Ophthalmic manifestations of systemic disease: Case

Framing the case

This four-year-old girl has the oligoarticular juvenile idiopathic arthritis with a positive antinuclear antibody, and the framework that organises the case is the silent sight-threatening uveitis and the ACR screening schedule. The swollen knee is the visible problem, but the danger sits behind the iris in the anterior chamber, where the low-grade inflammation may scar the eye while the child plays. The first priority is the slit-lamp screening by the ophthalmology service at the every-three-months interval, because the uveitis produces no eye symptom and the slit-lamp is the only detection. [1]

Confirming and screening for the uveitis

The uveitis is screened with the slit-lamp examination by the ophthalmologist, and the slit-lamp detects the cells and the flare of the anterior chamber inflammation, the keratic precipitates on the corneal endothelium, the posterior synechiae between the iris and the lens, and the band keratopathy of the chronic disease. The intraocular pressure is measured, because the uveitis and the steroid both raise the pressure and the secondary glaucoma is the complication. This girl carries the four high-risk factors, the oligoarticular disease, the positive antinuclear antibody, the onset under seven years and the short duration, and the ACR guideline schedules the slit-lamp every three months. [1]

The stepwise management

The treatment of the chronic uveitis builds in the stepwise fashion. 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, with the folate supplementation and the full blood count and the liver function monitoring. The adalimumab, the anti-tumour-necrosis-factor monoclonal antibody, is the standard second-line biologic when the methotrexate fails. The co-management with the rheumatology and the ophthalmology continues through the childhood, because the uveitis may flare in the quiet joint. [1]

Contrasting the type one diabetes

The examiner asks the candidate to contrast this child with a teenager with the type one diabetes. The diabetic retinopathy is the microvascular complication of the chronic hyperglycaemia, 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 declared by the duration. The sickle retinopathy is screened annually from age ten, and the candidate who names the contrast demonstrates the reasoning the boards reward. [2][7]

Contrasting the neurofibromatosis optic pathway glioma

The examiner asks the candidate to contrast this child with a child with the neurofibromatosis type one. The optic pathway glioma is 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 and the hypothalamus. 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 candidate who holds the two together demonstrates the breadth. [4][6]

Communication and the family

The family is counselled honestly and with the realistic information about the arthritis and the uveitis. The candidate names the chronic anterior uveitis, explains that it is silent and sight-threatening, and that the slit-lamp every three months is the safeguard against the irreversible blindness. The parents are introduced to the multidisciplinary team of the rheumatology and the ophthalmology, given a written screening schedule, taught the importance of the adherence to the slit-lamp interval, and supported by the social work and the educational liaison. The methotrexate is begun if the uveitis declares, with the monitoring and the folate, and the adalimumab is reserved for the refractory disease. The transition to the adult care is planned in the adolescence, because the ocular manifestations persist into the adulthood. [1][9]