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European Journal of Endocrinology Feb 2023The aim of the study is to assess the distinguishing features of pregnancy-related hypophysitis (PR-Hy) compared to non-pregnancy autoimmune hypophysitis and to evaluate...
OBJECTIVE
The aim of the study is to assess the distinguishing features of pregnancy-related hypophysitis (PR-Hy) compared to non-pregnancy autoimmune hypophysitis and to evaluate the changing therapeutic approaches and outcomes in PR-Hy over time.
DESIGN
Retrospective analysis of all published cases with PR-Hy and 6 own cases.
METHODS
A PubMed search was performed and abstracts screened for publications with information on cases with PR-Hy from which full-text review was performed. Clinical features, diagnostic findings, and outcome in relation to treatment modalities in PR-Hy were assessed.
RESULTS
One hundred and forty-eight cases with PR-Hy were identified. PR-Hy was significantly delimited from non-PR-Hy by the frequent occurrence of the chiasmal syndrome (50% vs 13%, P < .0001), higher rate of intrasellar origin (94% vs 74%, P = .0005), lower rate of pituitary stalk involvement (39% vs 86%, P < .0001), and low rate of diabetes insipidus (12% vs 54%, P < .0001). The role of surgery in PR-Hy decreased over time while noninvasive treatment modalities increased. The recurrence rate after high-dose glucocorticoid therapy (33%) was high and exceeded that of surgery (2%) and conservative management (2%). In contrast to initial reports on PR-Hy, recent literature regarding outcome of mother's and child's health was positive. The frequency of spontaneous preterm delivery was not increased. Recurrent PR-Hy in a subsequent pregnancy was reported in only two females.
CONCLUSION
PR-Hy has distinct features that delineate the disorder from non-PR-Hy. With increasing experience in diagnosis, availability of adequate replacement therapy, and improved treatment modalities, PR-Hy has lost its threat and the outcome is encouraging.
Topics: Female; Child; Infant, Newborn; Humans; Pituitary Diseases; Retrospective Studies; Pituitary Gland; Diabetes Insipidus; Hypophysitis; Autoimmune Hypophysitis; Hypopituitarism; Magnetic Resonance Imaging
PubMed: 36655394
DOI: 10.1093/ejendo/lvad003 -
Neuroimaging Clinics of North America Feb 2022Chemoradiation for head and neck cancer is associated with a variety of early and late complications. Toxicities may affect the aero-digestive tract (mucositis, salivary... (Review)
Review
Chemoradiation for head and neck cancer is associated with a variety of early and late complications. Toxicities may affect the aero-digestive tract (mucositis, salivary gland injury), regional osseous and cartilaginous structures (osteoradionecrosis (ORN) and chondronecrosis), vasculature (progressive radiation vasculopathy and carotid blow out syndromes), and neural structures (optic neuritis, myelitis, and brain injury). These may be difficult to distinguish from tumor recurrence on imaging, and may necessitate the use of advanced MRI and molecular imaging techniques to reach the correct diagnosis. Secondary radiation-induced malignancies include thyroid cancer and a variety of sarcomas that may manifest several years after treatment. Checkpoint inhibitors can cause a variety of adverse immune events, including autoimmune hypophysitis and encephalitis.
