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Panhypophysitis

MedGen UID:
1674527
•Concept ID:
C5190786
•
Disease or Syndrome
Synonyms: Infundibulo panhypophysitis; Infundibulo-panhypophysitis; panhypophysitis
SNOMED CT: Infundibulo panhypophysitis (783016009); Panhypophysitis (783016009)
 
Monarch Initiative: MONDO:0019839
Orphanet: ORPHA95513

Definition

A rare acquired pituitary hormone deficiency a type of primary hypophysitis with characteristics of inflammation of the entire pituitary gland. Common clinical presentation is diabetes insipidus with polyuria and polydipsia and partial or panhypopituitarism. Other symptoms may include headaches, nausea/vomiting, visual disturbances and fatigue. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPanhypophysitis

Professional guidelines

PubMed

Outcomes of Initial Management Strategies in Patients With Autoimmune Lymphocytic Hypophysitis: A Systematic Review and Meta-analysis.
Donegan D, Saeed Z, Delivanis DA, Murad MH, Honegger J, Amereller F, Oguz SH, Erickson D, Bancos I
J Clin Endocrinol Metab 2022 Mar 24;107(4):1170-1190. doi: 10.1210/clinem/dgab839. PMID: 35137155Free PMC Article
Early Pulse Glucocorticoid Therapy and Improved Hormonal Outcomes in Primary Hypophysitis.
Krishnappa B, Shah R, Sarathi V, Lila AR, Sehemby MK, Patil VA, Sankhe S, Shah N, Bandgar T
Neuroendocrinology 2022;112(2):186-195. Epub 2021 Mar 19 doi: 10.1159/000516006. PMID: 33744880
An Overview of Diagnosis of Primary Autoimmune Hypophysitis in a Prospective Single-Center Experience.
Chiloiro S, Tartaglione T, Angelini F, Bianchi A, Arena V, Giampietro A, Mormando M, Sciandra M, Laino ME, De Marinis L
Neuroendocrinology 2017;104(3):280-290. Epub 2016 May 11 doi: 10.1159/000446544. PMID: 27165294

Recent clinical studies

Etiology

Is There A Connection Between Primary Hypophysitis and Celiac Disease?
Kara Z, Eşkazan T, Şahin S, Durcan E, Sulu C, Demir AN, Uysal S, Özkaya HM, Yılmaz E, Hatemi Aİ, Bolayırlı İM, Kadıoğlu P
Exp Clin Endocrinol Diabetes 2024 Jan;132(1):33-38. Epub 2023 Nov 17 doi: 10.1055/a-2213-4375. PMID: 37977557
Early Pulse Glucocorticoid Therapy and Improved Hormonal Outcomes in Primary Hypophysitis.
Krishnappa B, Shah R, Sarathi V, Lila AR, Sehemby MK, Patil VA, Sankhe S, Shah N, Bandgar T
Neuroendocrinology 2022;112(2):186-195. Epub 2021 Mar 19 doi: 10.1159/000516006. PMID: 33744880
Single center study of 53 consecutive patients with pituitary stalk lesions.
Doknic M, Miljic D, Pekic S, Stojanovic M, Savic D, Manojlovic-Gacic E, Milenkovic T, Zdravkovic V, Jesic M, Damjanovic D, Lavrnic S, Soldatovic I, Djukic A, Petakov M
Pituitary 2018 Dec;21(6):605-614. doi: 10.1007/s11102-018-0914-2. PMID: 30276501
Human leucocyte antigens coeliac haplotypes and primary autoimmune hypophysitis in caucasian patients.
Chiloiro S, Capoluongo ED, Tartaglione T, Bianchi A, Giampietro A, Angelini F, Arena V, Pontecorvi A, De Marinis L
Clin Endocrinol (Oxf) 2018 May;88(5):692-699. Epub 2018 Feb 26 doi: 10.1111/cen.13566. PMID: 29418012
An Overview of Diagnosis of Primary Autoimmune Hypophysitis in a Prospective Single-Center Experience.
Chiloiro S, Tartaglione T, Angelini F, Bianchi A, Arena V, Giampietro A, Mormando M, Sciandra M, Laino ME, De Marinis L
Neuroendocrinology 2017;104(3):280-290. Epub 2016 May 11 doi: 10.1159/000446544. PMID: 27165294

