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Ocular Manifestations of Kawasaki Disease

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The ocular signs of KD are bilateral bulbar conjunctivitis without exudate, superficial punctate keratitis, uveitis, or vitreous opacities.

Written by

Dr. Palak Jain

Medically reviewed by

Dr. Asha Juliet Barboza

Published At December 14, 2023
Reviewed AtDecember 14, 2023

Introduction

Acute, self-limiting medium vessel pediatric vasculitis known as Kawasaki disease (KD) or mucocutaneous lymph node syndrome. It is the most prevalent cause of acquired heart disease in children living in wealthy nations because it has a special preference for the coronary arteries.

Anterior segment lesions are frequently seen in the different ocular symptoms of Kawasaki disease. Kawasaki disease is a condition that has the potential to be lethal since it can cause myocarditis, aneurysm development and rupture, myocardial infarction (MI), and unexpected death. Kawasaki disease most frequently affects young children between the ages of 6 months and 4 years, with Asian ancestry having the highest prevalence. However, it can appear at any age and is sometimes observed in adults.

What Is Kawasaki Disease?

A disorder that causes swelling in the walls of various blood vessels in the body. Infants and small children are most frequently affected. A rash and fever are symptoms of the early stages. High temperatures and skin rashes are symptoms. Vasculitis, an inflammation of medium-sized blood vessels, may develop in the latter stages. Lymph nodes, skin, and mucous membranes within the mouth are also affected. The majority of the time, Kawasaki's illness is curable. Aspirin and intravenous immunoglobulin therapy are two initial therapies administered at a hospital to treat the disease.

What Is the Pathophysiology Associated With Kawasaki Disease?

It primarily affects muscle arteries, frequently with a preference for the coronary arteries, and is classified as a pediatric medium artery vasculitis called Kawasaki disease. The stimulation of B-cells inside the related lymphoid tissue, which leads to their differentiation into IgA-producing plasma cells, is hypothesized to occur after inhalation or ingestion of an unknown pathogen.

These plasma cells circulate and harm artery walls through infiltration coupled with T-cells, neutrophils, macrophages, and eosinophils. Aneurysms reduce structural integrity, and dilated vessels are caused by these infiltrating cells' aberrant activation, which amplifies the inflammatory response within artery walls.

The systemic inflammatory response in Kawasaki disease impacts the extraocular muscles, the conjunctiva, cornea, sclera, uvea, vitreous, retina, and any vasculature in or around the eye. It was believed that acute bilateral conjunctivitis and the occurrence of a skin rash were causally connected because the inflammatory pathways in the conjunctiva and skin are similar. Histology of conjunctival swabs collected from patients during the acute phase of the disease occasionally reveals an abundance of neutrophils around conjunctival epithelial cells, known as "neutrophilic rosetting."

What Are the Ocular Manifestations of Kawasaki Disease?

A key characteristic of Kawasaki disease is its ocular appearance.

The following are the main features of KD's ocular symptoms, which are often restricted to the anterior region:

  • Bilateral bulbar conjunctivitis without exudate.

  • Superficial punctate keratitis.

  • Uveitis.

  • Vitreous opacities.

What Is Bulbar Conjunctivitis?

Bulbar conjunctivitis is an infection of the conjunctiva, the thin, transparent tissue that covers the white area of the eye. It can produce eye redness, inflammation, and discharge. The cause is frequently unclear; nevertheless, it can occur with several inflammatory illnesses, such as Kawasaki disease.

The following are some of the warning signs and symptoms of this condition:

  • Eye redness.

  • Irritation and itching.

  • Watery or slick discharge.

  • Sensitivity to light.

  • Blurred vision.

The therapy for bulbar conjunctivitis depends on the illness's underlying cause. A variety of treatment choices, including:

  • Antibiotic and antiviral eye drops or ointments.

  • Anti-inflammatory eye drops or ointments.

  • Allergy medication.

  • Artificial tears.

  • Warm compresses.

What Is Superficial Punctate Keratitis?

Superficial punctate keratitis is a corneal inflammation characterized by dispersed, fine, punctate corneal epithelial loss or damage. The eyes are often sore, watery, sensitive to bright light, bloodshot, and painful in superficial punctate keratitis. Vision may also be somewhat impaired. There is frequently a burning, gritty sensation or the sensation that something alien is lodged in the eye. The symptoms, whether the patient has been exposed to any known causes, and a slit lamp examination of the cornea (a device that allows a doctor to examine the eye under high magnification) are used to diagnose superficial punctate keratitis. The physician may use fluorescein eye drops, and yellow-green dyes, during the examination. Damaged corneal tissue is momentarily briefly stained by fluorescein, allowing for the observation of invisible damage.

What Is Uveitis?

An instance of eye inflammation is uveitis. It impacts the uvea, the middle layer of tissue in the eye wall. Uveitis warning signals frequently appear abruptly and swiftly worsen. They consist of discomfort, pain in the eyes, and blurred vision. The illness can affect people of all ages, including children, and impair one or both eyes. An infection, an injury, or an autoimmune or inflammatory condition may bring on Uveitis. There are many situations where no reason can be found. Uveitis can cause irreversible visual loss and be quite dangerous. To avoid problems and maintain eyesight, early diagnosis and treatment are crucial.

Uveitis may show the following signs, symptoms, or traits:

  • Redness of the eyes.

  • Eye discomfort.

  • Responsiveness to light.

  • Hazy vision.

  • Decrease in eyesight.

To avoid long-term issues, uveitis has to be treated soon. Corticosteroids, an eyedrop medication that lessens inflammation, are frequently used by ophthalmologists to treat uveitis. They could also apply an eye drop to dilate (widen) the pupil, easing discomfort and swelling. Sometimes it may be necessary to provide medicine orally or through injection (shots).

Conclusion

Although it sporadically affects adults, Kawasaki disease (KD) primarily affects children and is a vasculitis. Conjunctivitis, mucositis, a skin rash, changes in the extremities, and cervical lymphadenopathy are additional signs that have been present for more than five days.

Although the cause of KD is unknown, the consensus holds that genetically vulnerable children exposed to a pathogen (most likely a virus) would experience immune system dysregulation due to an inflammatory cascade that leads to inflammation. Ocular abnormalities appear in more than 90 percent of Kawasaki disease patients. The most frequent condition (90 percent) is conjunctivitis, which usually appears a few days after the fever starts, is bilateral, bulbar, nonexudative, limbic sparing, and bilateral. The existence of a skin rash is closely tied to it. Early diagnosis and prompt treatment help the individual.

Dr. Asha Juliet Barboza
Dr. Asha Juliet Barboza

Ophthalmology (Eye Care)

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keratitisconjunctivitiskawasaki diseaseuveitis
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