Topics: Head and Neck Neoplasms; Humans; Magnetic Resonance Imaging; Neoplasm Recurrence, Local; Osteoradionecrosis
PubMed: 34809846
DOI: 10.1016/j.nic.2021.08.012 -
Best Practice & Research. Clinical... May 2022Pituitary autoimmunity is one of the principal causes of hypopituitarism. Additionally, hypophysitis is one of the immune-related adverse events associated with... (Review)
Review
Pituitary autoimmunity is one of the principal causes of hypopituitarism. Additionally, hypophysitis is one of the immune-related adverse events associated with immunotherapy. Recent case-oriented research has revealed a novel type of autoimmune hypophysitis, anti-PIT-1 hypophysitis, related to isolated adrenocorticotropic hormone (ACTH) deficiency and immune checkpoint inhibitor-related hypophysitis, as a form of paraneoplastic syndrome. Under these conditions, the ectopic expression of pituitary antigens present in tumors evokes a breakdown of immune tolerance, resulting in the production of autoantibodies and autoreactive cytotoxic T cells that specifically harm pituitary cells. Consequently, an innovative clinical entity of paraneoplastic autoimmune hypophysitis has been purported. This novel concept and its underlying mechanisms provide clues for understanding the pathogenesis of autoimmune pituitary diseases and can be applied to other autoimmune diseases. This review discusses the etiology of paraneoplastic autoimmune hypophysitis and its future.
Topics: Adrenocorticotropic Hormone; Autoantibodies; Autoimmune Diseases; Autoimmune Hypophysitis; Endocrine System Diseases; Genetic Diseases, Inborn; Humans; Hypoglycemia; Hypophysitis; Pituitary Diseases
PubMed: 34876362
DOI: 10.1016/j.beem.2021.101601 -
Handbook of Clinical Neurology 2021Hypothalamitis is a rare inflammatory disorder involving the hypothalamus and classified as primary, or isolated, and secondary hypothalamitis. Secondary hypothalamitis... (Review)
Review
Hypothalamitis is a rare inflammatory disorder involving the hypothalamus and classified as primary, or isolated, and secondary hypothalamitis. Secondary hypothalamitis although very rare is more common than the primary one and may occur in patients affected by autoimmune diseases such as autoimmune hypophysitis, systemic autoimmune diseases, infective diseases in patients affected by immune-deficit, paraneoplastic encephalitis, or in patients treated with immune checkpoint inhibitors. In accordance with the rarity of this disease, diagnosis and management of hypothalamitis prove to be challenging. The diagnosis requires a high index of clinical suspicion. The main symptoms may be: various degrees of hypopituitarism, neuropsychiatric and behavioral disorders, and disturbances of autonomic and metabolic regulation. Magnetic resonance images play a crucial role in the diagnosis of hypothalamitis and in the exclusion of a neoplastic lesion. Therapeutic management should be oriented according to the disease etiology. In most cases, after ruling out infective hypothalamitis, the mainstay of therapy consists of immunosuppressive treatment. Great attention should be paid to hormonal replacement therapy, if partial or total hypopituitarism is present, in particular in patients affected by diabetes insipidus, central hypoadrenalism and hypothyroidism. According to the complexity of this disease, a multidisciplinary approach is strongly advocated to reach an early diagnosis and an integrated therapy.
Topics: Atrophy; Autoimmune Hypophysitis; Humans; Hypopituitarism; Magnetic Resonance Imaging; Pituitary Diseases; Pituitary Gland
PubMed: 34238454
DOI: 10.1016/B978-0-12-820683-6.00011-7 -
Best Practice & Research. Clinical... May 2022Immune checkpoint inhibitors (ICIs) have been recently proposed as a strategy for treating anti-malignant neoplasms. However, this treatment leads to immune-related... (Review)
Review
Immune checkpoint inhibitors (ICIs) have been recently proposed as a strategy for treating anti-malignant neoplasms. However, this treatment leads to immune-related adverse events (irAEs) such as autoimmune endocrinopathy. Early diagnosis and appropriate treatment of ICI-related hypophysitis are essential as it can manifest as a life-threatening condition due to an adrenal crisis. In this review, we summarize the pathogenesis, risk factors, diagnostic processes, clinical characteristics, and its current management. In particular, we discuss the different aspects of anti-CTLA-4 antibody-related and anti-PD-1/anti-PD-L1 antibody-related hypophysitis. We also propose key points for early detection and diagnosis by identifying the target group that should be monitored more carefully. Specific methods of hormone replacement therapy have also been described. We hope that this review will lead to a better understanding and management of this rare but serious condition during cancer treatment and further elucidate the pathophysiology of pituitary autoimmunity.
