Atopic keratoconjunctivitis

Summary

Atopic keratoconjunctivitis (MONDO:0015599) is a disease and 6 clinical trials. Top therapeutic interventions include lirentelimab and vehicle. A subtype of eye disorder — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

• Prevalence: 1-5 / 10 000 (Europe) [Orphanet-validated]
• Phenotypes (HPO): 15
• Clinical trials: 6

Clinical features

Epidemiology

Prevalence records

1 prevalence record(s), Orphanet:

Type	Class	Value	Geography	Validation
Point prevalence	1-5 / 10 000	15	Europe	Validated

Signs & symptoms

Clinical features (HPO)

15 HPO clinical features (Orphanet curated; top 15 by frequency):

HPO ID	Term	Frequency
HP:0000491	Keratitis	Very frequent (80-99%)
HP:0000958	Dry skin	Very frequent (80-99%)
HP:0001097	Keratoconjunctivitis sicca	Very frequent (80-99%)
HP:0007957	Corneal opacity	Very frequent (80-99%)
HP:0000492	Abnormal eyelid morphology	Frequent (30-79%)
HP:0000498	Blepharitis	Frequent (30-79%)
HP:0000613	Photophobia	Frequent (30-79%)
HP:0000622	Blurred vision	Frequent (30-79%)
HP:0007879	Allergic conjunctivitis	Frequent (30-79%)
HP:0011457	Loss of eyelashes	Frequent (30-79%)
HP:0011496	Corneal neovascularization	Frequent (30-79%)
HP:0030953	Conjunctival hyperemia	Frequent (30-79%)
HP:0200026	Ocular pain	Frequent (30-79%)
HP:0000559	Corneal scarring	Occasional (5-29%)
HP:0012375	Chemosis	Occasional (5-29%)

Identifiers

Disease identifiers

Field	Value
Canonical name	atopic keratoconjunctivitis
Mondo ID	MONDO:0015599
Orphanet	163934
ICD-11	1941631830
SNOMED CT	403434009
UMLS	C1274788
MedGen	698110
GARD	0020053
MedDRA	10069664
Is cancer (heuristic)	no

Disease family

This is a subtype of eye disorder. 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 › disorder of orbital region › eye disorder › atopic keratoconjunctivitis

Related subtypes (119): ptosis, eye accommodation disease, corneal disorder, asthenopia, lens disorder, keratomalacia, scleral disorder, ocular siderosis, coloboma, luxation of globe, mucopolysaccharidosis type 1, lacrimal apparatus disorder, Foster-Kennedy syndrome, anterior dislocation of lens, uveal disorder, eyelid disorder, ocular hypotension, scotoma, exophthalmos, ophthalmia nodosa, eye degenerative disorder, refractive error, glaucoma, retinal disorder, eye allergy, ocular vascular disorder, optic neuritis, conjunctival disorder, ocular hypertension, Tietz syndrome, Alagille syndrome, glaucoma-sleep apnea syndrome, Marshall syndrome, microcornea-glaucoma-absent frontal sinuses syndrome, nail-patella syndrome, oculodentodigital dysplasia, piebaldism, Sturge-Weber syndrome, cerebrotendinous xanthomatosis, ocular cystinosis, alpha-mannosidosis, megalocornea-intellectual disability syndrome, mucolipidosis type IV, mucopolysaccharidosis type 6, Netherton syndrome, galactosialidosis, Niemann-Pick disease type A, ocular motor apraxia, Cogan type, Peters plus syndrome, isolated Pierre-Robin syndrome, ectodermal dysplasia-blindness syndrome, Sandhoff disease, SHORT syndrome, Sjogren-Larsson syndrome, Smith-Lemli-Opitz syndrome, Tay-Sachs disease, tyrosinemia type II, Ito hypomelanosis, X-linked cone dysfunction syndrome with myopia, red color blindness, oculocerebrorenal syndrome, Lowry-MacLean syndrome, pigment dispersion syndrome, hereditary hyperferritinemia with congenital cataracts, dyssegmental dysplasia-glaucoma syndrome, mevalonic aciduria, familial cavitary optic disk anomaly, blindness - scoliosis - arachnodactyly syndrome, fatty acyl-CoA reductase 1 deficiency, microcephaly-intellectual disability-sensorineural hearing loss-epilepsy-abnormal muscle tone syndrome, neurotrophic keratopathy, Cogan syndrome, rhizomelic chondrodysplasia punctata, Ehlers-Danlos syndrome, kyphoscoliotic type 1, IRVAN syndrome, Rothmund-Thomson syndrome type 2, microcornea-corectopia-macular hypoplasia syndrome, isolated anophthalmia-microphthalmia syndrome, Spasmus nutans, toxic maculopathy due to antimalarial drugs, syndromic recessive X-linked ichthyosis, acute zonal occult outer retinopathy, acute annular outer retinopathy, phakomatosis pigmentovascularis, lamellar ichthyosis, idiopathic linear interstitial keratitis, chondroectodermal dysplasia with night blindness, galactosemia, GM1 gangliosidosis, Gaucher disease, visual snow syndrome, extensive peripapillary myelinated nerve fibers, IgG4-related ophthalmic disorder, global developmental delay-visual anomalies-progressive cerebellar atrophy-truncal hypotonia syndrome, vernal keratoconjunctivitis, Gardner syndrome, anterior segment dysgenesis, isolated ankyloblepharon filiforme adnatum, hereditary optic neuropathy, essential strabismus, Axenfeld anomaly, eye neoplasm, isolated blepharochalasis, punctate inner choroidopathy, eye infectious disorder, vitreous body disorder, 9q33.3q34.11 microdeletion syndrome, autoimmune/inflammatory optic neuropathy, LTBP2-related ocular dysgenesis, ocular growth disorder, ocular dysgenesis caused by defects in PAX6 regulation, choroidal neovascularization, anterior segment developmental abnormality with extraocular manifestations, congenital optic disk excavation, neuroocular syndrome, isolated angioid streaks, multiple evanescent white dot syndrome, stellate multiform amelanotic choroidopathy, macular telangiectasia

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: 6.

Phase distribution (across all retrieved trials)

Phase	Trials
Not specified	3
PHASE3	1
PHASE2	1
PHASE1	1

Top trials by phase / activity

NCT	Phase	Status	Title
NCT07395232	PHASE3	RECRUITING	An Efficacy and Safety Study of DFL24498 in the Treatment of AKC
NCT04296864	PHASE2	UNKNOWN	Evaluation of Dupilumab in Patients With Atopic Keratoconjunctivitis (AKC)
NCT03379311	PHASE1	COMPLETED	A Study of AK002 in Patients With Atopic Keratoconjunctivitis, Vernal Keratoconjunctivitis, and Perennial Allergic Conjunctivitis
NCT04973384	Not specified	ACTIVE_NOT_RECRUITING	Ocular Comorbidity in Atopic Dermatitis
NCT00987467	Not specified	COMPLETED	Long-term Topical Cyclosporine for Atopic Keratoconjunctivitis
NCT03557203	Not specified	UNKNOWN	Dermatologic Tacrolimus Ointment on Eyelids in the Treatment of Refractory Vernal Keratoconjunctivitis and Atopic Keratoconjunctivitis

Drugs tested across these trials (top 30)

Molecule	Max phase	Trials referencing
LIRENTELIMAB	3	1
VEHICLE	0	1