Atopic Keratoconjunctivitis (AKC)

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Aetiology

Severe and potentially sight-threatening allergic eye disease characterised by chronic inflammation of the ocular surface

Complex  immunopathology including T-cell mediated (type 4 hypersensitivity)
Symptoms of AKC typically begin in the late teens or early twenties and can persist until the fourth or fifth decade of life. The peak incidence of AKC occurs between the ages of 30 and 50 years

Sometimes follows childhood Vernal Keratoconjunctivitis (VKC) (see Clinical Management Guideline on Vernal Keratoconjunctivitis)

Predisposing factors

The majority of patients have a personal history of asthma, and eczema
(atopic dermatitis)
There may also be a family history of atopic disease
Most patients have eczema affecting the eyelids and periorbital skin
There is a strong association with staphylococcal lid margin disease
Specific allergens may exacerbate the condition

Symptoms of atopic keratoconjunctivitis

Ocular itching, burning, watering, usually bilateral
Blurred vision, photophobia
White stringy mucoid discharge
Onset of ocular symptoms may occur several years after onset of atopy
Symptoms are typically bilateral, occur year-round, with exacerbations

Signs of atopic keratoconjunctivitis

Eyelids may be thickened, crusted and fissured
Associated chronic staphylococcal blepharitis
Tarsal conjunctiva: giant papillary hypertrophy, subepithelial fibrosis, scarring and shrinkage
Entire conjunctiva hyperaemic
Limbal inflammation
Corneal involvement is common and may be sight-threatening: beginning with punctate epitheliopathy that may progress to macro-erosion, plaque formation (usually upper half), progressive corneal subepithelial scarring, neovascularisation/pannus, thinning, and rarely spontaneous perforation 
These patients are prone to develop herpes simplex keratitis (which may
be bilateral), corneal ectasia such as keratoconus, atopic (anterior or posterior polar) cataracts, retinal detachment 

Differential diagnosis

Vernal Keratoconjunctivitis (there is no consensus for distinguishing VKC and AKC in children)
Other causes of chronic allergic conjunctivitis, e.g. Giant Papillary Conjunctivitis (GPC) (often contact lens-related)
Toxic Keratoconjunctivitis

Management by optometrist

Practitioners should recognise their limitations and where necessary seek further advice or refer the patient elsewhere 

Non pharmacological 

Lid hygiene and treatment of associated staphylococcal blepharitis (see Clinical Management Guideline on Blepharitis)
Cool compresses
Ocular lubricants for symptomatic relief (drops for use during the day,
unmedicated ointment for use at bedtime)
Advise avoidance of specific allergens if known, e.g. elimination of pets and carpeting, where necessary; instillation of air filtering devices and alterations to bedding materials
(GRADE*: Level of evidence=low, Strength of recommendation=strong)

Pharmacological 

Mild cases:

Systemic antihistamines e.g. cetirizine
Topical mast cell stabilisers, e.g. gutt. sodium cromoglicate 2%, gutt. lodoxamide 0.1%, gutt, or dual acting agents e.g. gutt. olopatadine 0.1%, gutt ketotifen 0.025%, (off-label use may provide symptomatic relief)
Because of the sight-threatening nature of this condition, and the need for multidisciplinary specialist involvement, maintain a low threshold for referral
(GRADE*: Level of evidence=low, Strength of recommendation=weak)

Management category

Severe corneal complications are common and potentially sight-threatening.

If corneal epithelial macro-erosion or plaque are present:
A3: First aid measures followed by urgent referral (within one week) to an ophthalmologist

Milder cases (without active corneal involvement):
B1: Possible prescription of drugs; routine referral

Possible management by ophthalmologist

AKC often requires multidisciplinary management (ophthalmology, dermatology, immunology) requiring ongoing use of topical anti-inflammatory eye drops and intermittent use of systemic medication.
Topical steroids with monitoring and management of complications, e.g. steroid glaucoma and cataract
Topical/systemic antibiotics for lids
Topical immunosuppression (e.g. ciclosporin, tacrolimus)
Treatment of facial eczema and atopic blepharitis
Surgery for atopic cataract

Evidence base

*GRADE: Grading of Recommendations Assessment, Development and Evaluation (see www.gradeworkinggroup.org)

Sources of evidence

Brémond-Gignac D, Nischal KK, Mortemousque B, Gajdosova E, Granet DB, Chiambaretta F. Atopic Keratoconjunctivitis in Children: Clinical Features and Diagnosis. Ophthalmology. 2016;123(2):435-7

Chen JJ, Applebaum DS, Sun GS, Pflugfelder SC. Atopic keratoconjunctivitis: A review. J Am Acad Dermatol. 2014;70(3):569-75.

González-López JJ, López-Alcalde J, Morcillo Laiz R, Fernández Buenaga R, Rebolleda Fernández G. Topical cyclosporine for atopic keratoconjunctivitis. Cochrane Database of Systematic Reviews 2012;9:CD009078

Kim SE, Nowak V, Quartilho A, Larkin F, Hingorani M, Tuft S, Dahlmann-Noor A. Systemic interventions for severe atopic and vernal
keratoconjunctivitis in children and young people up to the age of 16 years. Cochrane Database Syst Rev. 2020;10(10):CD013298.

Nivenius E, Montan P. Spontaneous corneal perforation associated with atopic keratoconjunctivitis: a case series and literature review. Acta Ophthalmol. 2015;93(4):383-7

Patel N, Venkateswaran N, Wang Z, Galor A. Ocular involvement in atopic disease: a review. Curr Opin Ophthalmol. 2018;29(6):576-581

Power WJ, Tugal-Tutkun I, Foster CS. Long-term follow-up of patients with atopic keratoconjunctivitis. Ophthalmology. 1998;105:637-42

Zhan H, Smith L, Calder V, Buckley R, Lightman S. Clinical and immunological features of atopic keratoconjunctivitis. Int Ophthalmol Clin. 2003;43(1):59-71

Summary

What is Atopic keratoconjunctivitis?

Atopic keratoconjunctivitis is a chronic (long-lasting) allergic condition of the eyelids and front surface of the eye. It affects many patients who have the skin condition, atopic dermatitis (eczema). 

How is Atopic keratoconjunctivitis managed?

Atopic keratoconjunctivitis needs long-term treatment to prevent complications such as scarring of the cornea (the clear window at the front of the eye), which can damage sight. In the early stages of the disease, symptoms (e.g. itching or burning) can be controlled by standard anti-allergy drugs combined with the application of a cool compress to the closed eyes. However, courses of steroid eye drops can be needed when symptoms are more severe. Cases that do not respond to steroids, or need steroid eye drops for long periods, may benefit from ciclosporin or tacrolimus eye drops or ointment. Many cases need to be referred to the ophthalmologist for management, who in turn may refer them to specialists in other allergic diseases.

Atopic Keratoconjunctivitis (AKC)
Version 16
Date of search 08.12.22
Date of revision 23.02.23
Date of publication 29.03.23
Date for review 07.12.24
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