Diagnosis

Neuroimaging of hypophysitis: etiologies and imaging mimics.
Kurokawa R, Kurokawa M, Baba A, Nakaya M, Kato S, Bapuraj J, Nakata Y, Ota Y, Srinivasan A, Abe O, Moritani T
Jpn J Radiol 2023 Sep;41(9):911-927. Epub 2023 Apr 3 doi: 10.1007/s11604-023-01417-y. PMID: 37010787Free PMC Article
Hypophysitis.
Donegan D, Honegger J
Endocr Pract 2022 Sep;28(9):901-910. Epub 2022 Jun 30 doi: 10.1016/j.eprac.2022.06.009. PMID: 35779833
Imaging findings in hypophysitis: a review.
Caranci F, Leone G, Ponsiglione A, Muto M, Tortora F, Muto M, Cirillo S, Brunese L, Cerase A
Radiol Med 2020 Mar;125(3):319-328. Epub 2019 Dec 20 doi: 10.1007/s11547-019-01120-x. PMID: 31863360
An Overview of Diagnosis of Primary Autoimmune Hypophysitis in a Prospective Single-Center Experience.
Chiloiro S, Tartaglione T, Angelini F, Bianchi A, Arena V, Giampietro A, Mormando M, Sciandra M, Laino ME, De Marinis L
Neuroendocrinology 2017;104(3):280-290. Epub 2016 May 11 doi: 10.1159/000446544. PMID: 27165294
Pituitary autoimmune disease: nuances in clinical presentation.
Glezer A, Bronstein MD
Endocrine 2012 Aug;42(1):74-9. Epub 2012 Mar 17 doi: 10.1007/s12020-012-9654-7. PMID: 22426958

Therapy

Outcomes of Initial Management Strategies in Patients With Autoimmune Lymphocytic Hypophysitis: A Systematic Review and Meta-analysis.
Donegan D, Saeed Z, Delivanis DA, Murad MH, Honegger J, Amereller F, Oguz SH, Erickson D, Bancos I
J Clin Endocrinol Metab 2022 Mar 24;107(4):1170-1190. doi: 10.1210/clinem/dgab839. PMID: 35137155Free PMC Article
Early Pulse Glucocorticoid Therapy and Improved Hormonal Outcomes in Primary Hypophysitis.
Krishnappa B, Shah R, Sarathi V, Lila AR, Sehemby MK, Patil VA, Sankhe S, Shah N, Bandgar T
Neuroendocrinology 2022;112(2):186-195. Epub 2021 Mar 19 doi: 10.1159/000516006. PMID: 33744880
Diabetes insipidus is an unfavorable prognostic factor for response to glucocorticoids in patients with autoimmune hypophysitis.
Lupi I, Cosottini M, Caturegli P, Manetti L, Urbani C, Cappellani D, Scattina I, Martino E, Marcocci C, Bogazzi F
Eur J Endocrinol 2017 Aug;177(2):127-135. doi: 10.1530/EJE-17-0123. PMID: 28626085
Lymphocytic panhypophysitis in a young man with involvement of the cavernous sinus and clivus.
Kartal I, Yarman S, Tanakol R, Bilgic B
Pituitary 2007;10(1):75-80. doi: 10.1007/s11102-007-0003-4. PMID: 17273922

