Sympathetic Ophthalmia: Where Do We Currently Stand on Treatment Strategies?

Figures & data

Table 1 Triggering Events in SO Patients

Mechanism	Triggering Events
Trauma	Penetrating eye injuries^Citation83
	Closed globe injuries/Blunt trauma^Citation25,Citation26
	Chemical ocular injuries^Citation29
	Intraocular foreign body^Citation28
	Thermal burn^Citation30
Procedures	Pars plana vitrectomy^Citation17
	Transscleral cyclophotocoagulation^Citation31
	Cataract surgery^Citation20
	Intravitreal injection^Citation21
	Scleral buckling^Citation32
	Helium ion therapy for choroidal melanoma^Citation34
	Ruthenium plaque for iris melanoma^Citation35
	Proton beam radiation for choroidal melanoma^Citation33
	Enucleation^Citation36
	Penetrating keratoplasty^Citation37
	Repair of cyclodialysis cleft^Citation43
	Phakic intraocular lens implantation^Citation39
	Evisceration^Citation40
	Iridectomy^Citation41
	Paracentesis^Citation44
	Iris inclusions in wound
	Intravitreal injection^Citation21
	Trabeculectomy^Citation42
	Iridencleisis^Citation22
	Conjunctival flap procedure for perforated corneal ulcer^Citation38
Other diseases	Acanthamoeba keratitis^Citation23
	Fungal keratitis^Citation24

Figure 1 After the inciting event, the antigen-presenting cells (APC) present the antigen peptide to autoreactive CD4+ cells. This activates the CD4+ cells and brings about the autoimmune reaction damaging the other (sympathizing) eye.

Abbreviations: TCR, T-cell receptor; HLA, human leucocyte antigen; Ag, antigen.

Figure 2 (A) Fundus photo of the posterior pole of the sympathizing eye shows a blurred margin of the optic disc, radial retinal folds around the optic disc, and subretinal fluid at the posterior pole. (B) FFA image at early venous phase shows disc leak, choroidal folds, and pinpoint leaks. (C) FFA image at mid-venous phase shows the typical multiple pinpoint leaks more clearly. (D) FFA image at late venous phase shows disc leak and blurred margins of pinpoint leaks.

Figure 3 (A) Anterior segment photo shows a phthisical eye with evidence of repair of globe rupture. (B) Posterior segment photo of the other eye shows depigmented fundus (sunset glow) with areas of fibrotic pigmentary changes.

Table 2 Clinical Features of Patients with Sympathetic Ophthalmia (SO)

Symptoms	Signs
Redness Sensitivity to light Vision loss (near vision loss may be early) Pain Watering of eye Floaters Flashes (photopsia) Metamorphopsia Hearing complaints (tinnitus, hearing loss, dysacusis) Headache Meningismus	Keratic precipitates (granulomatous or nongranulomatous) Anterior chamber cells/Flare Synechiae Vitritis Optic disc hyperemia Serous retinal detachment (may be multifocal) Choroiditis Dalen–Fuchs nodule Macular edema Vasculitis (rare) Cataract Choroidal neovascularization Sub-retinal hemorrhage Subretinal fibrosis Pigmentary atrophy

Table 3 The Spectrum of Various Immunomodulatory Agents Used in the Treatment of Sympathetic Ophthalmia (SO)

Class	Drug	Route of Administration	Dosage
Inhibitors of T-cell signaling	Cyclosporine	Oral	2.5–5 mg/kg/day
Antimetabolites	Azathioprine	Oral	1–3mg/kg/day
	Methotrexate	Oral/Subcutaneous/ Intramuscular/Intravenous	7.5–25mg/week
	Mycophenolate mofetil	Oral	1–3 mg once a day
Alkylating agents	Cyclophosphamide	Oral/Intravenous	1–3 mg/kg/day orally or1 g/m^2(BSA) infusions
	Chlorambucil	Oral	2–12 mg/kg/day
Biologics	Infliximab	Intravenous	5–20 mg/kg/day^Citation129
	Adalimumab	Subcutaneous	40 mg once every 2 weeks^Citation161

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