Autoimmune Hypophysitis

Immune-mediated inflammation of the PITUITARY GLAND often associated with other autoimmune diseases (e.g., HASHIMOTO DISEASE; GRAVES DISEASE; and ADDISON DISEASE).

Also Known As:

Anti-PIT-1 Antibody Syndrome; Hypophysitis, Autoimmune; Hypophysitis, Lymphocytic; Idiopathic Granulomatous Hypophysitis; IgG4-Related Hypophysitis; Lymphocytic Adenohypophysitis; Lymphocytic Hypophysitis; Lymphocytic Infundibuloneurohypophysitis; Lymphocytic Panhypophysitis; Lymphoid Hypophysitis; Adenohypophysitides, Lymphocytic; Adenohypophysitis, Lymphocytic; Anti PIT 1 Antibody Syndrome; Anti-PIT-1 Antibody Syndromes; Antibody Syndrome, Anti-PIT-1; Antibody Syndromes, Anti-PIT-1; Autoimmune Hypophysitides; Granulomatous Hypophysitides, Idiopathic; Granulomatous Hypophysitis, Idiopathic; Hypophysitides, Autoimmune; Hypophysitides, Idiopathic Granulomatous; Hypophysitides, IgG4-Related; Hypophysitides, Lymphocytic; Hypophysitides, Lymphoid; Hypophysitis, Idiopathic Granulomatous; Hypophysitis, IgG4-Related; Hypophysitis, Lymphoid; Idiopathic Granulomatous Hypophysitides; IgG4 Related Hypophysitis; IgG4-Related Hypophysitides; Infundibuloneurohypophysitides, Lymphocytic; Infundibuloneurohypophysitis, Lymphocytic; Lymphocytic Adenohypophysitides; Lymphocytic Hypophysitides; Lymphocytic Infundibuloneurohypophysitides; Lymphocytic Panhypophysitides; Lymphoid Hypophysitides; Panhypophysitides, Lymphocytic; Panhypophysitis, Lymphocytic; Syndrome, Anti-PIT-1 Antibody; Syndromes, Anti-PIT-1 Antibody

Networked: 258 relevant articles (14 outcomes, 14 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Hypopituitarism (Sheehan's Syndrome)
2.	Immunoglobulin G4-Related Disease
3.	Idiopathic Thrombocytopenic Purpura (Thrombocytopenic Purpura, Autoimmune)
4.	Alcoholic Fatty Liver
5.	Chronic Disease (Chronic Diseases)

Experts

1.	Caturegli, Patrizio: 15 articles (01/2021 - 06/2007)
2.	Takahashi, Yutaka: 11 articles (01/2022 - 01/2014)
3.	Bando, Hironori: 8 articles (01/2022 - 01/2014)
4.	Iguchi, Genzo: 7 articles (11/2019 - 01/2014)
5.	Sugimura, Yoshihisa: 6 articles (01/2022 - 07/2015)
6.	Lupi, Isabella: 6 articles (08/2017 - 06/2007)
7.	Fukuoka, Hidenori: 5 articles (11/2019 - 01/2014)
8.	Matsumoto, Ryusaku: 5 articles (11/2019 - 01/2014)
9.	Nishizawa, Hitoshi: 5 articles (11/2019 - 01/2014)
10.	Suda, Kentaro: 5 articles (11/2019 - 01/2014)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Autoimmune Hypophysitis:

