(s)
Adult , Female , Humans , Pregnancy , Biopsy , Diagnosis , Fibrosis , Fingers , Follow-Up Studies , Germinoma , Head , Histiocytosis , Hypopituitarism , Intraocular Pressure , Lymphoma , Magnetic Resonance Imaging , Pituitary Gland , Pituitary Neoplasms , Postpartum Period , Pregnancy Trimester, Third , Pupil , Pupil Disorders , Telephone , Visual Acuity , Visual Field Tests , Visual FieldsABSTRACT
Lymphocytic hypophysitis is a rare disorder characterized by focal or diffuse inflammatory infiltration and destruction of the pituitary gland. It has classically been described as affecting female patients during peripartum period and its pathogenesis has been attributed with autoimmunity. Pressure symptoms and visual impairment may arise from the sellar mass lesion, and mass effect, involvement of the pituitary stalk, and pituitary destruction may result in endocrine dysfunction. Rathke's cleft cyst is a cystic sellar or suprasellar lesion thought to originate from the remnants of Rathke's pouch. It is characteristically lined by a single layer of ciliated cuboidal or columnar epithelium. Although small asymptomatic Rathke's cleft cysts have been reported at autopsy in 2~26% of normal pituitary glands, symptomatic cysts are rare. In symptomatic Rathke's cleft cysts, the common presentations include headache, pituitary dysfunction, and visual loss caused by compression of the optic chiasm in the event of suprasellar extension. Recently we experienced a case of lymphocytic hypophysitis with Rathke's cleft cyst resected by transsphenoidal approach under the preoperative impression of pituitary adenoma. This coexistence is exceedingly rare event, and to our best knowledge, this may be the first case of lymphocytic hypophysitis with Rathke's cleft cyst in Korea.
Subject(s)
Female , Humans , Autoimmunity , Autopsy , Central Nervous System Cysts , Epithelium , Headache , Korea , Optic Chiasm , Peripartum Period , Pituitary Gland , Pituitary Neoplasms , Vision DisordersABSTRACT
Lymphocytic hypophysitis, a rare inflammatory disorder in the pituitary gland that is often associated with pregnancy, is caused by autoimmune destruction of the pituitary gland evidenced by diffuse lymphocytic infiltration in the pituitary gland. We report a case of lymphocytic hypophysitis in a 38-year-old woman presenting with aggravating headache and sudden visual loss during pregnancy, which was misdiagnosed as pituitary adenoma before neurosurgery. Magnetic resonance imaging, with enhancement, showed an expanding 2.5x2.0x1.7 cm sized sellar mass. The patient underwent transsphenoidal surgery with a preoperative diagnosis of pituitary macroadenoma. A histological examination revealed diffuse lymphocytes and fibrosis of adenohypophysis with atrophy, which are characteristic of lymphocytic hypophysitis. We report a case of lymphocytic hypophysitis occurred during pregnancy with a brief review of literatures.
Subject(s)
Adult , Female , Humans , Pregnancy , Atrophy , Diagnosis , Fibrosis , Headache , Lymphocytes , Magnetic Resonance Imaging , Neurosurgery , Pituitary Gland , Pituitary Gland, Anterior , Pituitary NeoplasmsABSTRACT
Lymphocytic hypophysitis is a rare but important cause of pituitary hypofunction which predominantly affects young women in the peripartum period. It is believed to be an autoimmune disorder with an association with other autoimmune disorders. Clinically, it presents most frequently with symptoms and signs attributable to pituitary hypofunction, headache, visual disturbance and amenorrhea. It is difficult to distinguish lymphocytic hypophysitis from a pituitary adenoma on preoperative imaging. So the diagnosis must be confirmed pathologically. The frozen section must be done for saving the risk of permanent hypopituitarism that would have attended a more radical debulking procedure. If lymphocytic hypophysitis is confirmed by frozen section, extensive surgical debulking may not be necessary. A 31-year-old woman presented during the third trimester of pregnancy in July of 2003 with headache, vomiting and gradually progressive bilateral visual loss. There was a large 8 shaped homogeneous sellar mass on MR images before delivery. The subtotal excision of tumor was done via transsphenoidal surgery three weeks after cesarean delivery. Visual field testing revealed marked resolution of her visual field defects in both eyes. We report a case of lymphocytic hypophysitis occurred in the third trimester of pregnancy with a brief review of literatures.
