NORD gratefully acknowledges Stefano Bonini, MD, Professor and Chairman, Department of Ophthalmology, University of Rome Campus BioMedico, for assistance in the preparation of this report.
VKC symptoms include inflammation of the outer membrane of the eye. This causes the eyes to become red and may cause blurred vision. The eyes become sensitive to light and itch intensely. Usually both eyes are affected, and cobblestone-like changes appear in the upper eyelid linings (palpebral conjunctiva). In other patients, a gelatinous nodule may develop in the tissue adjacent to the cornea (limbus). In very severe cases, the corneas may scar (shield ulcers) or clouding of the lens (cataract) may occur, leading to temporarily or permanently reduced vision.
The cause of VKC is a hypersensitivity or allergic reaction of the eyes to airborne allergens. The pathogenesis of ocular allergies is related to a complex exchange of information between tissues through cell-to-cell communications, chemical mediators, cytokines, and adhesion molecules. It is also possible that the neural and endocrine systems may influence ocular allergic responses.
The longer a patient suffers from seasonal VKC, the more likely he or she is to develop the disease chronically. That is why it is important to obtain a swift diagnosis and treat the disease as soon as possible.
Onset of VKC typically occurs at about 11 years of age, but onset can be between 3 and 25 years of age. The disorder appears to affect more males than females. Usually patients with VKC have a family history of atopic diseases, such as asthma, eczema, or rhinitis. It occurs most often during the spring or summer but up to 60% of patients experience recurrences in the winter periodically. It is more present in climates that are dry and warm.
The diagnosis of VKC is usually straightforward and can almost always be diagnosed based on signs and symptoms. However, atypical presentations or patients that experience incomplete forms of VKC may have a more difficult time obtaining a diagnosis. Having asthma or dermatitis also helps support the diagnosis of VKC.
Clinical Testing and Work-Up
Difficult cases of VKC can be diagnosed and treated by conjunctival scraping, demonstrating the presence of infiltrating eosinophils.
VKC usually subsides at the onset of puberty, and treatment is both preventive and therapeutic. To prevent flare-ups, the agent that causes the allergy should be avoided if possible. Wearing dark sunglasses in the daytime, avoiding dust, and not going out on hot afternoons are also recommended. Mast cell stabilizer eye drops can be used at the beginning of the season or the first sign of a flare-up to prevent severe symptoms.
Topical eye drops are generally preferred as the first source of treatment. Cold compresses and artificial tears and ointments also may soothe, lubricate and dilute the antigen. Topical antihistamines producing constriction of the blood vessels and ducts may help. Mast-cell stabilizers may prevent further flare-ups of the disorder or may help control a flare-up, but do little to reduce the symptoms of VKC. Non-steroid anti-inflammatories (NSAIDS) may relieve symptoms in moderate cases but topical steroids should be reserved for more severe cases. Topical steroid preparations are the most effective therapy for moderate to severe forms of VKC; however, their use should be carefully monitored because long-term use can cause glaucoma. Cyclosporine eye drops can also be useful.
It is important to begin treatment of VKC immediately upon receiving a diagnosis because the longer a patient suffers with the disease, there is a decrease in visual function and the chances of developing cataracts or permanent blindness increases.
A few prescription drugs are also available for the treatment of VKC, such as the orphan drug cromolyn sodium. Cromolyn has been shown to reduce signs and symptoms of VKC.
The orphan products lodoxamide tromethamine (Alomide ophthalmic solution) and Levocabastine have been approved by the US Food and Drug Administration (FDA) for treatment of VKC.
Oral administration of montelukast (Singulair), a drug usually prescribed for asthma, has been shown to be an effective treatment of VKC.
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Bonini S, Coassin M, Aronni S, and Lambiase A. Vernal keratoconjunctivitis. Eye. 2004: 18, 345-351. http://www.nature.com/eye/journal/v18/n4/full/6700675a.html
Leonardi A, Secchi AG. Vernal keratoconjunctivitis. Int Ophthalmol Clin. 2003;43:41-58.
Bielory L, Mongia A. Current Opinion of immunotherapy for ocular allergy. Curr Opin Allergy Clin Immunol. 2002;2:447-52.
Bielory L Kempuraj D, Theoharides T. Topical immunopharmacology of ocular allergies. Curr Opin Allergy Clin Immunol. 2002;2:435-45.
Trocme SD, Sra KK. Spectrum of ocular allergy. Curr Opin Allergy Clin Immunol. 2002;2:423-27.
Friedlander MH. Conjunctival provocation testing: overview of recent clinical trials in ocular allergy.. Curr Opin Allergy Clin Immunol. 2002;2:413-17.
M-Eye Conditions. University of Michigan, Kellogg Eye Center. Vernal Keratoconjunctivitis. http://www.kellogg.umich.edu/patientcare/conditions/vernal.html Accessed February 1, 2017.
Chang-Godinich A. Medscape. Atopic Keratoconjunctivitis. Last Update March 17, 2015 http://emedicine.medscape.com/article/1194480-overview Accessed February 1, 2017
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