Atopic Keratoconjunctivitis (AKC)

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Aetiology 

Severe ocular surface disease affecting some atopic individuals

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

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 usually year-round, with exacerbations

Signs 

Eyelids may be thickened, crusted and fissured
Associated chronic staphylococcal blepharitis
Tarsal conjunctiva: giant papillary hypertrophy, subepithelial 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, thinning, and rarely spontaneous performation 
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
Other 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
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 

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-licence use; maximum duration 4 months) may also provide symptomatic relief
(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

Often requires multidisciplinary management (ophthalmology, dermatology, immunology)
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 http://www.gradeworkinggroup.org/index.htm)

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

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

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

Lay summary

Atopic keratoconjunctivitis is a chronic (long-term) allergic condition of the eyelids and front surface of the eye. It is present in a high percentage of patients who have the skin condition, atopic dermatitis. Atopic keratoconjunctivitis requires long-term treatment to prevent sight-threatening complications such as scarring of the cornea (the clear window at the front of the eye). In the early stages of the disease, symptoms can be controlled by standard anti-allergy drugs. However, short- term use of steroid eye drops is often required when symptoms are severe. There is some evidence that cases that do not respond to steroids, or those requiring steroids eye drops long term, 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 14
Date of search 23.11.18 
Date of revision 26.11.18
Date of publication 09.05.19
Date for review 22.11.20
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