raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:615970

Chronic intervillositis of unknown etiology

Synonyms: CIUE

Type
Disease
Group
Disorder
Inheritance
Not applicable
Age of onset
Adolescent, Adult
Status
active

A rare disorder related to pregnancy characterized by a placental inflammatory process with clusters of histiocytes and associated fibrin in intervillous spaces, resulting in adverse pregnancy outcomes including intrauterine growth restriction, preterm birth, and pregnancy loss. Higher grading according to the proportion of involved intervillous space has been associated with poorer outcomes. There is a significant risk of recurrence in subsequent pregnancies.

1

Names in Orphadata languages

10 of 12 languages
LangNameSynonyms
enChronic intervillositis of unknown etiologyCIUE
frIntervillite chronique d'étiologie indéterminéeICEI · Intervillite chronique sans étiologie connue
deChronische Intervillositis unbekannter Ätiologie= en
esIntervillositis crónica de etiología desconocida
itIntervillosite cronica a eziologia ignota= en
nlChronische intervillositis met onbekende etiologie= en
ptIntervilosite crónica de etiologia desconhecida= en
plPrzewlekłe zapalenie międzykosmków o nieznanej etiologii= en
csChronická intervilozitida neznámé etiologie= en
ukХронічний інтервілозит невідомої етіології= en

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10O43.8NTBTattributedvalidated
ICD-11JA86.4NTBTattributedvalidated
MONDO0035882Evalidated
UMLSC5680386Evalidated

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

1 record
TypeClassValueAreaQualificationValidationSource
Point prevalenceUnknown—WorldwideClass onlyValidated32841927[PMID]_ORPHANET

Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.

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HPO phenotypes

Orphanet has no HPO annotation for this code in this release.

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Sources for this card