1.	GlucocorticoidsIBA 02/01/2023 - "High-dose glucocorticoids are associated with improved recovery of deficits in primary autoimmune hypophysitis (PAH), but optimal dosing, route, and duration are unclear. " 07/29/2020 - "First-line treatment of lymphocytic hypophysitis is high-dose glucocorticoids. " 01/01/2010 - "Lymphocytic infundibuloneurohypophysitis: long-term follow-up of a case cured with glucocorticoid." 01/15/2022 - "Poor response to glucocorticoids can be used as an exclusion criterion in the clinical diagnosis of IgG4-related hypophysitis." 01/01/2022 - "The role of glucocorticoids in primary autoimmune hypophysitis (PAH) has been fraught with variability in regimens, leading to inconsistent outcomes in terms of anterior pituitary (AP) hormonal recovery. "
2.	Azathioprine (Imuran)FDA LinkGeneric 11/01/2003 - "This is the first evidence of the efficacy of azathioprine treatment in a patient with lymphocytic hypophysitis." 11/01/2003 - "Lymphocytic hypophysitis successfully treated with azathioprine: first case report." 01/01/2011 - "The objective of the study was to evaluate the efficacy of glucocorticoid treatment combined with azathioprine for treating three cases of recurrent autoimmune lymphocytic hypophysitis encountered within a two-year period. " 06/01/2014 - "This is the first case of IgG4-related hypophysitis demonstrating improvement with azathioprine, where corticosteroids were initially tried but ceased due to concern regarding enlargement of the pituitary infiltrate. " 09/01/2011 - "Review of the literature identified 30 articles, among case reports and case series, reporting a total of 44 cases of autoimmune hypophysitis treated with glucocorticoids and/or azathioprine. "
3.	Adrenal Cortex Hormones (Corticosteroids)IBA 09/01/2021 - "Complete Resolution of the Pituitary Mass Lesion and Improvement of Pituitary Function with Corticosteroid in Autoimmune Hypophysitis: A Case Report." 03/01/2018 - "Corticosteroids in the management of lymphocytic hypophysitis: Case series." 09/01/1998 - "We think lymphocytic infundibuloneurohypophysitis can be diagnosed without histological examinations and can be treated conservatively without corticosteroid treatment. " 09/01/1994 - "We report the first case of pseudotumoral lymphocytic hypophysitis successfully treated by corticosteroids without surgery. " 09/01/1994 - "Pseudotumoral lymphocytic hypophysitis successfully treated by corticosteroid alone: first case report."
4.	Rituximab (Mabthera)FDA Link 12/15/2021 - "This case illustrates that rituximab might be an effective alternative treatment in B-cell predominant autoimmune hypophysitis." 12/15/2021 - "Recurrent autoimmune hypophysitis treated with rituximab: a case report." 06/01/2020 - "A Case Series of Patients with Isolated IgG4-related Hypophysitis Treated with Rituximab." 01/01/2018 - "Rituximab treatment for isolated IgG4-related hypophysitis in a teenage female." 06/01/2017 - "Rituximab was used to treat recurrent IgG4-related hypophysitis with ophthalmopathy as the initial presentation: A case report and literature review."
5.	Immunoglobulin G (IgG)IBA 01/01/2014 - "This is a typical case of IgG4-related hypophysitis in which PSL causes marked improvement of pituitary mass and pituitary function along with the reduction of serum IgG4 levels. " 04/01/2020 - "Among pathology specimens available for IgG and IgG4 immunostaining (n=10), none fulfilled the criteria for IgG4-related hypophysitis. " 06/01/2018 - "A case series of atypical features of patients with biopsy-proven isolated IgG4-related hypophysitis and normal serum IgG4 levels." 01/01/2017 - "Based on various findings including the swelling of the pituitary gland, increase of serum IgG4 level and abundant IgG4-positive plasma cell infiltration in immunostaining of the pituitary gland, we diagnosed this subject as IgG4-related hypophysitis. " 10/01/2015 - "Pituitary biopsy specimen showed IgG4-positive cells, with a 43% IgG4(+)/IgG(+) ratio, which met the criteria for IgG4-related hypophysitis. "
