Vernal Keratonconjunctivitis

Vernal Keratonconjunctivitis

National Organization for Rare Disorders, Inc.

Important

It is possible that the main title of the report Vernal Keratonconjunctivitis is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

  • Spring Ophthalmia
  • VKC

Disorder Subdivisions

  • None

General Discussion

Vernal keratoconjunctivitis (VKC) is a chronic, non-contagious allergic disorder with seasonal recurrences usually appearing during the spring or warm weather. VKC is caused by a hypersensitivity to airborne-allergens. It usually affects younger members of the population, ages 3-25 and most patients are males. Major symptoms include itching, sensitivity to light (photophobia) and redness. Signs consist of inflammation of the mucous membrane lining the inside of the eyelid (conjunctiva) and the outer coat of the eyeball (sclera); hard, cobblestone-like bumps (papillae) on the upper eyelid; and stringy or mucous discharge.

Symptoms

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 cases, 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.

Causes

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.

Affected Populations

Onset of VKC typically occurs between ages 3 and 25 years. 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.

Standard Therapies

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.



Treatment

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.



It is important to begin treatment of VKC immediately upon receiving a diagnosis because the longer a patient suffers with the disease, 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. For more information about cromolyn, please contact:



Pharmascience

6111 Ave Royalmount, Suite 100

Montréal

Quebec

H4P 2T4

Canada



Phone: (514) 340-9800

medinfo@pendopharm.com



The orphan product lodoxamide tromethamine (Alomide Ophthalmic Solution) has also been approved for treatment of vernal keratoconjunctivitis. The product is manufactured by:



Alcon Laboratories

6201 South Freeway

Ft. Worth, TX 76134



The orphan drug Levocabastine has also been approved by the FDA for the treatment of this disorder. For more information on this drug, physicians may contact:



Iolab Pharmaceuticals

500 Iolab Drive

Claremont, CA 91711



Oral administration of montelukast (Singulair), a drug usually prescribed for asthma, has been shown to be an effective treatment of VKC. For more information on Montelukast, please contact Merck:



One Merck Drive

P.O. Box 100

Whitehouse Station, NJ 08889-0100 USA

Phone: 908-423-1000

Monday-Friday 8:30 AM - 5:30 PM ET\

Phone: (800) 444-2080

Investigational Therapies

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.



For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:



Tollfree: (800) 411-1222

TTY: (866) 411-1010

Email: prpl@cc.nih.gov



For information about clinical trials sponsored by private sources, contact:

www.centerwatch.com

References

TEXTBOOKS

Beers MH, Berkow R, eds. The Merck Manual, 17th ed. Whitehouse Station, NJ: Merck Research Laboratories; 1999:714-16, 1050.



Berkow R., ed. The Merck Manual-Home Edition. Whitehouse Station, NJ: Merck Research Laboratories; 1997:826-27.



Larson DE, ed. Mayo Clinic Family Health Book. New York, NY: William Morrow and Company, Inc; 1996:542-43.



JOURNAL ARTICLES

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.



FROM THE INTERNET

M-Eye Conditions. University of Michigan, Kellogg Eye Center. Vernal Keratoconjunctivitis. http://www.kellogg.umich.edu/patientcare/conditions/vernal.html. Accessed 8/18/11.



Handbook of Ocular Disease Management. Vernal keratoconjunctivitis (VKC).

http://cms.revoptom.com/handbook/oct02_sec2_3.htm Accessed 8/18/11.



Handbook of Ocular Disease Management. Allergic conjunctivitis & Vernal Keratocon-junctivitis (VKC). http://cms.revoptom.com/handbook/SECT2A.HTM Accessed 8/18/11.

Chang-Godinich A. Medscape. Atopic Keratoconjunctivitis. http://emedicine.medscape.com/article/1194480-clinical Updated 6/15/11. Accessed 8/18/11.

Resources

NIH/National Eye Institute

31 Center Dr

MSC 2510

Bethesda, MD 20892-2510

United States

Tel: (301)496-5248

Fax: (301)402-1065

Email: 2020@nei.nih.gov

Internet: http://www.nei.nih.gov/



NIH/National Institute of Allergy and Infectious Diseases

Office of Communications and Government Relations

6610 Rockledge Drive, MSC 6612

Bethesda, MD 20892-6612

Tel: (301)496-5717

Fax: (301)402-3573

Tel: (866)284-4107

TDD: (800)877-8339

Email: ocpostoffice@niaid.nih.gov

Internet: http://www.niaid.nih.gov/



Genetic and Rare Diseases (GARD) Information Center

PO Box 8126

Gaithersburg, MD 20898-8126

Tel: (301)251-4925

Fax: (301)251-4911

Tel: (888)205-2311

TDD: (888)205-3223

Internet: http://rarediseases.info.nih.gov/GARD/



For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders, Inc.® (NORD). Cigna members can access the complete report by logging into myCigna.com. For non-Cigna members, a copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html.

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