Kimura disease

Summary

Kimura disease (MONDO:0018830) is a disease. A subtype of lymphadenitis — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

• Prevalence: <1 / 1 000 000 (Worldwide) [Orphanet-validated]
• Phenotypes (HPO): 5

Clinical features

Epidemiology

Prevalence records

2 prevalence record(s), Orphanet:

Type	Class	Value	Geography	Validation
Cases/families		300	Worldwide	Validated
Point prevalence	<1 / 1 000 000		Worldwide	Validated

Signs & symptoms

Clinical features (HPO)

5 HPO clinical features (Orphanet curated; top 5 by frequency):

HPO ID	Term	Frequency
HP:0001880	Eosinophilia	Very frequent (80-99%)
HP:0002729	Follicular hyperplasia	Very frequent (80-99%)
HP:0003212	Increased circulating IgE level	Very frequent (80-99%)
HP:0002716	Lymphadenopathy	Frequent (30-79%)
HP:0010286	Abnormal salivary gland morphology	Occasional (5-29%)

Identifiers

Disease identifiers

Field	Value
Canonical name	Kimura disease
Mondo ID	MONDO:0018830
EFO	EFO:1000722
MeSH	D000082242, D000796
Orphanet	482
DOID	DOID:7365
ICD-11	1229046951
NCIT	C26867
UMLS	C0033838
MedGen	46183
GARD	0006835
MedDRA	10048640
Is cancer (heuristic)	no

Also known as: angiolymphoid hyperplasia with eosinophilia · eosinophilic granuloma of soft tissue · eosinophilic hyperplastic lymphogranuloma · eosinophilic lymphofollicular granuloma · eosinophilic lymphofolliculosis · eosinophilic lymphogranuloma · Kimura’s disease

Disease family

This is a subtype of lymphadenitis. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.

Classification path: disease › human disease › disease by body system or component › immune system disorder › lymphoid system disorder › lymphatic system disorder › lymph node disorder › lymphadenitis › Kimura disease

Related subtypes (9): mesenteric lymphadenitis, cervical lymphadenitis, postauricular lymphadenitis, suppurative lymphadenitis, axillary lymphadenitis, cat-scratch disease, sialadenitis, Kawasaki disease, Kikuchi-Fujimoto disease

Genetics & variants

GWAS landscape

No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.

Variant details and genetic-evidence tiers

No tiered GWAS variants or ClinVar records for this disease.

Genes & proteins

No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).

Function

No pathway enrichment — requires an associated-gene cohort.

Therapeutics

No druggable-target or therapeutic data for this disease’s cohort.

Clinical trials & evidence

Clinical trials

Clinical trials: 0.