Practical Diagnostic Approach to Uveitis

Anthony Grillo; Ralph D Levinson; Lynn K Gordon


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

In This Article

Intermediate Uveitis

Intermediate uveitis refers to inflammation that primarily manifests in the vitreous, presumably due to inflammation of the pars plana and peripheral retinal vasculature.[29,77,78] Patients with IU may also demonstrate peripheral vascular sheathing and macular edema. The term pars planitis is reserved for patients who have inflammatory membranes or precipitates, termed snowbank or snowball formation, without an associated infectious disease or systemic autoimmune condition. However, the presence of snowballs/banks alone do not establish the diagnosis of pars planitis. IU is often bilateral and patients typically complain of mild symptoms of blurred vision and floaters. The anterior chamber may be quiet in intermediate uveitis, but alternatively may have minimal cells/flare and small KPs. The classic findings in IU are vitritis, snowbanking (inflammatory exudates found on the pars plana) and peripheral retinal vascular sheathing. The most common diagnoses in IU are idiopathic (in ~two-thirds of cases), sarcoidosis and multiple sclerosis. Other much less common etiologies include IBD and infectious diseases such as primary toxoplasmosis prior to the appearance of the retinitis, toxocara if the disease is unilateral (particularly if a peripheral granuloma is observed), Lyme disease in endemic areas, Epstein–Barr virus, human T-lymphotropic virus type 1 (HTLV-1), cat-scratch disease (Bartonella species), syphilis, tuberculosis and hepatitis C, leptospirosis, brucellosis, Whipple's disease or noninfectious masquerade syndromes such as amyloidosis or foreign body (including tarantula or caterpillar 'hairs'). Intraocular lymphoma is a concern as a masquerade syndrome in patients with IU and is typically diagnosed using neuroimaging of the brain, evaluation of the cerebrospinal fluid and vitreous biopsy. Usually there will be subretinal lesions in lymphoma, but these may be very subtle.

All patients with IU should also undergo an evaluation to, when possible, diagnose a specific etiology. The history should be reviewed to determine whether the patient has any neurologic symptoms suggestive for MS or for CNS lymphoma. The patient should be queried about constitutional symptoms, exposure to cats, history of tick bites, endemic exposure to Lyme disease and the presence of rashes or arthralgias. The answers to these questions will help to define the required laboratory and radiologic evaluations. At a minimum a CXR, IFN-γ-release assay for evaluation of tuberculosis, and syphilis serologies are required. As previously described, additional studies for the evaluation of sarcoidosis, including CXR, CT scan and ACE and lysozome determinations may be indicated. Additional serologic studies should be directed by review of the clinical findings and the history.

Evaluation for MS would include a full neurologic consultation and brain neuroimaging using MRI with a contrast agent. Although less than half of patients with MS-associated IU will be unaware of the MS diagnosis at time of ocular presentation, up to 16% of individuals diagnosed with pars planitis had or subsequently developed MS, underscoring the importance of neurologic evaluation in suspected cases.[79–81] Evaluation for possible MS is very important if uveitis therapy with tumor necrosis inhibitors is being considered, as these agents may be associated with increased demyelination and are generally contraindicated in MS. Uveitis is present in approximately 14.5% of patients infected with HTLV-1, a disease endemic to Japan, the Caribbean and parts of Africa and South America, and the majority of these cases have unilateral IU. Diagnosis is made using serologic antibody testing, followed by Western blotting to confirm the diagnosis.


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