Topics: Endocrine System Diseases; Humans; Hypophysitis; Immune Checkpoint Inhibitors; Immunotherapy; Neoplasms
PubMed: 35562229
DOI: 10.1016/j.beem.2022.101668 -
Expert Review of Endocrinology &... 2023Immune-checkpoint inhibitor therapy modulates the response of the immune system acting against cancer. Two pathways impacted by this kind of treatment are the CTLA4 and... (Review)
Review
INTRODUCTION
Immune-checkpoint inhibitor therapy modulates the response of the immune system acting against cancer. Two pathways impacted by this kind of treatment are the CTLA4 and the PD-1/PD-L1 pathways. ICI therapy can trigger autoimmune adverse effects, known as immune-related Adverse Events (irAEs).
AREAS COVERED
This review focuses on irAEs which affect the endocrine system. This review elucidates the pathways used by these drugs with a focus on the hypothetical pathogenesis at their basis. In fact, the pathophysiology of irAEs concerns the possibility of an interaction between cellular autoimmunity, humoral immunity, cytokines, chemokines, and genetics. The endocrine irAEs examined are thyroid dysfunctions, immune related-hypophysitis, diabetes, peripheral adrenal insufficiency, and hypoparathyroidism.
EXPERT OPINION
There is still much to investigate in endocrine irAES of checkpoint inhibitors. In the future, checkpoint inhibitors will be increasingly utilized therapies, and therefore it is crucial to find the proper diagnostic-therapeutic program for irAEs, especially as endocrine irAEs are nonreversible and require lifelong replacement therapies.
Topics: Humans; Immune Checkpoint Inhibitors; Antineoplastic Agents, Immunological; Immunotherapy; Endocrine System; Endocrine System Diseases; Drug-Related Side Effects and Adverse Reactions
PubMed: 37682107
DOI: 10.1080/17446651.2023.2256841 -
Trends in Endocrinology and Metabolism:... Sep 2019Hypophysitis is a rare and potentially life-threatening disease, characterized by an elevated risk of complications, such as occurrence of acute central hypoadrenalism,... (Review)
Review
Hypophysitis is a rare and potentially life-threatening disease, characterized by an elevated risk of complications, such as occurrence of acute central hypoadrenalism, persistent hypopituitarism, or extension of the inflammatory process to the neighboring neurological structures. In recent years, a large number of patients have been described as being affected by hypophysitis, due to the increased administration of immuno-chemotherapies. At the present time, the heterogeneous nature of hypophysitis diagnostic criteria and of the treatment protocols makes the management of affected patients difficult. We review the current data and evidence on primary and secondary hypophysitis, in order to suggest a diagnostic and therapeutic protocol that should be focused on a multidisciplinary approach, for reaching a prompt diagnosis and an appropriate and safe treatment.
