Practical Diagnostic Approach to Uveitis

Anthony Grillo; Ralph D Levinson; Lynn K Gordon

Disclosures

Expert Rev Ophthalmol. 2011;6(4):449-459. 

In This Article

Panuveitis

Sympathetic ophthalmia may present as a multifocal choroiditis, but more commonly is a bilateral granulomatous panuveitis that presents several weeks to months after either surgery or penetrating trauma.[98–100] It is believed that the inflammation is actually a T-cell response to choroidal melanocytes, which explains its bilateral nature. There is usually accompanying vitritis and white choroidal lesions. This condition is rare but should be considered when there is a history of intraocular surgery, trauma and other causes of uveitis have been ruled out.[100,101]

Vogt–Koyanagi–Harada disease is a systemic inflammatory condition characterized by a panuveitis, often granulomatous, of sudden onset, which may be associated with neurologic, dermatologic and auditory involvement.[16,102,103] The disease is more common in individuals from Asian, Hispanic or American–Indian heritage. The ophthalmologic findings include a bilateral, granulomatous panuveitis. VKH disease is broken into four phases, which may overlap:

  • • Prodromal, in which the patients may often experience flu and/or meningitis-like symptoms. Headache is a common complaint even without meningismus;

  • Acute uveitic, during which patients present with anterior cell/flare, exudative retinal detachments, diffuse choroiditis and optic disc edema;

  • Chronic, with regression of active inflammation and appearance of the hallmark 'sunset-glow' fundus appearance. Although some patients continue to have chronic bilateral AU, posterior segment recurrences are rare except when treatment is weaned too rapidly. Patients may often demonstrate poliosis or vitiligo during this stage of the disease;

  • Recurrent, in which anterior inflammation is more prominent than posterior.

Associated symptoms or signs are often helpful in diagnosing VKH disease. Neurologic symptoms may be multiple and severe, including but not limited to, meningeal symptoms and signs, headache, confusion, cranial nerve palsies and inner ear disorders, although this is rare. The presence of neurologic symptoms may require brain neuroimaging and CSF analysis to rule out other etiologies. Often in VKH there will be cellular pleiocytosis of the CSF. Fluorescein angiography will confirm choroiditis at the time of disease onset, with multiple areas of leakage at the level of the retinal pigment epithelium. Ultrasonography typically demonstrates a thickened choroid. An important differential diagnosis to VKH is sympathetic ophthalmia.

Other causes of panuveitis include Behçet's disease, sarcoidosis and infectious endophthalmitis.[1,14,17,41,48,89,104,105] Infectious endophthalmitis may initially present with vitritis, but typically a severe inflammation involving all ocular structures is observed. Postoperative endopthalmitis is usually suspected when the inflammation occurs after surgery and can be acute or chronic. In the latter case, it may partially respond to corticosteroids. Late-onset postoperative endophthalmitis, even years after surgery, must be suspected in patients after glaucoma surgery with new-onset inflammation, especially after use of mitomycin. Endogenous endopthalmitis often is suspect when there are risk factors such as recent bowel perforation or surgery, indwelling lines, intravenous drug use or known recent infections elsewhere. If endogenous endophthalmitis is suspected, vitreous sampling with culture and potentially with molecular diagnostics is indicated.[11] If there is no prior history of trauma or eye surgery then echocardiography, CXR and blood cultures should also be performed. HIV testing should also be considered in otherwise healthy individuals presenting with suspected endogenous endophthalmitis. Other important considerations in the differential diagnosis are masquerade syndromes and primary intraocular B-cell lymphoma.[10,76,94]

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