Overview
- Definition
Bilateral granulomatous panuveitis that develops after ocular surgery or penetrating trauma to one eye, causing disruption to the immune privilege of the eye
- Symptoms
Redness
Photophobia
Pain
Blurry vision
Floaters
- Laterality
Bilateral
- Course
- Average time between injury/surgery to onset of SO: 2 weeks to 3 months (range: 5 days to 66 years).
90% of cases manifest within 12 months of insult.
Onset can be insidious in the sympathizing, noninjured eye.
Severity of inflammation and its sequelae are wide-ranging, and relapsing nature of SO requires long-term monitoring.
75% of patients retain ≥20/200.
- Age of onset
All ages affected
- Gender/race
Males more affected, likely due to higher risk of ocular injury
No racial predisposition
- Systemic association
Patients with SO are more likely to express HLA-DR4, HLA-DQw3, HLA-DRw53 (also seen in VKH).
VKH-like integumentary changes (poliosis and vitiligo) have been reported, but very uncommon.
Exam: Ocular
Anterior Segment
Mild-to-severe anterior uveitis with mutton-fat precipitates.
Corneal endothelium may decompensate with chronic inflammation → bullous keratopathy.
Posterior synechiae.
Secondary cataract is common.
Posterior Segment
Mild-to-moderate vitritis
Dalen-Fuchs nodules: multiple yellowish-white choroidal lesions in the periphery (also seen in VKH and sarcoidosis)
Diffuse choroiditis
Papillitis
Exudative RD
Subretinal fibrosis
Retinochoroidal and optic atrophy
Exam: Systemic (Uncommon)
Vitiligo
Hearing dysfunction
Imaging
- OCT
Varied disruptions of the outer retinal segments
Subretinal fluid corresponding to exudative RD
Intraretinal edema and thickening
Diffuse choroidal thickening best seen on EDI-OCT: may be used to monitor disease and treatment response
- FA
Multiple hyperfluorescent leakage at the level of RPE during the venous phase that persist into the late phase
Dye pooling in subretinal spaces in severe cases
Areas of early blocked fluorescence corresponding to Dalen-Fuchs nodules
- ICG
Multiple hypofluorescent foci that become more prominent as angiography progresses
- B-scan
Marked choroidal thickening
Laboratory and Radiographic Testing
HLA typing may help confirm diagnosis.
- Labs are done to rule out DDx:
ACE/lysozyme
PPD or QuantiFERON-Gold
FTA-ABS/RPR
Differential Diagnosis
VKH (Table 16.1)
Sarcoidosis
Syphilis
Tuberculosis
Intraocular lymphoma
Comparison of sympathetic ophthalmia (SO) and Vogt-Konayagi-Harada syndrome (VKH)
Characteristics | SO | VKH |
|---|---|---|
Age | All ages | 20–50 years |
Racial predisposition | None | Asia and black |
Penetrating injury | Always present | Absent |
Skin changes | Uncommon | Common (60–90%) |
CNS findings | Uncommon | Common (85%) |
Hearing dysfunction | Uncommon | Common (75%) |
Optic nerve inflammation | Occasional | Frequent |
Exudative RD | Rare | Frequent |
Choriocapillaris involvement | Usually absent | Frequent |
CSF findings | Usually normal | Pleocytosis (84%) |
Treatment
Corticosteroids – both systemic and local – should be instituted as soon as possible.
- Steroid-sparing IMT should be started at time of diagnosis, as inflammation is certain to relapse upon steroid discontinuation.
Cyclosporine, azathioprine, mycophenolate mofetil, chlorambucil, cyclophosphamide, and infliximab have all shown efficacy.
Fluocinolone acetonide (Retisert), if IMT not effective or not tolerated.
Enucleation may lower chance of SO if done within 2 weeks of open globe injury; ineffective after development of autoimmune inflammation.
Referral/Comanagement
Rheumatology