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 62)
# Related Disease Score Top Affiliating Genes
1 thyroiditis 10.3
2 lymphocytic hypophysitis 10.2
3 hashimoto thyroiditis 10.2
4 adenoma 10.2
5 hyperprolactinemia 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 hypoadrenalism 10.0
15 secondary adrenal insufficiency 10.0
16 autoimmune polyendocrinopathy type 4 10.0
17 diabetes insipidus 10.0
18 pituitary gland disease 9.9
19 carotid artery occlusion 9.9
20 renal cell carcinoma, nonpapillary 9.8
21 melanoma, uveal 9.8
22 vitiligo-associated multiple autoimmune disease susceptibility 6 9.8
23 myasthenia gravis 9.8
24 ocular motor apraxia 9.8
25 vitiligo-associated multiple autoimmune disease susceptibility 1 9.8
26 sexual disorder 9.8
27 pituitary apoplexy 9.8
28 tetanus 9.8
29 goiter 9.8
30 primary biliary cirrhosis 9.8
31 candidiasis 9.8
32 thrombocytopenia due to platelet alloimmunization 9.8
33 thrombocytopenia 9.8
34 melanoma 9.8
35 hepatitis 9.8
36 kidney cancer 9.8
37 dermatitis 9.8
38 empty sella syndrome 9.8
39 craniopharyngioma 9.8
40 liver cirrhosis 9.8
41 lupus erythematosus 9.8
42 sheehan syndrome 9.8
43 hypoglycemia 9.8
44 anti-pit-1 antibody syndrome 9.8
45 headache 9.8
46 syncope 9.8
47 acute adrenal insufficiency 9.8
48 pituitary hormone deficiency, combined, 2 9.7
49 asplenia, isolated congenital 9.7
50 pituitary adenoma, prolactin-secreting 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 169)
# Title Authors PMID Year
1
Clinical aspects of autoimmune hypothalamitis, a variant of autoimmune hypophysitis: Experience from one center. 61
31779500 2019
2
Hypophysitis induced by immune checkpoint inhibitors: a 10-year assessment. 61
31842671 2019
3
Autoimmune phenomena involving the pituitary gland in children: New developing data about diagnosis and treatment. 61
31401342 2019
4
Disease heterogeneity in IgG4-related hypophysitis: report of two histopathologically proven cases and review of the literature. 61
30911814 2019
5
Machine learning defined diagnostic criteria for differentiating pituitary metastasis from autoimmune hypophysitis in patients undergoing immune checkpoint blockade therapy. 61
31415986 2019
6
Xanthomatous hypophysitis: A rare case report with review of literature. 61
31361237 2019
7
Intracranial germinoma masquerading as secondary granulomatous hypophysitis: A case report and review of literature. 61
31269501 2019
8
Primary hypophysitis: Experience of a Single Tertiary Center. 61
31234218 2019
9
Idiopathic and isolated adrenocorticotropic hormone deficiency presenting as continuous epigastric discomfort without symptoms of hypoglycemia: a case report. 61
31036085 2019
10
A case of severe rhabdomyolysis associated with secondary adrenal insufficiency and autoimmune hepatitis. 61
30898957 2019
11
Hypophysitis - new insights into diagnosis and treatment. 61
31290557 2019
12
Immunotherapy-induced autoimmune hypophysitis. 61
28825378 2019
13
Primary hypophysitis and other autoimmune disorders of the sellar and suprasellar regions. 61
30547288 2018
14
Hypophysitis Outcome and Factors Predicting Responsiveness to Glucocorticoid Therapy: A Prospective and Double-Arm Study. 61
30085134 2018
15
MECHANISMS IN ENDOCRINOLOGY: Hypophysitis: diagnosis and treatment. 61
29880706 2018
16
Neuro-radiological features can predict hypopituitarism in primary autoimmune hypophysitis. 61
29752700 2018
17
Idiopathic granulomatous hypophysitis: A report of an uncommon disorder. 61
30004061 2018
18
Ipilimumab cystic hypophysitis mimicking metastatic melanoma. 61
29977435 2018
19
Human leucocyte antigens coeliac haplotypes and primary autoimmune hypophysitis in caucasian patients. 61
29418012 2018
20
Critical role of rabphilin-3A in the pathophysiology of experimental lymphocytic neurohypophysitis. 61
29377134 2018
21
Clinical, Endocrine and Imaging Characteristics of Patients with Primary Hypophysitis. 61
29458219 2018
22
[Autoimmune hypophysitis associated with new anti-cancer immunotherapies]. 61
29475597 2018
23
Diabetes insipidus is an unfavorable prognostic factor for response to glucocorticoids in patients with autoimmune hypophysitis. 61
29141992 2018
24
Autoimmune hypophysitis. 61
29413466 2018
25
Combination immune checkpoint inhibitor therapy nivolumab and ipilimumab associated with multiple endocrinopathies. 61
29511565 2018
26
IgG4-related hypophysitis is highly prevalent among cases of histologically confirmed hypophysitis. 61
27864854 2017
27
Case of metastatic uveal melanoma in which an antitumor effect appeared after ipilimumab discontinuation due to autoimmune hypophysitis. 61
27864834 2017
28
Intrachiasmatic abscess caused by IgG4-related hypophysitis. 61
28799078 2017
29
Diabetes insipidus is an unfavorable prognostic factor for response to glucocorticoids in patients with autoimmune hypophysitis. 61
28626085 2017
30
Intratumoral granulomatous reaction in recurrent pituitary adenoma: A unique presentation. 61
28862230 2017
31
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
32
In Situ Activation of Pituitary-Infiltrating T Lymphocytes in Autoimmune Hypophysitis. 61
28262761 2017
33
An Overview of Diagnosis of Primary Autoimmune Hypophysitis in a Prospective Single-Center Experience. 61
27165294 2017
34
Autoimmune Hypophysitis Presenting as Solid-Cystic Mass Managed Conservatively. 61
29662575 2017
35
Pituitary Antibodies in an Adolescent with Secondary Adrenal Insufficiency and Turner Syndrome. 61
27355580 2017
36
Pituitary Autoimmunity. 61
28245451 2017
37
Revisitation of autoimmune hypophysitis: knowledge and uncertainties on pathophysiological and clinical aspects. 61
27503372 2016
38
The management of hypophysitis. 61
26963662 2016
39
A complicated case of primary hypophysitis with bilateral intracavernous carotid artery occlusion. 61
27376431 2016
40
Immunotherapy and hypophysitis: clinical presentation, treatment, and biologic insights. 61
26186958 2016
41
Xanthomatous hypophysitis associated with autoimmune disease in an elderly patient: A rare case report. 61
27500004 2016
42
A case of anti-aminoacyl tRNA synthetase antibody syndrome complicated by hemophagocytic syndrome. 61
28049963 2016
43
Autoimmune polyendocrinopathy and hypophysitis after Puumala hantavirus infection. 61
27857837 2016
44
Ipilimumab-Induced Autoimmune Hypophysitis: Diagnostic and Management Challenges Illustrated by a Clinical Case. 61
27265913 2015
45
Diagnosis of Primary Hypophysitis in Germany. 61
26262437 2015
46
Hypophysitis in 2014. 61
26514950 2015
47
Treatment of Primary Hypophysitis in Germany. 61
26091204 2015
48
Effect of cancer treatment on hypothalamic-pituitary function. 61
25873572 2015
49
Early recognition of ipilimumab-related autoimmune hypophysitis in patients with metastatic melanoma: Case studies and recommendations for management. 61
25855890 2015
50
Hypophysitis. 61
25732650 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
Content
Loading form....