Uveitis

Uveitis is inflammation of the iris, ciliary body and choroid, often with involvement of adjacent retina, vitreous and optic nerve. Incidence is 17 to 52 in 100,000 a year. It causes about 10% of preventable blindness in working age adults.

Anatomic classes: anterior (iritis, iridocyclitis), intermediate (vitreous predominant, pars planitis), posterior (choroiditis, retinitis, vasculitis) and panuveitis. Course: acute (under 3 months), recurrent, or chronic (relapse within 3 months of stopping treatment).

Causes: idiopathic (40 to 50%), HLA-B27 related disease, juvenile idiopathic arthritis, sarcoidosis, Behçet disease, tuberculosis, syphilis, herpes simplex and zoster, cytomegalovirus, toxoplasmosis, Vogt-Koyanagi-Harada disease, sympathetic ophthalmia, birdshot chorioretinopathy, multiple sclerosis (intermediate), lymphoma masquerade, and drugs (rifabutin, bisphosphonates, checkpoint inhibitors). Complications: posterior synechiae, band keratopathy, cataract, glaucoma, cystoid macular edema, hypotony, retinal detachment and choroidal neovascularization.

• Anterior: pain, photophobia, redness, small irregular pupil
• Intermediate and posterior: floaters and painless blurring
• Grading of anterior chamber cells in a 1 x 1 mm beam: 0 (under 1), 0.5+ (1 to 5), 1+ (6 to 15), 2+ (16 to 25), 3+ (26 to 50), 4+ (over 50). Flare is graded 0 to 4+. Vitreous haze is graded 0 to 4 by the clarity of fundus detail.
• Systemic clues: back pain, arthritis, oral and genital ulcers, skin lesions, cough, hearing loss and headache
• Red flags: hypopyon, afferent pupillary defect, bilateral disease, vitreous haze, immunosuppression, a young child (painless)

• Slit lamp and dilated fundus examination with cell and haze grading and intraocular pressure measurement.
• Tier 1 tests: treponemal serology and RPR, tuberculosis interferon gamma release assay, chest radiograph, full blood count, urea and creatinine, urinalysis.
• Targeted tests: HLA-B27, serum ACE and lysozyme, chest CT for sarcoidosis, HIV, HLA-A29 (birdshot), antinuclear antibody in children, MRI of sacroiliac joints and brain.
• Ocular imaging: optical coherence tomography for macular edema, fluorescein angiography for vasculitis and leakage, indocyanine green angiography for choroiditis, B-scan for opaque media.
• Aqueous or vitreous sampling for PCR (herpes viruses, toxoplasma) or cytology when infection or lymphoma is suspected.

Infection is treated first, and immunosuppression is added once it is excluded.

Noninfectious, sight threatening disease:

• Topical prednisolone acetate 1% hourly, tapering over 4 to 6 weeks, with cyclopentolate 1% three times daily or atropine 1% daily.
• Periocular triamcinolone acetonide 40 mg sub-Tenon, or intravitreal triamcinolone 4 mg in 0.1 mL.
• Dexamethasone implant 0.7 mg intravitreally every 4 to 6 months, or fluocinolone implant 0.18 mg (about 36 months of effect). Cataract and raised pressure are the main risks.
• Oral prednisolone 1 mg/kg daily (maximum 60 to 80 mg), tapering over 6 to 8 weeks. Add omeprazole 20 mg daily and bone protection.
• Steroid sparing agents when more than 10 mg of prednisolone is needed beyond 3 months or relapses occur: methotrexate 15 to 25 mg orally or subcutaneously weekly with folic acid 5 mg weekly on a different day; mycophenolate mofetil 1 g twice daily; azathioprine 2 to 2.5 mg/kg daily (check thiopurine methyltransferase); ciclosporin 2.5 to 5 mg/kg daily in 2 divided doses (monitor blood pressure and creatinine).
• Adalimumab 80 mg subcutaneously once, then 40 mg every 2 weeks, after screening for tuberculosis and hepatitis B. Infliximab 5 mg/kg intravenously at 0, 2 and 6 weeks, then every 4 to 8 weeks, for Behçet disease.
• Monitor full blood count and liver enzymes every 2 weeks for the first 3 months, then every 3 months.

Infectious causes: syphilis, benzylpenicillin 18 to 24 million units daily intravenously for 10 to 14 days. Tuberculosis, 2 months of rifampicin, isoniazid, pyrazinamide and ethambutol, then 4 months of rifampicin and isoniazid. Herpetic disease, valaciclovir 1 g three times daily or aciclovir 800 mg five times daily.

Children: adalimumab 24 mg/m2 (maximum 40 mg) every 2 weeks for juvenile idiopathic arthritis uveitis. Screen children with positive antinuclear antibody every 3 months for 4 years.

Pregnancy: avoid methotrexate and mycophenolate. Azathioprine, ciclosporin and adalimumab are used.

Raised pressure: timolol 0.5% twice daily and dorzolamide 2% three times daily.

Surgery: cataract extraction after 3 months of quiescence, with prednisolone 0.5 to 1 mg/kg daily for 1 week before and after. Vitrectomy for diagnostic sampling, dense opacities and detachment.

Review at 1 to 2 weeks, then every 4 to 6 weeks until quiescent for 3 months. Counsel on early return with pain or loss of vision.