Uveitis, a term used to describe inflammation inside the eye, is often one of the more challenging diagnostic and treatment areas encountered by ophthalmology residents and fellows. In this course, learn the modern framework for uveitis diagnosis and treatment, including clinical pearls for differential diagnosis.
Uveitis, a term used to describe inflammation inside the eye, is often one of the more challenging diagnostic and treatment areas encountered by ophthalmology residents and fellows. Although uveitis can result from autoimmune diseases and infectious processes, nearly half of cases are idiopathic with no known attributable cause. Management of both acute and chronic inflammation require both systemic and ocular workup and timely treatment is essential to minimize vision loss. The myriad of complex presentations represent challenging cases for ophthalmologists at all levels and successful outcomes require an approach that addresses all possible relevant causes. Here, I present a modern approach to the diagnosis and management of uveitis.
Terminology has links to etiology and clinical evolution and thus is relevant to any approach for the diagnosis and management of uveitis. Specifically, ophthalmology residents need to be aware of the following nomenclature.
When considering a uveitic diagnosis, the top priority is to rule out infectious process!
Figure 1: A patient presenting with progressive outer retinal necrosis (PORN) viral retinitis with a known history of acquired immunodeficiency syndrome (AIDS) secondary to human immunodeficiency virus (HIV).
Uveitis secondary to infectious processes can present as follows:
Figure 2: Slit-lamp visualization of the Ixodes tick larvae attached to the nasal conjunctiva of the patient's right eye, 1.5 mm posterior to the limbus. There was 2 + conjunctival injection and prominent episceral vessels. Adapted with permission from Kuriakose RK, Grant LW, Chin EK, Almeida DRP. Deer tick masquerading as pigmented conjunctival lesion. Am J Ophthalmol Case Rep. 2016;5:97-98. doi:10.1016/j.ajoc.2016.12.018
Presence of red flags indicate possible atypical presentation which can progress quickly with severe vision loss and systemic morbidity. These include:
Remember that common diagnoses are most common!
In cases of anterior uveitis (e.g., iritis), most common causes are idiopathic, HLA-B27 associated disease, juvenile idiopathic arthritis in pediatric patients, and anterior chamber reaction secondary to herpes simplex virus and varicella zoster virus.
With respect to posterior segment inflammation, the most common worldwide cause is Toxoplasmosis infection.
Primary location of inflammation allows one to organize differential diagnosis as follows:
In adults, the most common cause of anterior uveitis is idiopathic iridocyclitis, representing 10% of all uveitis cases.
Figure 3: Presentation of idiopathic iridocyclitis with conjunctival injection, anterior chamber reaction, iris transillumination defects and posterior synechiae.
Pars planitis, also known as idiopathic intermediate uveitis, is the most common cause of intermediate uveitis and represents 80 to 90% of cases involving the posterior ciliary body and pars plana as the primary anatomical inflammatory sites. Clinical signs include vitreous inflammation (vitritis, snowballs, snowbanks) in the absence of retinal findings. Patients may have a concomitant mild iritis in cases of pars planitis.
Figure 4: Presentation of pars planitis with vitreous inflammation consisting of snowballs and snowbanking over the pars plana.
Pars planitis typically presents during the ages of 5-15 years old and is bilateral in 80% of cases. Careful dilated depressed examination will reveal vitreous inflammation consisting of snowballs (vitritis) and snowbanks (vitreous inflammation over pars plana inferiorly). Associated signs include cataract with posterior synechiae, cystoid macular edema, epiretinal and epimacular membrane formation; severe cases can demonstrate peripheral neovascularization with vitreous hemorrhage and tractional components. In patients with pars planitis, vision loss occurs primarily from cystoid macular edema (50% of cases), cataract, neovascularization (10% of cases) and vitreous hemorrhage (5% of cases).
Differential diagnosis of intermediate uveitis is SIMPLE:
Figure 5: Presentation of intermediate uveitis with peripheral periphlebitis in a patient with multiple sclerosis.
Toxoplasmosis, a form of infectious posterior uveitis, is the most common cause of posterior uveitis and represents 7% of all cases of uveitis. Toxoplasmosis gondii is a protozoan that typically presents as a focal area of white retinitis proximal to a pigmented scar with associated prominent vitritis. Management involves oral treatment with 800 mg sulfamethoxazole and 160 mg trimethoprim. Although oral and intraocular corticosteroids can be employed for inflammatory control, concomitant anti-protozoal coverage is necessary.
Figure 6: Classical presentation of posterior uveitis secondary to Toxoplasmosis with a focal area of retinitis proximal to a chronic pigmented scar.
Figure 7: Presentation of posterior uveitis secondary to syphilis with optic disc swelling (left) associated with extensive leakage on angiography (right).
Figure 8: Presentation of posterior uveitis secondary to tuberculosis infection with posterior pole plaques. Angiography displays characteristic early hypofluorescence followed by late hyperfluorescence.
Figure 9: Presentation of posterior uveitis (disc edema, focal retinitis) secondary to infection with the spirochete Borrelia burgdorferi in Lyme disease.
Uveitis remains a daunting and challenging disease area for ophthalmology residents and fellows. Although most cases of uveitis are idiopathic, the need to manage complex autoimmune diseases and infectious processes requires a careful, detailed and systematic approach to diagnosis. In this course, we have reviewed a modern approach to the diagnosis of ocular inflammation with direct emphasis on critical diseases and clinical pearls.