6.	SteroidsIBA 08/01/2001 - "While the natural history of lymphocytic hypophysitis remains elusive and its management is not well established, our report shows that spontaneous resolution may occur with steroid supplementation even in men." 07/01/1991 - "In patients with suspected lymphocytic hypophysitis and a pituitary mass, a trial of steroids may be therapeutic." 01/01/2021 - "She was diagnosed with postpartum lymphocytic adenohypophysitis and treated with steroids. " 06/01/2018 - "During follow-up, the lesion of lymphocytic hypophysitis showed spontaneous shrinkage in the absence of steroid treatment after RCC resection. " 04/01/2015 - "LYMPHOCYTIC HYPOPHYSITIS: A RARE AUTOIMMUNE DISORDER PRESENT IN A LARGE SUPRA SELLAR MASS HAVING COMPLETE RESOLUTION WITH STEROIDS."
7.	Growth Hormone (Somatotropin)IBA 03/01/2022 - "We here presented a rare case of a child with lymphocytic infundibuloneurohypophysitis who developed NASH and showed substantial improvement in liver function after growth hormone treatment." 01/01/2014 - "Autoimmune hypophysitis can result in growth hormone deficiency (GHD). " 03/01/2022 - "A 17-year-old boy with lymphocytic infundibuloneurohypophysitis who developed non-alcoholic steatohepatitis effectively treated with growth hormone." 09/01/1991 - "A case of sparsely granulated growth hormone cell adenoma associated with lymphocytic hypophysitis." 03/01/1999 - "The index patient's phenotype was severe, manifested by classic features of the illness (adrenal insufficiency, hypoparathyroidism, candidiasis, and keratoconjunctivitis with alopecia universalis), as well as by severe exocrine pancreatic insufficiency, diabetes mellitus, hepatic inflammation, growth hormone (GH) deficiency due to lymphocytic hypophysitis, and primary ovarian failure. "
8.	Prednisone (Sone)FDA LinkGeneric 11/01/2003 - "A putative diagnosis of lymphocytic hypophysitis was made and prednisone was prescribed. " 09/01/2011 - "We describe the case of a 41-yr-old man with autoimmune hypophysitis in whom prednisone therapy induced remission of the disease but was followed by a sudden relapse after withdrawal. " 02/01/2020 - "Suspecting a primary lymphocytic hypophysitis, she was treated with prednisone 60 mg/day and hormonal replacement therapy. " 09/01/1994 - "Lymphocytic hypophysitis was then suspected; in the absence of visual complication and because this patient refused surgery, corticosteroids were attempted at a daily dose of 60 mg of prednisone for 3 months, progressively decreased for the next 6 months. "
9.	HLA Antigens (Human Leukocyte Antigens)IBA 01/01/2014 - "Based on the patient's endocrinological findings, he was diagnosed with hypopituitarism possibly due to lymphocytic hypophysitis, as supported by positive results for human leukocyte antigen A24 and Cw3. "
10.	AntibodiesIBA 12/01/2023 - "Recently, the presence of anti-rabphilin-3A antibodies has been found to be a highly sensitive and specific marker of lymphocytic infundibuloneurohypophysitis, an autoimmune-mediated CDI. " 01/01/2022 - "Recently, anti-rabphilin-3A antibodies were demonstrated to be a highly sensitive and specific marker of lymphocytic infundibuloneurohypophysitis (LINH). " 11/01/2021 - "Anti-pituitary antibodies (APA) have been described in sera from patients suffering from autoimmune hypophysitis, though their pathophysiological significance remains largely unknown and their diagnostic value limited. " 01/28/2021 - "The negative results for anti-pituitary antibodies and anti-Rabphilin-3A antibodies suggested that upward extension of lymphocytic adenohypophysitis or infundibulo-neurohypophysitis was unlikely. " 06/01/2017 - "Anti-pituitary antibodies against corticotrophs in IgG4-related hypophysitis."

