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

Posterior Uveitis

The term posterior uveitis refers to inflammation that primarily involves the retina and/or the choroid. The description includes focal retinitis, focal choroiditis, multifocal retinitis and multifocal choroiditis and is dictated by the clinical pattern of disease. Optic disc and peripapillary involvement may be observed in the setting of posterior uveitis. It is critically important to recognize that masquerade syndromes can present with any of these types of posterior uveitis. Other causes for a focal retinitis include infectious etiologies such as toxoplasmosis, acute retinal necrosis, cysticercosis or onchocerciasis.[82–85] A history of immunosuppression should suggest unusual etiologies such as CMV, retinitis, or progressive outer retinal necrosis, although these are now rare in AIDS patients who are undergoing highly active antiretroviral therapy.[7,39,86] Multifocal retinitis may be seen with sarcoidosis, syphilis, viral infections (CMV and Herpes family infections), fungal infections (e.g., candida) and bacterial infections.

Focal choroiditis may be secondary to toxocariasis, tuberculosis or fungal infection.[30,87] Multifocal choroiditis is caused by many etiologies including sarcoidosis, VKH disease, sympathetic ophthalmia, histoplasmosis, birdshot chorioretinopathy, serpiginous choroidopathy, multiple white-dot syndromes and nematode-associated disease.[44,88–90] A discussion of the white-dot syndromes is beyond the scope of this paper; however, the diagnoses are made based on clinical history, ocular examination, clinical course, characteristic findings on ocular imaging and in some cases electrophysiologic testing.

Birdshot chorioretinopathy is a bilateral, posterior uveitis that is characterized by subretinal white birdshot lesions.[91–93] Birdshot lesions have protean manifestations. They are generally hypopigmented choroidal lesions that vary in size and may become confluent and pigmented over time. They tend to be most common inferonasally or in clusters around the optic nerve head. There may be minimal anterior and/or vitreous inflammations, but this is primarily an isolated posterior uveitis. This disease is very strongly associated with HLA-A29 (>90%), one of the strongest HLA associations for any disease. However, as 7% of the population is HLA-A29 positive, the test is not diagnostic, but is strongly supportive and should be reserved for cases of suspected disease.[52] While HLA-A29 testing does not have high predictive positivity, a negative HLA-A29 test strongly suggests that the patient does not have birdshot chorioretinopathy and that the test should first be repeated and if still negative, another diagnosis such as sarcoidosis or even lymphoma should be considered, although rare HLA-A29 negative cases have been described.

Intraocular lymphoma can arise primarily within the CNS, where it is either primary intraocular lymphoma or primary CNS lymphoma or from outside the CNS with ocular metastasis.[9,76,83,94–97] Regardless of the origin, intraocular lymphoma tends to present bilaterally (80%) as either an intermediate and/or posterior uveitis. Patient complaints are usually decreased vision or floaters. The classic posterior findings in intraocular lymphoma have a very characteristic 'creamy'-appearing deep retinal lesion, although numerous atypical presentations have been reported. Fluorescein angiography is a useful adjunct in diagnosis of primary intraocular lymphoma, as it may reveal a distinct pattern of retinal pigment epithelial perturbations, but is not diagnostic. The diagnosis is established using MRI of the brain and cerebrospinal fluid (CSF) analysis. If CSF cytology is negative, the next step is a diagnostic vitrectomy. After diagnosis, neurology and oncology consultations are in order. It is also important to remember that primary CNS lymphoma may be associated with HIV and this should be a consideration in any of the patients.

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