Outcomes of Initial Management Strategies in Patients With Autoimmune Lymphocytic Hypophysitis: A Systematic Review and Meta-analysis

Donegan, Diane; Saeed, Zeb; Delivanis, Danae A.; Murad, Mohammad Hassan; Honegger, Juergen; Amereller, Felix; Oğuz, Seda Hanife; Erickson, Dana; Bancos, Irina

doi:10.1210/clinem/dgab839

Cited by 15 publications

(10 citation statements)

References 49 publications

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“…2 ). Nonetheless, the APH recovery rate of 45% with oral glucocorticoids in milder cases seen in our study is better than that achieved by observation alone (21%) in the meta-analysis cohort by Donegan et al ( 4 ). The decision of observation vs glucocorticoid treatment in mild-moderate cases of hypophysitis is still debatable.…”

Section: Discussioncontrasting

confidence: 82%

“…Besides, the indication for therapeutic glucocorticoid administration, if needed, remains to be established. In the recent meta-analysis, high-dose glucocorticoids are associated with improved recovery of deficits, but optimal dosing, route, and duration are unclear ( 4 ). In the current IPD-MA, better visual field-deficit recovery, overall hormonal response (66.7% vs 45.5%), in particular corticotroph axis recovery, and a lesser need for rescue therapy were observed with an i.v.…”

Section: Discussionmentioning

confidence: 99%

“…A recent meta-analysis concluded that patients with milder presentation be observed, reserving supraphysiological dose of glucocorticoid therapy for those with severe or progressive neurologic deficits. Anterior pituitary hormonal (APH) recovery rate was highest with glucocorticoids (45.5%) when compared to surgery (14%) and observation (21%) but with a disadvantage of a higher proportion of patients having disease progression or relapse ( 4 ). In the former study, treatment with any supraphysiological dose of glucocorticoids was considered a high dose; however, the optimal initiating dose, route of administration, and duration of treatment are not known.…”

Section: Introductionmentioning

confidence: 99%

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Glucocorticoid therapy as first-line treatment in primary hypophysitis: a systematic review and individual patient data meta-analysis

Krishnappa

Shah

Memon

et al. 2023

Endocrine Connections

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Objectives: High-dose glucocorticoids are associated with improved recovery of deficits in primary autoimmune hypophysitis (PAH), but optimal dosing, route, and duration are unclear. Design: We reviewed literature for first-line glucocorticoid treatment in PAH until December 2021 and performed an individual-patient data meta-analysis to analyze clinical, hormonal, and radiological outcomes with respect to route, dose, and duration (<6.5 vs. 6.5-12 vs. >12 weeks) of glucocorticoid treatment according to disease severity. Results: 153 PAH patients from 83 publications were included. The median age at presentation was 41(32.5-48) years with a female preponderance (70.3%). Visual field recovery was significantly better with intravenous (91.7%) as compared to oral (54.5%) route, high dose (100%) and very-high dose (90.9%) as compared to medium dose (20%) of glucocorticoids. Corticotroph axis recovery was greater in intravenous (54.8% vs. 28.1% oral, p=0.033) route and increasing glucocorticoid dose group (0% vs. 38.1% vs. 57.1%), attaining statistical significance (p=0.012) with very high-dose. A longer duration of treatment (>6.5 weeks) was associated with better corticotroph and thyrotroph recovery. Need for rescue therapy with lower with intravenous route (38% vs 17.5%, p=0.012) and with increasing glucocorticoid doses (53.3% vs. 34.3% vs. 17.3%, p=0.016). In severe disease, visual field and corticotroph axis recovery was significantly higher with intravenous route and very-high dose steroids. The adverse effects of glucocorticoids were independent of dose and duration of treatment. Conclusions: Very high-dose glucocorticoids by intravenous route and cumulative longer duration (>6.5 weeks) lead to better outcomes and could be considered as first-line treatment of severe PAH cases.