Prognosis

Early Pulse Glucocorticoid Therapy and Improved Hormonal Outcomes in Primary Hypophysitis.
Krishnappa B, Shah R, Sarathi V, Lila AR, Sehemby MK, Patil VA, Sankhe S, Shah N, Bandgar T
Neuroendocrinology 2022;112(2):186-195. Epub 2021 Mar 19 doi: 10.1159/000516006. PMID: 33744880
Imaging findings in hypophysitis: a review.
Caranci F, Leone G, Ponsiglione A, Muto M, Tortora F, Muto M, Cirillo S, Brunese L, Cerase A
Radiol Med 2020 Mar;125(3):319-328. Epub 2019 Dec 20 doi: 10.1007/s11547-019-01120-x. PMID: 31863360
Single center study of 53 consecutive patients with pituitary stalk lesions.
Doknic M, Miljic D, Pekic S, Stojanovic M, Savic D, Manojlovic-Gacic E, Milenkovic T, Zdravkovic V, Jesic M, Damjanovic D, Lavrnic S, Soldatovic I, Djukic A, Petakov M
Pituitary 2018 Dec;21(6):605-614. doi: 10.1007/s11102-018-0914-2. PMID: 30276501
Neuro-radiological features can predict hypopituitarism in primary autoimmune hypophysitis.
Tartaglione T, Chiloiro S, Laino ME, Giampietro A, Gaudino S, Zoli A, Bianchi A, Pontecorvi A, Colosimo C, De Marinis L
Pituitary 2018 Aug;21(4):414-424. doi: 10.1007/s11102-018-0892-4. PMID: 29752700
Lymphocytic infundibulo-neurohypophysitis and infundibulo-panhypophysitis regarded as lymphocytic hypophysitis variant.
Abe T
Brain Tumor Pathol 2008;25(2):59-66. Epub 2008 Nov 6 doi: 10.1007/s10014-008-0234-8. PMID: 18987830

Clinical prediction guides

Is There A Connection Between Primary Hypophysitis and Celiac Disease?
Kara Z, Eşkazan T, Şahin S, Durcan E, Sulu C, Demir AN, Uysal S, Özkaya HM, Yılmaz E, Hatemi Aİ, Bolayırlı İM, Kadıoğlu P
Exp Clin Endocrinol Diabetes 2024 Jan;132(1):33-38. Epub 2023 Nov 17 doi: 10.1055/a-2213-4375. PMID: 37977557
Early Pulse Glucocorticoid Therapy and Improved Hormonal Outcomes in Primary Hypophysitis.
Krishnappa B, Shah R, Sarathi V, Lila AR, Sehemby MK, Patil VA, Sankhe S, Shah N, Bandgar T
Neuroendocrinology 2022;112(2):186-195. Epub 2021 Mar 19 doi: 10.1159/000516006. PMID: 33744880
Imaging findings in hypophysitis: a review.
Caranci F, Leone G, Ponsiglione A, Muto M, Tortora F, Muto M, Cirillo S, Brunese L, Cerase A
Radiol Med 2020 Mar;125(3):319-328. Epub 2019 Dec 20 doi: 10.1007/s11547-019-01120-x. PMID: 31863360
Single center study of 53 consecutive patients with pituitary stalk lesions.
Doknic M, Miljic D, Pekic S, Stojanovic M, Savic D, Manojlovic-Gacic E, Milenkovic T, Zdravkovic V, Jesic M, Damjanovic D, Lavrnic S, Soldatovic I, Djukic A, Petakov M
Pituitary 2018 Dec;21(6):605-614. doi: 10.1007/s11102-018-0914-2. PMID: 30276501
Neuro-radiological features can predict hypopituitarism in primary autoimmune hypophysitis.
Tartaglione T, Chiloiro S, Laino ME, Giampietro A, Gaudino S, Zoli A, Bianchi A, Pontecorvi A, Colosimo C, De Marinis L
Pituitary 2018 Aug;21(4):414-424. doi: 10.1007/s11102-018-0892-4. PMID: 29752700

Recent systematic reviews

Outcomes of Initial Management Strategies in Patients With Autoimmune Lymphocytic Hypophysitis: A Systematic Review and Meta-analysis.
Donegan D, Saeed Z, Delivanis DA, Murad MH, Honegger J, Amereller F, Oguz SH, Erickson D, Bancos I
J Clin Endocrinol Metab 2022 Mar 24;107(4):1170-1190. doi: 10.1210/clinem/dgab839. PMID: 35137155Free PMC Article

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