Pain, photophobia, and decreased vision; symptoms may be minimal.

Critical

Elevated IOP with uveitis; open angle or PAS on gonioscopy; white blood cells, macrophages, and proteins cause outflow blockage and trabeculitis resulting in elevated IOP. Characteristic glaucomatous optic nerve changes occur late in the disease course. Due to the NFL edema in the acute phase, early on the NFL OCT may look normal despite having glaucomatous optic neuropathy and visual field defect.

Other

Miotic pupil, KP, conjunctival injection, ciliary flush, posterior synechiae, and increased TM pigmentation, especially inferiorly. Angle-closure glaucoma may occur from progressive PAS formation which generally start from inferior angle. 

NOTE Acute IOP increase from any etiology is distinguished from chronic IOP increase by the presence of corneal edema, pain, and visual symptoms.

• Steroid-response glaucoma: Open angle. Patient on steroids (including for uveitis). Can be difficult to differentiate from uveitis-induced IOP elevation. If significant inflammation is present, IOP elevation should be assumed inflammatory in nature and goal should be to quiet the eye with steroids. See 9.8, Steroid-Response Glaucoma.

• Pigmentary glaucoma: Open angle. Acute increase in IOP, often after exercise or pupillary dilation; pigmented cells in the anterior chamber; 3+ to 4+ TM pigmentation, pigment on the endothelium (Krukenberg spindle) and on the posterior zonules attachment line to lens (Scheie or Zentmayer line), radial iris TIDs. See 9.9, Pigment Dispersion Syndrome/Pigmentary Glaucoma.

• Neovascular glaucoma. Nonradial, misdirected blood vessels along the pupillary margin, the TM, or high PAS in regressed NV. See 9.13, Neovascular Glaucoma.

• Pseudoexfoliation: Open or closed angle. Grayish-white flaky material deposited throughout anterior segment structures. Classically, European descent, but not always. Occasionally exfoliative material on cornea can be mistaken for KP. Deposits are more angular, less round than inflammatory KP. See 9.10, Pseudoexfoliation Syndrome/Exfoliative Glaucoma.

• Fuchs heterochromic iridocyclitis: Unilateral, more common in middle-aged women. Low-grade inflammation with loss of iris pigmented epithelium causing heterochromia (affected eye typically lighter). Fine bridging vessels in the angle are present and may bleed (Amsler sign). Not neovascular. No PAS. See 12.1, Anterior Uveitis (Iritis/Iridocyclitis).

• Uveitis: Anterior, intermediate, posterior, or panuveitis.

• Keratouveitis: Herpetic infections are classically associated with unilateral elevated IOP in the setting of early/acute inflammation, whereas other etiologies may cause low IOP from ciliary body shutdown and hyposecretion.

• Glaucomatocyclitic crisis/Posner–Schlossman syndrome: A rare subtype of inflammatory open-angle glaucoma possibly associated with a viral etiology (e.g., cytomegalovirus), although the mechanism is unknown. Presents as recurrent episodes, typically unilateral, in young to middle-aged patients. IOP is markedly increased (usually 40 to 60 mm Hg) with minimal conjunctival injection, very mild anterior chamber reaction, and an open angle without PAS.

• After trauma or intraocular surgery.

1. History: Previous attacks? Systemic disease (e.g., juvenile idiopathic arthritis, ankylosing spondylitis, sarcoidosis, acquired immunodeficiency syndrome [AIDS], V1 distribution varicella zoster, toxoplasmosis)? Previous corneal disease, especially herpetic keratitis?

2. Slit-lamp examination: Assess the degree of conjunctival injection and aqueous cell and flare. Posterior synechiae present? Dilated fundus examination for signs of posterior uveitis. Gonioscopy.

3. Complete baseline glaucoma evaluation. See 9.1, Primary Open-Angle Glaucoma.

1. Topical steroid (e.g., prednisolone acetate 1%) q1–6h, depending on the severity of the anterior chamber inflammation.

   NOTE Topical steroids are not used, or are used with extreme caution, in patients with an infectious process.

2. Mydriatic/cycloplegic (e.g., cyclopentolate 1% t.i.d.).

3. One or more of the following pressure-reducing agents can be used in addition to the other treatments, depending on the IOP and status of the optic nerve:

   • Topical β-blocker (e.g., timolol 0.5% daily or b.i.d.) if not contraindicated (e.g., asthma, COPD, bradycardia).

   • Topical α₂ agonist (e.g., brimonidine 0.1% to 0.2% b.i.d. to t.i.d.). 

   • Topical CAI (e.g., dorzolamide 2% t.i.d.) or oral CAI (e.g., methazolamide 25 to 50 mg p.o. b.i.d. to t.i.d. or acetazolamide 500 mg sequel p.o. b.i.d.) if renal function tolerates and no allergy to sulfa medications.

   • Topical rho kinase inhibitor (e.g., netarsudil 0.02% q.d.).

   • Intravenous medications when IOP is acutely increased (e.g., mannitol 20% 1 to 2 g/kg i.v. over 45 minutes, if cardiopulmonary function permits; acetazolamide 500 mg).

   • Anterior chamber paracentesis if reduction in IOP is urgent, if IOP is refractory to topical therapy, i.v. medications are not feasible or contraindicated (see Appendix 13, Anterior Chamber Paracentesis).

4. Manage the underlying problem.

5. If IOP remains dangerously elevated despite maximal medical therapy, glaucoma filtering surgery may be indicated. Trabeculectomy surgery has high rates of failure in cases of inflammatory glaucoma. Tube shunt is often the preferred alternative.

6. If HSV suspected, start antiviral coverage (e.g., acyclovir 400 mg p.o. 5 times daily or valacyclovir 500 mg p.o. t.i.d. for 7 to 14 days).

   NOTE Prostaglandin agonists (e.g., latanoprost) and miotics (e.g., pilocarpine) should be used with caution in active inflammatory glaucoma, but may be considered once the eye is quiet or if the benefits outweigh the risks.

1. Patients are seen every 1 to 7 days at first. Higher IOP and more advanced glaucomatous cupping warrant more frequent follow-up.

2. IOP lowering medications are tapered as IOP returns to normal.

3. Steroid-response glaucoma should always be considered if IOP remains high when inflammation has subsided (see 9.8, Steroid-Response Glaucoma). However, IOP elevation in the presence of significant uveitis suggests the need for additional anti-inflammatory medications and additional or alternative IOP lowering therapy.