Rheumatic Inflammatory Eye Diseases of Childhood

Chronic inflammatory eye diseases are a common manifestation of pediatric rheumatologic diseases, potentially leading to lifelong vision impairment and disability. The mechanisms leading to the breach of the blood eye/brain barrier and the subsequent immune attack against a variety of intraocular mostly unidentified antigens remains poorly understood. Pediatric rheumatologists need to be familiar with the various inflammatory eye diseases because they are often responsible for selecting and supervising treatment in close collaboration with the ophthalmologist. This article provides an update of recent developments in the pathogenesis and treatment of the most relevant ocular diseases encountered in rheumatologic practice.

Key points

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Vision-threatening complications of pediatric uveitis are significant.
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The clinical presentation of inflammatory eye diseases in children is often asymptomatic, leading to a delay in diagnosis and causing a distinct challenge for treatment.
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A close collaboration with ophthalmologists and pediatric rheumatologists remains essential to improve the long-term outcome of these patients.

Introduction

Chronic inflammatory eye diseases (IEDs) are a common manifestation of pediatric rheumatologic diseases, potentially leading to lifelong vision impairment and disability. The mechanisms leading to the breach of the blood eye/brain barrier and the subsequent immune attack against a variety of intraocular mostly unidentified antigens remains poorly understood.

Pediatric rheumatologists need to be familiar with the various IEDs because they are often responsible for selecting and supervising treatment in close collaboration with the ophthalmologist. This article provides an update of recent developments in the pathogenesis and treatment of the most relevant ocular diseases encountered in rheumatologic practice.

Chronic inflammatory and degenerative eye diseases in children consist of a wide array of infectious, traumatic, malignant, or autoimmune conditions including conjunctivitis, keratitis, scleritis, and uveitis. Noninfectious uveitis remains one of the leading causes of preventable, irreversible vision loss, particularly in the Western world. Most chronic, vision-threatening inflammatory eye conditions are autoimmune in nature. Because initial symptoms are often subtle, patients are frequently unaware of a disease process and do not consult an ophthalmologist until they experience visual impairment. This aspect is of particular importance in children since children have a higher rate of posterior uveitis which is associated with a higher risk for visison loss. Complications from chronic eye disease or its treatment such as band keratopathy, synechiae, cataracts, glaucoma, and retinal edema can further compromise visual acuity and severely affect quality of life. Table 1 summarizes the more common ocular manifestations in rheumatologic diseases.

Table 1

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