History of Present Illness
“My rheumatologist asked me to have an eye examination.” A 6-year-old girl with a recent diagnosis of juvenile idiopathic arthritis (JIA) is referred to an ophthalmologist for a routine eye examination. The child has no complaints, and the parents have not noticed any problem with her vision or eyes. She was diagnosed with JIA at 4 years of age, and previous eye examinations every 3 months have been normal. Her joint symptoms have been controlled with oral methotrexate (15 mg/m 2 ) weekly ( Fig. 10.1 ).
| OD | OS | |
|---|---|---|
| Visual acuity | 20/20 | 20/40 |
| Intraocular pressure (IOP) (mm Hg) | 12 | 10 |
| Sclera/conjunctiva | Clear, no injection | Clear, no injection |
| Cornea | Clear with nongranulomatous (NG) keratic precipitates (KPs) in Arlt triangle | Clear with NG KPs in Arlt triangle |
| Anterior chamber (AC) | 1+ flare, 1+ cell | 1+ flare, 3+ cell |
| Iris | Round pupil | Intermittent posterior synechiae (see Fig. 10.1 ) |
| Lens | Clear | Clear |
| Vitreous cavity | Clear | 2+ vitreous cells |
| Retina/optic nerve | Normal | Swollen optic nerve head with normal retina |
Questions to Ask
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Has her vision changed in either eye since seeing her ophthalmologist?
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What diagnostic tests has she had to confirm her diagnosis of JIA?
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Has anyone in her family had a viral infection like herpesvirus, measles, or mumps?
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Does she have a skin rash?
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Has she been hospitalized for any serious infections, or has she been exposed to anyone with a serious infection like tuberculosis (TB)?
Her vision has not changed before seeing her eye doctor until recently. She had several blood tests to establish a diagnosis of JIA, but the family does not know the exact results. No one in the family has had a recent viral infection, and the patient does not have a skin rash and has not been hospitalized for a serious infection.
Assessment
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Anterior uveitis both eyes (OU), with optic disc edema and cystoid macular edema (CME) left eye (OS)
Differential Diagnosis
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JIA-associated anterior uveitis
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Infectious anterior uveitis (herpes simplex virus, varicella zoster virus, cytomegalovirus, TB, borreliosis, syphilis)
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Intermediate uveitis (pars planitis)
Working Diagnosis
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JIA-associated anterior uveitis OU, OS > OD (right eye)
Testing
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Antibody testing:
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Antinuclear antibodies (ANA) positive, antineutrophil cytoplasmic antibodies (ANCA) negative
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Rheumatoid factor (immunoglobulin M [IgM]-RF): negative
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HLA-B27: negative
In patients with an established diagnosis of JIA, no further workup is necessary. The presence of optic disc edema OS is secondary to the intraocular inflammation in that eye. If inflammation in the OS resolves and optic disc edema persists for several months, then further evaluation is required. For atypical cases of anterior uveitis, the potential infectious causes, noted earlier, should be further investigated by detailed history and laboratory testing as indicated. Coordination with a pediatric rheumatologist is strongly encouraged.
Management
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Topical corticosteroid (e.g., prednisolone acetate suspension 1%), OU, every 4 to 6 hours (q4–6h) while awake
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Topical cyclopentolate hydrochloride 0.5%, 1 qtt, OU twice a day to three times a day (BID to TID)
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Follow up in 2 weeks
Follow-up Care
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The patient returned after 2 weeks without a noticeable improvement. She was continued on topical corticosteroid and cycloplegic therapy OU and changed from oral to subcutaneous methotrexate (15 mg/m 2 ) to increase bioavailability.
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Follow up in 1 month.
Follow-up
The patient returns with improved visual acuity (VA) OS to 20/25, no anterior chamber (AC) inflammation OU, and resolving optic disc edema OS. Topical corticosteroids were slowly tapered OU, and cycloplegia was stopped.
Key Points
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Uveitis screening by an ophthalmologist has to start immediately after a diagnosis of JIA, and screening should be continued throughout childhood at appropriate intervals. A recent study of JIA concluded that almost all children developed uveitis within 4 years of diagnosis after arthritis onset and that the most important predictor for the development of uveitis was ANA positivity.
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Many patients with JIA will present with asymmetric disease, including the presence of optic disc edema. The latter does not require further evaluation unless it persists after the resolution of intraocular inflammation.
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As much as 80% of anterior uveitis cases in pediatrics are associated with JIA. The cumulative incidence of JIA-associated uveitis is 12.4% in the oligoarticular group, 4.3% in the polyarticular group, and 1.8% in the systemic group.
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Three major complications of JIA-associated anterior uveitis are band keratopathy ( Fig. 10.2 ), cataract, CME, and glaucoma. Cataract surgery is technically demanding, but “small incision surgery” and control of perioperative inflammation can result in satisfactory surgical outcomes. Persistent CME without active inflammation can be treated with periocular or intravitreal corticosteroids or intravitreal anti–vascular endothelial growth factor (VEGF) agents.