Subject(s)
Adult , Female , Humans , Pregnancy , Pregnancy , Amenorrhea , Diagnosis , Frozen Sections , Headache , Hypopituitarism , Peripartum Period , Pituitary Neoplasms , Pregnancy Trimester, Third , Visual Field Tests , Visual Fields , VomitingABSTRACT
Lymphocytic hypophysitis is a rare inflammatory disorder in the pituitary gland. The lesion is usually confined to the adenohypophysis. Although the involvement of the posterior pituitary gland or the stalk is rare, such patients with diabetes insipidus have been reported. Surgery has been used to make the definitive diagnosis. Recent studies suggest, however, that the pathologic diagnosis may not be necessary always. We reported a case of Lymphocytic hypophysitis managed by methylprednisolone pulse therapy. A 50-year-old premenopausal woman with Lymphocytic hypophysitis and diabetes insipidus was treated with methylprednisolone pulse therapy. Her adenopituitary lesion disappeared and the diabetes insipidus resolved. The optimal management for patients with lymphocytic hypophysitis may be the high index of the suspicion prior to the extensive surgical resection. In addition, methylprednisolone pulse therapy may improve the clinical and MRI findings.
Subject(s)
Female , Humans , Middle Aged , Anti-Inflammatory Agents/administration & dosage , Diabetes Insipidus/drug therapy , Lymphocytosis/complications , Methylprednisolone/administration & dosage , Pituitary Diseases/complications , Pulse Therapy, DrugABSTRACT
Lymphocytic hypophysitis(LH) is a rare but increasingly recognized autoimmune endocrine condition that causes partial or total hypopituitarism and is often associated with peripartum young women.We here report a 28-year-old patient who had a spontaneous and uneventful pregnancy following LH that had been treated with transspenoidal surgery and followed by anti-inflammatory agent.The woman failed to lactate and developed frontal headaches 3 months after normal delivery of her first child 3 years ago.Lab test showed the reduced concentrations of thyroxine,estradial and cortisol,suggesting hypopituitarism.Magnetic resonance imaging of the brain with contrast was performed and showed a uniformly enhancing pituitary mass with elevated optic chiasm.She underwent transsphenoidal surgery and histological examination of the resected specimen was consistent with lymphocytic hypophysitis.Anti-inflamation was started with prednisolone 40 mg per day because of a recurrence of headache that had completely recovered after surgery and regularly withdrawn to a long term maintenance dose of 10 mg per day.Physiological thyroxine replacement therapy was maintained.Her menstruation was restored without sex hormone replacement after 3 months.Three years after surgery,she got pregnant spontaneously and had normal breastfeeding after delivery.LH did not recur during this peripartum.
ABSTRACT
The authors report two cases of lymphocytic hypophsitis: a 38-year-old male patient with headache and diabetes insipidus and in a 64-year-old female patient with headache. Magnetic resonance(MR) image of the patients showed mass-like lesions in the hypophysis as well as loss of high signal on T1-weighted MR images. There was evidence of hypopituitarism. Both patients underwent transsphenoidal approaches for histological diagnosis, which was compatable with lymphocytic hypophysitis. When MR image reveals a characteristic pituitary in the hypophysis lesion with relevant clinical symptoms in a patient, one should include "lymphocytic hypophysitis" as one of the diagnostic possibilities, since the therapeutic options are different from those of pituitary tumors.
Subject(s)
Adult , Female , Humans , Male , Middle Aged , Diabetes Insipidus , Diabetes Insipidus, Neurogenic , Diagnosis , Headache , Hypopituitarism , Pituitary Gland , Pituitary Gland, Posterior , Pituitary NeoplasmsABSTRACT
Lymphocytic hypophysitis, an inflammatory disease of the pituitary gland that is often associated with pregnancy, is caused by autoimmune destruction of the pituitary gland evidenced by diffuse inflammatory cell infiltration of the pituitary gland and some kinds of detectable autoantibodies. We report a case of lymphocytic hypophysitis in a 31-year-old woman presenting with severe hypoglycemia and hyponatremia after delivery. Hormonal study revealed panhypopituitarism and magnetic resonance imaging with enhancement showed the bulging contour of the right side pituitary gland with an ill-defined mass-like lesion and nodular thickening of the stalk. The patient's symptoms and biochemical data improved greatly with replacement of L-thyroxine and glucocorticoid. Partial recovery of panhypopituitarism was also seen. The follow-up tests revealed dramatic resolution of the pituitary lesion.