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]

Question 2 (10 marks)

Discuss the management of the chronic uveitis and contrast it with the ocular screening of the child with the type one diabetes and the neurofibromatosis type one. [1]

A full-mark answer reproduces the stepwise uveitis treatment and the two contrasting screening schedules. [1][2][4]

The stepwise treatment of the chronic uveitis (4 marks). The treatment builds in the stepwise fashion the boards reward. The topical corticosteroid, the prednisolone acetate one percent, is the first line for the acute inflammation, and the cycloplegic prevents the posterior synechiae. The systemic treatment is added when the topical steroid fails to control the inflammation or when the steroid-induced glaucoma and cataract threaten the sight, and the ACR guideline strongly recommends the methotrexate, at ten to fifteen milligrams per square metre once weekly, as the first-line steroid-sparing agent. The adalimumab, the anti-tumour-necrosis-factor monoclonal antibody, is the standard second-line biologic when the methotrexate fails, approved for the juvenile arthritis uveitis and proven to control the inflammation and to spare the steroid. [1]

The contrast with the type one diabetes (3 marks). The diabetic retinopathy is the microvascular complication, and it differs from the uveitis in the mechanism and the schedule. The retinopathy is screened annually from age eleven after two to five years of the type one diabetes, with the dilated fundus examination or the retinal photography, because the retinopathy is rare before five years of duration but rises steeply through the teenage years. The tight glycaemic and blood-pressure control prevents the progression, and the laser treats the proliferative disease. The annual interval contrasts with the three-monthly interval of the juvenile arthritis, because the retinopathy is slower and the diabetes declares the risk through the duration and the control. [2][7]

The contrast with the neurofibromatosis type one (3 marks). The neurofibromatosis type one carries the optic pathway glioma, the low-grade pilocytic astrocytoma of the optic nerve, the chiasm and the hypothalamus, which develops in roughly fifteen percent of the children and is the commonest central nervous system tumour of the disease. The annual ophthalmology review with the visual acuity, the colour vision and the fundus is performed through the childhood, with the surveillance magnetic resonance imaging of the orbits and the brain in the first years of life, because the glioma may grow silently before it compresses the visual pathway. The reduced vision, the proptosis or the precocious puberty is the red flag that demands the urgent imaging. The contrast is the developmental tumour versus the immune inflammation, and the annual review versus the three-monthly slit-lamp. [4][6]