Ophthalmic manifestations of systemic disease: SAQ

This child has the oligoarticular juvenile idiopathic arthritis with a positive antinuclear antibody, and she carries the highest risk of the chronic anterior uveitis of any child in the paediatric clinic, yet her eye examination is normal and she has no eye symptom, because the uveitis is silent. The task is to outline the risk, the screening schedule and the rationale, and then to discuss the management of the chronic uveitis and to contrast it with the ocular screening of the child with the type one diabetes and the neurofibromatosis type one. ^[1]

Question 1 (10 marks)

Outline the ophthalmic risk, the screening schedule and the rationale for this four-year-old girl with the oligoarticular juvenile idiopathic arthritis. ^[1]

A full-mark answer covers the silent sight-threatening uveitis, the high-risk profile, the screening schedule and the rationale. ^[1]

The risk of the silent uveitis (3 marks). The chronic anterior uveitis of the juvenile idiopathic arthritis is the single most important ocular manifestation of any systemic disease in childhood, because it is common, it is silent and it is blinding. Roughly one in six of the children with the juvenile arthritis develops the uveitis, and the inflammation produces no eye pain and no redness in the majority, so the child plays while the low-grade inflammation scars the anterior chamber with the cells and the flare, the keratic precipitates and the posterior synechiae, the band keratopathy, the cataract and the secondary glaucoma. The only safeguard is the slit-lamp screening of the asymptomatic child. ^[1]

The high-risk profile (3 marks). The risk concentrates in the child with the oligoarticular or the polyarticular rheumatoid-factor-negative disease, the positive antinuclear antibody, the young age of onset under seven years and the short disease duration under four years. This girl carries all four factors, and the ACR guideline of twenty-nineteen places her in the highest-risk band, because the oligoarticular antinuclear-antibody-positive disease in the young child is the profile that carries the uveitis in the greatest proportion. ^[1]

The screening schedule and the rationale (4 marks). The ACR guideline schedules the slit-lamp examination every three months for this highest-risk child, because the uveitis may declare itself within the weeks and the three-monthly interval detects the inflammation before it scars the eye. As the risk factors fall away, the interval lengthens to every six months and then to every twelve months, and the screening continues for years after the arthritis remits, because the uveitis may flare in the quiet joint. The slit-lamp is the only detection, the every-three-months schedule is the safeguard, and the rationale is the prevention of the irreversible blindness. ^[1]