raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:284264

IgG4-related disease

Synonyms: IgG4-related sclerosing disease · Immunoglobulin G4-related sclerosing disease

Type
Clinical group
Group
Group of disorders
Inheritance
Not applicable
Age of onset
Adult
Status
active

This term does not characterize a disease but a group of diseases. To learn about the diseases included under this term, you can consult the classifications.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enIgG4-related diseaseIgG4-related sclerosing disease · Immunoglobulin G4-related sclerosing disease
frMaladie associée aux IgG4Maladie sclérosante liée aux IgG4 · Maladie sclérosante liée aux immunoglobulines G4
deIgG4-assoziierte KrankheitImmunglobulin-G4-assoziierte sklerosierende Krankheit · IgG4-assoziierte Systemkrankheit · IgG4-assoziierte sklerosierende Krankheit
esEnfermedad asociada a IgG4Enfermedad esclerosante ligada a inmunoglobulinas G4
itMalattia IgG4-correlataMalattia sclerosante legata alle IgG4 · Malattia sclerosante legata alle immunoglobuline G4 · Malattia sistemica legata alle IgG4
nlIgG4-gerelateerde ziekteIgG4-gerelateerde scleroserende ziekte · IgG4-gerelateerde systemische ziekte · Immunoglobuline G4-gerelateerde scleroserende ziekte
ptDoença IgG4-relacionadaDoença esclerosante relacionada com imunoglobulina G4
plChoroba stwardniająca związana z immunoglobuliną G4Choroba stwardniająca związana z IgG4 · Choroba układowa związana z IgG4
csIgG4 asociovaná onemocněníIgG4 asociovaná sklerotizující onemocnění · IgG4 asociovaná systémová onemocnění · Imunoglobulin G4 asociovaná sklerotizující onemocnění
ukЗахворювання, повязане з IgG4Склерозуюча хвороба, повязана з IgG4 · Імуноглобулін G4 асоційована склерозуюча хвороба
zhIgG4相关疾病免疫球蛋白G4相关硬化性疾病
trIgG4-ilişkili hastalıkIgG4-ilişkili sistemik hastalık · IgG4-ilişkili sklerozan hastalık · İmmünoglobulin G4-ilişkili sklerozan hastalık
2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-114A43.0Especificvalidated
MONDO17287Evalidated
MeSHD000077733Evalidated
UMLSC3203653Evalidated
GARD12521Evalidated
MedDRA10071569Evalidated

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):

Children (5):

4

Genes

0 direct

Orphanet records no gene directly on this code.

5

Prevalence and incidence

1 record
TypeClassValueAreaQualificationValidationSource
Point prevalenceUnknown—WorldwideClass onlyNot yet validatedORPHANET

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

6

HPO phenotypes

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

7

Sources for this card