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Prolactin and other Lactogenic Hormones
Published in Istvan Berczi, Pituitary Function and Immunity, 2019
An autoimmune disease of the pituitary gland, lymphocytic hypophysitis, occurs most frequently in young women in association with pregnancy and lactation. It may mimic prolactin-producing pituitary adenomas clinically and may be accompanied by mild hyperprolactinemia. Hypersecretion of other pituitary hormones has not been reported. Histologically the disease is characterized by a massive lymphocytic infiltration and a widespread adenohypophysial cell damage. Most of the infiltrating cells are lymphocytes, although plasma cells and macrophages are also present. Occasionally multinucleated giant cells and epithelioid cells can be detected, which may form non-causeating granulomas. Sometimes lymphoid follicles with germinal centers can be recognized. Electron microscopically the interdigitation of activated lymphocytes with adenohypophysial cells has been observed. No immune complex deposits could be found, and vascular impairment or thrombus formation was not noticed. In patients with lymphocytic hypophysitis, hypopituitarism occurs, which is not restricted to the secretion of PRL.57
Neurological Manifestations of Medical Disorders
Published in John W. Scadding, Nicholas A. Losseff, Clinical Neurology, 2011
David and John Scadding Werring, John Scadding
Pregnancy causes the pituitary gland to enlarge. Sheehan syndrome is a rare condition of pituitary infarction, usually caused by postpartum hemorrhage and systemic hypotension, or by the vascular demands of an enlarging pituitary gland exceeding the available vascular supply. Infarction may transform into haemorrhage. Infarction or haemorrhage can result in acute pituitary insufficiency and shock, hence the term pituitary apoplexy. Lymphocytic hypophysitis is thought to be of autoimmune origin and is usually self-limiting. Treatment with corticosteroids is indicated if there is visual impairment due to chiasmal compression.
Neuroendocrine disease
Published in Philip E. Harris, Pierre-Marc G. Bouloux, Endocrinology in Clinical Practice, 2014
Autoimmune (lymphocytic) hypophysitis44 (Figure 1.14) is a chronic inflammatory condition that affects the anterior pituitary, posterior pituitary, or both. It has an estimated prevalence of about 9 per million. Isolated involvement of the anterior pituitary has a striking predilection for young females (female:male ratio 6:1), particularly in late pregnancy or in the postpartum period. Involvement of both the anterior and the posterior pituitary occurs about twice as commonly in females, whereas involvement of the pituitary stalk and posterior pituitary (infundibular neurohypophysitis) appears to affect males and females equally. It typically presents with headache, suprasellar pituitary mass, and hypopituitarism. Unlike pituitary adenomas, ACTH secretion is usually affected first, followed by TSH, LH, FSH, and GH and prolactin. Visual field defects are common. Diabetes insipidus occurs with involvement of the posterior pituitary and may be associated with thickening of the pituitary stalk on MRI. Failure of lactation may occur in the postpartum period. Less commonly, hyperprolactinemia may occur, as with other large pituitary tumors, presumably at least in part, due to stalk compression. An important differential diagnosis in the postpartum period is Sheehan’s syndrome42 and granulomatous conditions such as histiocytosis X and sarcoidosis (Figure 1.15). A definitive diagnosis can only be made by biopsy. If the diagnosis is considered to be a possibility and the patient is not at risk from compressive symptoms, then a conservative approach should be adopted, as in some patients the endocrine dysfunction is only transient. High-dose glucocorticoids may be considered in some cases, if interventional therapy is deemed to be necessary.
Complete and Uneventful Recovery in a Case of Lymphocytic Hypophysitis Causing a Third Nerve Palsy
Published in Neuro-Ophthalmology, 2023
Jamie M. Nord, Paras P. Shah, Rashmi Verma
Lymphocytic hypophysitis (LH) was first described in 1962.1 It is a neuroendocrine disorder characterised by autoimmune inflammation of the pituitary gland with pituitary dysfunction. The disease is more common in women and typically manifests during pregnancy or the postpartum period. In fact, 60% of women are diagnosed in relation to pregnancy, likely due to altered pituitary perfusion with increased accessibility to the immune system.2 Additionally, 70–80% of patients with LH have another autoimmune disease, with the most common being autoimmune thyroid disease.3 Patients typically present with symptoms due to mass effect, adenohypophyseal hypofunction, hyperprolactinaemia, and possible neurohypophysis involvement.4 Although presumptive diagnoses can sometimes be made based on history and imaging, a definitive diagnosis requires biopsy.5
The autoimmune basis of hypopituitarism in traumatic brain injury: fiction or reality?
Published in British Journal of Neurosurgery, 2019
Viraat Harsh, Sukriti Jha, Hitesh Kumar, Anil Kumar
APAs and AHAs are far from being considered strong useful prognosticators of PTHP due to lack of larger studies, however, the idea does not seem to be far-fetched. It is difficult to detect APAs and AHAs due to the uncertain character of their corresponding antigens. Therefore, antibody quantification and interactions to target epitopes are still far from being clinically relevant or accessible. Some studies have demonstrated disappearance of APAs and spontaneous partial or total pituitary recovery during the course of lymphocytic hypophysitis. Hence, longitudinal studies with more patients are required to investigate the apparent disappearance of APAs over time and to assess the predictive value of AHAs as markers of PTHP and possible clinical outcomes. Furthermore, at the time of writing, lack of standardized and commercially viable assays restrains antibody detection as a useful diagnostic tool. Variable results on APA positivity may be due to small cohorts studied or discrepancy related to IF assays used.4 Specificity and sensitivity of hormonal assays need to be established and the methods standardized. Lastly, PTHP prevention is theoretically possible with anti-autoantibodies in the future but that is as yet some way off.
Granulomatous hypophysitis in a postpartum patient: A case report
Published in Cogent Medicine, 2018
Alexey Youssef, Rahaf Ali, Maya Marouf
Hypophysitis is a rare inflammatory disorder of the pituitary gland, with an approximate incidence of one case per 9 million people per year. Based on pathological findings, it is classified into lymphocytic, granulomatous and xanthomatous hypophysitis. While lymphocytic hypophysitis LH is the most common (397 reported cases in the literature), granulomatous hypophysitis PGH comes in the second place with 85 proven cases reported in the literature (Bhansali, Velayutham, Radotra, & Pathak, 2004; Hunn et al., 2014; Joneja et al., 2016; Su, Zhang, Yue, & Zhang, 2011). Furthermore, GRH is classified into primary and secondary disorders. Secondary causes include systemic inflammatory disorders such as sarcoidosis, Wegener granulomatosis, tuberculosis and Langerhans cell histiocytosis. Unlike LH, PGH is not associated with pregnancy nor with autoimmune diseases. However, both are similar in that they have a female predilection (Hunn et al., 2014).