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Section: Discussioncontrasting

confidence: 82%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Glucocorticoid therapy as first-line treatment in primary hypophysitis: a systematic review and individual patient data meta-analysis

Krishnappa

Shah

Memon

et al. 2023

Endocrine Connections

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“…Other mostly non-neoplastic pathologies include lymphocytic hypophysitis, Langerhans cell histiocystosis (LCH), abscesses and neurosarcoidosis. 16,27 Additional infiltrative disorders include granulomatosis with polyangiitis, 28 and immunoglobulin (Ig)G4-related hypophysitis. 29,30 Most of these cases also present with concomitant anterior pituitary hormonal deficiencies.…”

Section: Systemic And/or Infiltrative Disordersmentioning

confidence: 99%

New developments and concepts in the diagnosis and management of diabetes insipidus (AVP‐deficiency and resistance)

Angelousi

Alexandraki

Mytareli

et al. 2023

J Neuroendocrinology

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Diabetes insipidus (DI) is a disorder characterised by the excretion of large amounts of hypotonic urine, with a prevalence of 1 per 25,000 population. Central DI (CDI), better now referred to as arginine vasopressin (AVP)‐deficiency, is the most common form of DI resulting from deficiency of the hormone AVP from the pituitary. The less common nephrogenic DI (NDI) or AVP‐resistance develops secondary to AVP resistance in the kidneys. The majority of causes of DI are acquired, with CDI developing when more than 80% of AVP‐secreting neurons are damaged. Inherited/familial CDI causes account for approximately 1% of cases. Although the pathogenesis of NDI is unclear, more than 280 disease‐causing mutations affecting the AVP2 protein or AVP V2 receptor, as well as in aquaporin 2 (AQP2), have been described. Although the cAMP/protein kinase A pathway remains the major regulatory pathway of AVP/AQP2 action, in vitro data have also revealed additional cAMP independent pathways of NDI pathogenesis. Diagnosing partial forms of DI, and distinguishing them from primary polydipsia, can be challenging, previously necessitating the use of the water deprivation test. However, measurements of circulating copeptin levels, especially after stimulation, are increasingly replacing the classical tests in clinical practice because of their ease of use and high sensitivity and specificity. The treatment of CDI relies on desmopressin administration, whereas NDI requires the management of any underlying diseases, removal of offending drugs and, in some cases, administration of diuretics. A better understanding of the pathophysiology of DI has led to novel evolving therapeutic agents that are under clinical trial.

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“…Prompt medical care is vital because AH can be fatal if neglected [ 3 , 4 ], although fatal cases are less often seen due to increased awareness of the disease. AH patients are often managed by corticosteroids [ 5 ]; however, long-term use of steroids may lead to considerable side effects [ 6 ]. AH may reoccur after tapering and discontinuation of corticosteroids [ 7 ].…”

Section: Introductionmentioning

confidence: 99%

Inhibition of IRAK1 Is an Effective Therapy for Autoimmune Hypophysitis in Mice

Huang

Chen

Lin

et al. 2022

IJMS

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Autoimmune hypophysitis (AH) is an autoimmune disease of the pituitary for which the pathogenesis is incompletely known. AH is often treated with corticosteroids; however, steroids may lead to considerable side effects. Using a mouse model of AH (experimental autoimmune hypophysitis, EAH), we show that interleukin-1 receptor-associated kinase 1 (IRAK1) is upregulated in the pituitaries of mice that developed EAH. We identified rosoxacin as a specific inhibitor for IRAK1 and found it could treat EAH. Rosoxacin treatment at an early stage (day 0–13) slightly reduced disease severity, whereas treatment at a later stage (day 14–27) significantly suppressed EAH. Further investigation indicated rosoxacin reduced production of autoantigen-specific antibodies. Rosoxacin downregulated production of cytokines and chemokines that may dampen T cell differentiation or recruitment to the pituitary. Finally, rosoxacin downregulated class II major histocompatibility complex expression on antigen-presenting cells that may lead to impaired activation of autoantigen-specific T cells. These data suggest that IRAK1 may play a pathogenic role in AH and that rosoxacin may be an effective drug for AH and other inflammatory diseases involving IRAK1 dysregulation.

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Outcomes of Initial Management Strategies in Patients With Autoimmune Lymphocytic Hypophysitis: A Systematic Review and Meta-analysis

Cited by 15 publications

References 49 publications

Glucocorticoid therapy as first-line treatment in primary hypophysitis: a systematic review and individual patient data meta-analysis

Glucocorticoid therapy as first-line treatment in primary hypophysitis: a systematic review and individual patient data meta-analysis

New developments and concepts in the diagnosis and management of diabetes insipidus (AVP‐deficiency and resistance)

Inhibition of IRAK1 Is an Effective Therapy for Autoimmune Hypophysitis in Mice

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