Topics: Hormone Replacement Therapy; Humans; Hypophysitis; Immunosuppressive Agents; Pituitary Gland
PubMed: 31345630
DOI: 10.1016/j.tem.2019.06.004 -
Clinical Rheumatology Feb 2020Granulomatosis with polyangiitis (GPA) is a necrotizing granulomatous vasculitis of small vessels that affect the pituitary gland in less than 1% of cases being... (Review)
Review
Granulomatosis with polyangiitis (GPA) is a necrotizing granulomatous vasculitis of small vessels that affect the pituitary gland in less than 1% of cases being exceptionally rare. To describe the clinical, biochemical, radiological findings, treatment, and outcomes of 4 patients with GPA-related hypophysitis. A systematic review of published cases with the same diagnosis is presented as well. A cross-sectional case series of patients with hypophysitis due to GPA from 1981 to 2018 at a third level specialty center. Literature review was performed searching in seven different digital databases for terms "granulomatosis with polyangiitis" and "pituitary gland" or "hypophysitis," including in the analysis all published cases between 1950 and 2019 with a minimum follow-up of 6 months. We found 197 patients with GPA in our institution of whom 4 patients (2.0%) had pituitary involvement. Clinical characteristics and outcomes are described. We also reviewed 7 case series, and 36 case reports describing pituitary dysfunction related to GPA from 1953 to 2019, including the clinical picture of an additional 74 patients. Pituitary dysfunction due to GPA is rare. Treatment is targeted to control systemic manifestations; nevertheless, the outcome of the pituitary function is poor. Central diabetes insipidus, particularly in younger women with other systemic features, should raise suspicion of GPA.Key Points• Involvement of the pituitary gland is an uncommon manifestation in GPA patients. The presence of central diabetes insipidus in the setting of systemic symptoms should prompt its suspicion.• In patients with pituitary involvement due to GPA, affection of other endocrine glands is rare, neither concomitant nor in different times during the disease course. This may arise the hypothesis of a local or regional pathogenesis affection of the gland.• There is no consensus on the best therapy strategy for GPA hypophysitis. Although the use of glucocorticoids with CYC is the most common drug combination, no differences in the outcome of the pituitary function and GPA disease course are seen with other immunosuppressants.• Poor prognosis regarding pituitary function is expected due to possible permanent pituitary tissue damage that results in the need of permanent hormonal replacement.
Topics: Antidiuretic Agents; Autoimmune Hypophysitis; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Female; Glucocorticoids; Granulomatosis with Polyangiitis; Humans; Hyperprolactinemia; Hypopituitarism; Immunosuppressive Agents; Magnetic Resonance Imaging; Male; Middle Aged
PubMed: 31446541
DOI: 10.1007/s10067-019-04735-7 -
Endocrinology and Metabolism Clinics of... Sep 2019Diagnosis of lymphocytic hypophysitis occurring in the peripartum period is based on clinical and neuroradiological data and does not require a biopsy. Its course is... (Review)
Review
Diagnosis of lymphocytic hypophysitis occurring in the peripartum period is based on clinical and neuroradiological data and does not require a biopsy. Its course is generally spontaneously favorable in terms of mass effect but may require the administration of corticosteroids or even transsphenoidal resection. The course of pituitary deficiencies is highly variable; some cases recover over time, whereas others persist indefinitely. Sheehan syndrome is very rare in developed countries. Because agalactia and amenorrhea are often neglected, the diagnosis is generally delayed. Diabetes insipidus occurring in late pregnancy is caused by the increased placental production of vasopressinase and disappears after delivery.
Topics: Diabetes Insipidus; Female; Humans; Hypopituitarism; Pituitary Diseases; Pregnancy; Pregnancy Complications
PubMed: 31345525
DOI: 10.1016/j.ecl.2019.05.005 -
Radiologic Clinics of North America May 2021Neurologic injury arises from treatment of central nervous system malignancies as result of direct toxic effects or indirect vascular, autoimmune, or infectious effects.... (Review)
Review
Neurologic injury arises from treatment of central nervous system malignancies as result of direct toxic effects or indirect vascular, autoimmune, or infectious effects. Multimodality treatment may potentiate both therapeutic and toxic effects. Symptoms range from mild to severe and permanent. Injuries can be immediate or delayed. Many early complications are nonspecific. Other early and delayed neurologic injuries, such as posterior reversible encephalopathy syndrome, dural sinus thrombosis, infarctions, myelopathy, leukoencephalopathy, and hypophysitis, have unique imaging features. This article reviews treatment options for neurologic malignancies and common and uncommon neurologic injuries that can result from treatment, focusing on radiologic features.
Topics: Adult; Brain; Brain Neoplasms; Chemotherapy-Related Cognitive Impairment; Child; Combined Modality Therapy; Diagnostic Imaging; Humans; Immunotherapy; Neurocognitive Disorders; Radiation Injuries
PubMed: 33926687
DOI: 10.1016/j.rcl.2021.01.008