Therapies and Procedures

1.	Therapeutics 09/01/2011 - "Medical therapy of autoimmune hypophysitis with immunosuppressive drugs can be effective to induce remission of the disease by treating both pituitary dysfunction and compression symptoms. " 11/29/2022 - "Inhibition of IRAK1 Is an Effective Therapy for Autoimmune Hypophysitis in Mice." 12/01/2013 - "Current advances in DI concern the etiological work-up, with in particular the identification of IgG4-related hypophysitis or many genetic abnormalities, opening the field of targeted therapies in the years to come." 06/25/2012 - "[Lymphocytic hypophysitis due to ipilimumap therapy]." 01/01/2006 - "Lymphocytic hypophysitis: disease spectrum and approach to diagnosis and therapy."
2.	Immunotherapy 01/01/2019 - "Immunotherapy-induced autoimmune hypophysitis." 01/01/2021 - "New treatment modalities, such as immunotherapy and molecular targeted therapy have the potential to cause autoimmune hypophysitis. " 03/01/2018 - "[Autoimmune hypophysitis associated with new anti-cancer immunotherapies]." 02/01/2016 - "Hypophysitis occurs in a significant minority of patients treated with Ipi, in contrast to the relative rarity of idiopathic autoimmune hypophysitis or hypophysitis after treatment with other immunotherapies. " 01/01/2020 - "The frequent emergence of lymphocytic hypophysitis following checkpoint blockade for other cancers, as well as the expression of PD-L1 on pituitary adenomas, suggest a role for immunotherapy. "
3.	Radiotherapy 09/01/2003 - "Stereotactic radiotherapy for the treatment of lymphocytic hypophysitis. " 12/15/2021 - "Mass-related symptoms in patients with autoimmune hypophysitis are treated with anti-inflammatory therapy, surgery, and/or radiotherapy. " 09/01/2003 - "Two patients with symptomatic lymphocytic hypophysitis, which recurred after standard therapy, were treated with low-dose stereotactic radiotherapy. " 11/01/2017 - "Patients with hyponatremia were divided into two groups, based on the etiology of hypopituitarism: Group 1. with NFPA n = 15 (5F/10M), mean age 71.47 ± 4.8 years, who were significantly older compared to patients with hyponatremia from other rare causes of hypopituitarism in Group 2. n = 10 (8F/2M), mean age 40.2 ± 15.3 years (p < 0.01), such as: congenital hypopituitarism(n = 2), Sheehan's syndrome (n = 2), intracranial aneurysm (n = 2), lymphocytic hypophysitis (n = 1), traumatic brain injury (n = 1), surgery and radiotherapy for astrocytoma (n = 1), pituitary metastasis from bronchial carcinoma (n = 1). " 09/01/2003 - "The authors conclude that stereotactic radiotherapy represents an effective, noninvasive treatment option for patients with lymphocytic hypophysitis, particularly if the disease is recurrent after surgery or resistant to corticosteroid medications."
4.	Aftercare (After-Treatment) 02/01/2016 - "Hypophysitis occurs in a significant minority of patients treated with Ipi, in contrast to the relative rarity of idiopathic autoimmune hypophysitis or hypophysitis after treatment with other immunotherapies. " 10/01/2009 - "We report on a case of autoimmune hypophysitis with severe clinical symptoms that resolved rapidly after treatment with steroids. " 06/09/2020 - "Transnasal biopsy suggested a diagnosis of autoimmune hypophysitis, and the patient displayed radiological and clinical improvement after treatment with glucocorticoids and hormone replacement. "
5.	Hypophysectomy 06/01/2016 - "The authors report the first patient of vasospasm following transsphenoidal hypophysectomy in the treatment of lymphocytic hypophysitis; a rare inflammatory disorder characterized by lymphocytic infiltration of the pituitary gland causing destruction glandular tissue. " 11/01/1999 - "Biopsy revealed lymphocytic adenohypophysitis, and no hypophysectomy was performed. " 06/01/2016 - "Cerebral Vasospasm Following Transsphenoidal Hypophysectomy in the Treatment of Lymphocytic Hypophysitis."