MCID: PRM331
MIFTS: 18

Primary Hypophysitis

Categories: Endocrine diseases, Rare diseases

Aliases & Classifications for Primary Hypophysitis

MalaCards integrated aliases for Primary Hypophysitis:

Name: Primary Hypophysitis 58
Autoimmune Hypophysitis 58

Characteristics:

Orphanet epidemiological data:

58
primary hypophysitis
Inheritance: Not applicable; Age of onset: Adult;

Classifications:

Orphanet: 58  
Rare endocrine diseases


External Ids:

ICD10 via Orphanet 33 E23.6
UMLS via Orphanet 72 C0342410
Orphanet 58 ORPHA95506

Summaries for Primary Hypophysitis

MalaCards based summary : Primary Hypophysitis, also known as autoimmune hypophysitis, is related to thyroiditis and lymphocytic hypophysitis. Affiliated tissues include pituitary, thyroid and thymus.

Related Diseases for Primary Hypophysitis

Diseases related to Primary Hypophysitis via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 61)
# Related Disease Score Top Affiliating Genes
1 thyroiditis 10.3
2 lymphocytic hypophysitis 10.2
3 hashimoto thyroiditis 10.2
4 hyperprolactinemia 10.2
5 adenoma 10.1
6 granulomatous hypophysitis 10.1
7 graves' disease 10.1
8 hyperthyroidism 10.1
9 autoimmune disease 10.0
10 enterocolitis 10.0
11 uveitis 10.0
12 amenorrhea 10.0
13 autoimmune polyendocrine syndrome 10.0
14 secondary adrenal insufficiency 10.0
15 autoimmune polyendocrinopathy type 4 10.0
16 diabetes insipidus 10.0
17 pituitary hormone deficiency, combined, 2 9.9
18 hypogonadism 9.9
19 pituitary gland disease 9.9
20 carotid artery occlusion 9.9
21 melanoma, uveal 9.8
22 vitiligo-associated multiple autoimmune disease susceptibility 6 9.8
23 diabetes mellitus, type i 9.8
24 myasthenia gravis 9.8
25 ocular motor apraxia 9.8
26 vitiligo-associated multiple autoimmune disease susceptibility 1 9.8
27 sexual disorder 9.8
28 bacterial infectious disease 9.8
29 pituitary apoplexy 9.8
30 tetanus 9.8
31 goiter 9.8
32 primary biliary cirrhosis 9.8
33 candidiasis 9.8
34 thrombocytopenia due to platelet alloimmunization 9.8
35 thrombocytopenia 9.8
36 melanoma 9.8
37 hepatitis 9.8
38 kidney cancer 9.8
39 dermatitis 9.8
40 empty sella syndrome 9.8
41 craniopharyngioma 9.8
42 liver cirrhosis 9.8
43 lupus erythematosus 9.8
44 sheehan syndrome 9.8
45 hypoglycemia 9.8
46 anti-pit-1 antibody syndrome 9.8
47 headache 9.8
48 syncope 9.8
49 acute adrenal insufficiency 9.8
50 asplenia, isolated congenital 9.7

Graphical network of the top 20 diseases related to Primary Hypophysitis:



Diseases related to Primary Hypophysitis

Symptoms & Phenotypes for Primary Hypophysitis

Drugs & Therapeutics for Primary Hypophysitis

Search Clinical Trials , NIH Clinical Center for Primary Hypophysitis

Genetic Tests for Primary Hypophysitis

Anatomical Context for Primary Hypophysitis

MalaCards organs/tissues related to Primary Hypophysitis:

40
Pituitary, Thyroid, Thymus, Placenta, Testes, Hypothalamus

Publications for Primary Hypophysitis

Articles related to Primary Hypophysitis:

(show top 50) (show all 175)
# Title Authors PMID Year
1
Recurring Primary Xanthomatous Hypophysitis Behaving Like Pituitary Adenoma: Additional Case and Literature Review. 61
32081821 2020
2
Role of 18F-fluorodeoxyglucose (FDG) and 18F-2-fluorodeoxy sorbitol (FDS) in autoimmune hypophysitis: a case report. 61
32517690 2020
3
Clinical Characteristics, Management, and Treatment Outcomes of Primary Hypophysitis: A Monocentric Cohort. 61
32268423 2020
4
Autoimmune hypophysitis secondary to therapy with immune checkpoint inhibitors: Four cases describing the clinical heterogeneity of central endocrine dysfunction. 61
32164491 2020
5
Intracranial Germinoma Masquerading as Secondary Granulomatous Hypophysitis: A Case Report and Review of Literature. 61
31269501 2020
6
A Remarkable Response of Granulomatous Hypophysitis to Infliximab in a Patient With a Background of Crohn's Disease-A Case Report. 61
32547497 2020
7
Hypophysitis: An update on the novel forms, diagnosis and management of disorders of pituitary inflammation. 61
31866206 2019
8
Clinical aspects of autoimmune hypothalamitis, a variant of autoimmune hypophysitis: Experience from one center. 61
31779500 2019
9
Hypophysitis induced by immune checkpoint inhibitors: a 10-year assessment. 61
31842671 2019
10
Autoimmune phenomena involving the pituitary gland in children: New developing data about diagnosis and treatment. 61
31401342 2019
11
Disease heterogeneity in IgG4-related hypophysitis: report of two histopathologically proven cases and review of the literature. 61
30911814 2019
12
Machine learning defined diagnostic criteria for differentiating pituitary metastasis from autoimmune hypophysitis in patients undergoing immune checkpoint blockade therapy. 61
31415986 2019
13
Xanthomatous hypophysitis: A rare case report with review of literature. 61
31361237 2019
14
Primary hypophysitis: Experience of a Single Tertiary Center. 61
31234218 2019
15
Idiopathic and isolated adrenocorticotropic hormone deficiency presenting as continuous epigastric discomfort without symptoms of hypoglycemia: a case report. 61
31036085 2019
16
A case of severe rhabdomyolysis associated with secondary adrenal insufficiency and autoimmune hepatitis. 61
30898957 2019
17
Immunotherapy-induced autoimmune hypophysitis. 61
28825378 2019
18
Hypophysitis - new insights into diagnosis and treatment. 61
31290557 2019
19
Primary hypophysitis and other autoimmune disorders of the sellar and suprasellar regions. 61
30547288 2018
20
Hypophysitis Outcome and Factors Predicting Responsiveness to Glucocorticoid Therapy: A Prospective and Double-Arm Study. 61
30085134 2018
21
MECHANISMS IN ENDOCRINOLOGY: Hypophysitis: diagnosis and treatment. 61
29880706 2018
22
Neuro-radiological features can predict hypopituitarism in primary autoimmune hypophysitis. 61
29752700 2018
23
Idiopathic granulomatous hypophysitis: A report of an uncommon disorder. 61
30004061 2018
24
Ipilimumab cystic hypophysitis mimicking metastatic melanoma. 61
29977435 2018
25
Human leucocyte antigens coeliac haplotypes and primary autoimmune hypophysitis in caucasian patients. 61
29418012 2018
26
Critical role of rabphilin-3A in the pathophysiology of experimental lymphocytic neurohypophysitis. 61
29377134 2018
27
Clinical, Endocrine and Imaging Characteristics of Patients with Primary Hypophysitis. 61
29458219 2018
28
[Autoimmune hypophysitis associated with new anti-cancer immunotherapies]. 61
29475597 2018
29
Diabetes insipidus is an unfavorable prognostic factor for response to glucocorticoids in patients with autoimmune hypophysitis. 61
29141992 2018
30
Autoimmune hypophysitis. 61
29413466 2018
31
Combination immune checkpoint inhibitor therapy nivolumab and ipilimumab associated with multiple endocrinopathies. 61
29511565 2018
32
IgG4-related hypophysitis is highly prevalent among cases of histologically confirmed hypophysitis. 61
27864854 2017
33
Case of metastatic uveal melanoma in which an antitumor effect appeared after ipilimumab discontinuation due to autoimmune hypophysitis. 61
27864834 2017
34
Intrachiasmatic abscess caused by IgG4-related hypophysitis. 61
28799078 2017
35
Diabetes insipidus is an unfavorable prognostic factor for response to glucocorticoids in patients with autoimmune hypophysitis. 61
28626085 2017
36
Intratumoral granulomatous reaction in recurrent pituitary adenoma: A unique presentation. 61
28862230 2017
37
A study of primary hypophysitis, i.e., two cases of lymphocytic hypophysitis and one IgG4-related variant: The importance of measuring serum IgG4 levels to allow early diagnosis and prompt treatment for the IgG4-related variant. 61
27375049 2017
38
In Situ Activation of Pituitary-Infiltrating T Lymphocytes in Autoimmune Hypophysitis. 61
28262761 2017
39
Autoimmune Hypophysitis Presenting as Solid-Cystic Mass Managed Conservatively. 61
29662575 2017
40
An Overview of Diagnosis of Primary Autoimmune Hypophysitis in a Prospective Single-Center Experience. 61
27165294 2017
41
Pituitary Antibodies in an Adolescent with Secondary Adrenal Insufficiency and Turner Syndrome. 61
27355580 2017
42
Pituitary Autoimmunity. 61
28245451 2017
43
Revisitation of autoimmune hypophysitis: knowledge and uncertainties on pathophysiological and clinical aspects. 61
27503372 2016
44
The management of hypophysitis. 61
26963662 2016
45
A complicated case of primary hypophysitis with bilateral intracavernous carotid artery occlusion. 61
27376431 2016
46
Immunotherapy and hypophysitis: clinical presentation, treatment, and biologic insights. 61
26186958 2016
47
Xanthomatous hypophysitis associated with autoimmune disease in an elderly patient: A rare case report. 61
27500004 2016
48
Autoimmune polyendocrinopathy and hypophysitis after Puumala hantavirus infection. 61
27857837 2016
49
A case of anti-aminoacyl tRNA synthetase antibody syndrome complicated by hemophagocytic syndrome. 61
28049963 2016
50
Ipilimumab-Induced Autoimmune Hypophysitis: Diagnostic and Management Challenges Illustrated by a Clinical Case. 61
27265913 2015

Variations for Primary Hypophysitis

Expression for Primary Hypophysitis

Search GEO for disease gene expression data for Primary Hypophysitis.

Pathways for Primary Hypophysitis

GO Terms for Primary Hypophysitis

Sources for Primary Hypophysitis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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