raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:95513

Panhypophysitis

Synonyms: Infundibulo-panhypophysitis

Type
Disease
Group
Disorder
Age of onset
Adolescent, Adult, Childhood, Elderly
Status
active

A type of primary hypophysitis characterized by an 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.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enPanhypophysitisInfundibulo-panhypophysitis
frPanhypophysitePanhypophysite infundibulaire
deHypophysitis, nekrotisierendeInfundibulohypophysitis, nekrotisierende
esPanhipofisitisPanhipofisitis-infundibulo
itPanipofisitePanipofisite infundibolare
nlPanhypofysitisInfundibulo-panhypofysitis
ptHipofisite necrotizante
plZapalenie przysadki= en
csPanhypofyzitidaInfundibulo-panhypofyzitida
ukПангіпофізитІнфундибуло-пангіпофізит
zh全垂体炎漏斗-全垂体炎
trPanhipofizitİnfundibulo-panhipofizit

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10E23.6NTBTattributedvalidated
ICD-115A61.0NTBTattributedvalidated
MONDO0019839Evalidated
UMLSC5190786Evalidated

E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.

3

Classification

Preferential parent (Orphanet linearisation):

Parents in the classifications (1):

No children.

4

Genes

0 direct

Orphanet records no gene directly on this code.

5

Prevalence and incidence

0 records

No prevalence record in this release.

6

HPO phenotypes

35 annotations · validated 2017-06-28
HPOTerm (as annotated by Orphanet)Diagnostic
Very frequent (99-80%)
HP:0011751Abnormality of the posterior pituitary
HP:0000863Central diabetes insipidus
HP:0000871Panhypopituitarism
Frequent (79-30%)
HP:0012504Abnormal size of pituitary gland
HP:0012696Abnormal thalamic MRI signal intensity
HP:0011748Adrenocorticotropic hormone deficiency
HP:0011735Adrenocorticotropin deficient adrenal insufficiency
HP:0000141Amenorrhea
HP:0000622Blurred vision
HP:0008163Decreased circulating cortisol level
HP:0030018Decreased female libido
HP:0040306Decreased male libido
HP:0008214Decreased serum estradiol
HP:0040171Decreased serum testosterone concentration
HP:0008213Gonadotropin deficiency
HP:0002315Headache
HP:0003158Hyposthenuria
HP:0000802Impotence
HP:0000870Increased circulating prolactin concentration
HP:0002018Nausea
HP:0001895Normochromic anemia
HP:0001278Orthostatic hypotension
HP:0000980Pallor
HP:0008245Pituitary hypothyroidism
HP:0001959Polydipsia
HP:0007987Progressive visual field defects
HP:0008240Secondary growth hormone deficiency
Occasional (29-5%)
HP:0003493Antinuclear antibody positivity
HP:0007041Chronic lymphocytic meningitis
HP:0000651Diplopia
HP:0000872Hashimoto thyroiditis
HP:0002902Hyponatremia
HP:0004396Poor appetite
HP:0008202Reduced circulating prolactin concentration
HP:0000407Sensorineural hearing impairment

Run the differential on the 3 obligate / very frequent terms

Annotation source: 23885185[PMID]_27008424[PMID]_18987830[PMID]_17273922[PMID]_14606714[PMID]_27165294[PMID]

7

Sources for this card