Classification and Diagnosis of Anterior Uveitis

Diagnosis

The Standardization of Uveitis Nomenclature (SUN) Working Group developed criteria for reporting clinical data in uveitis. The disease is classified based on onset, duration, and course. Onset of inflammation is considered either sudden or insidious. Duration is divided into limited (≤3 months) and persistent (>3 months). Disease course can be described as acute, recurrent, or chronic. Acute uveitis refers to an episode of sudden onset and limited duration. Recurrent uveitis describes repeated episodes of uveitis with periods of quiescence off all treatment for more than three months. In chronic uveitis, a patient is not free of inflammation for longer than three months while off treatment.

Symptoms of Anterior Uveitis

Characteristic symptoms of acute anterior uveitis include pain, redness, photophobia, and occasional tearing. Their severity may vary with the underlying etiology, abruptness of onset, and tolerance of the patient. The pain of anterior ocular inflammation results from congestion and irritation of the anterior ciliary nerves and, if severe, can produce ciliary spasm and photophobia. Pain is often localized to the eye but can also be referred to the periorbital region, forehead, or temple. Nausea and vomiting may occur if there is accompanying angle closure glaucoma. Blurred vision may be seen in cases of severe inflammation with fibrin or with reactive cystoid macular edema (CME).

Pain is uncommon in chronic anterior uveitis. Instead, blurred vision and dull ache are the more common presenting symptoms; however, many cases will be asymptomatic. This is especially the case, for example, in juvenile idiopathic arthritis (JIA). Associated redness is typically mild. Decreased vision is more common in chronic anterior uveitis because of extensive posterior synechiae formation, pupillary seclusion, cataract, glaucoma, cyclitic membrane formation, or CME.

History

The chief complaint and history of present illness are important in the diagnosis of uveitis. Pertinent history includes symptoms at onset, duration, laterality, clinical course, prior treatment and response, and history of antecedent illnesses.

Historical information should include details of any similar episodes. Previous diagnoses that can complicate the management of the disorder, such as steroid-induced intraocular pressure elevation, glaucoma, and herpes keratitis, also should be documented. Finally, a detailed history of previous ocular surgery, including strabismus and cyclodestructive procedures, or a history of penetrating trauma or foreign body, is crucial when considering a diagnosis of sympathetic ophthalmia or exogenous endophthalmitis. In this situation, timing is important, with exogenous endophthalmitis more common after recent trauma or surgery.

Demographic Information

Certain types of uveitis are more common in particular age groups ( Table 102.1 ). These groups are not intended to be mutually exclusive, but instead should serve as general guidelines for establishing a differential diagnosis.

Consideration of gender, race, and ethnicity of the patient also may prove useful when considering several diagnoses. For example, ankylosing spondylitis is more common in males, whereas pauciarticular JIA occurs most frequently in females.

Occupation may provide clues to possible infectious etiologies. Slaughterhouse workers, butchers, veterinarians, and farmers may be exposed to tissues or milk products infected with Brucella. Medical workers are at risk for a variety of infectious agents, such as tuberculosis, herpes simplex, and human immunodeficiency virus (HIV).

Current and past residences and recent travel provide information on possible exposure to infectious agents. A history of tick bites or traveling in wooded areas, particularly in endemic regions such as Connecticut or Wisconsin as well as western coastal states, should raise the possibility of Lyme disease. Individuals residing in the southwestern United States, Mexico, or Central and South America may be exposed to coccidioidomycosis. Leprosy should be considered in immigrants from developing regions. Onchocerciasis, or river blindness, is endemic in Africa and Central America. Fuchs heterochromic iridocyclitis may be more common in patients from countries without a rubella vaccination program.

Past Medical History and Review of Systems

The past medical history should include specific questions regarding infectious diseases or exposure, including sexually transmitted diseases, as well as other past conditions, such as immunodeficient, autoimmune, and rheumatologic diseases. Certain medications, such as rifabutin, bisphosphonates, sulfonamide, cidofovir, BRAF and MEK inhibitors, and immune checkpoint inhibitors, can cause or exacerbate anterior uveitis. The historical belief that prostaglandin analogs are associated with anterior segment inflammation and cystoid macular edema has been disproved.

Immunization history should be queried as immigrants may not have been vaccinated for infectious disease, and there has been a recent increase in parents choosing not to immunize their children in the United States. There have been occasional reports of vaccination preceding uveitis.

The review of systems is directed toward specific signs and symptoms suggestive of an underlying etiology for the uveitis ( Table 102.2 ). These findings help provide the basis for the selection of appropriate diagnostic tests.

Social and Family History

Social history should include dietary history, pet exposure, and history of drug abuse. Information regarding the consumption of raw meat (toxoplasmosis), raw fish, or unpasteurized dairy products (brucellosis) may yield further diagnostic clues. Exposure to cats may be associated with toxoplasmosis and bartonellosis, whereas contact with puppies is a risk factor for toxocariasis. Patients with a history of intravenous drug abuse are at increased risk for metastatic endophthalmitis, particularly with fungal organisms. Family history is occasionally useful in the diagnosis of uveitis, especially in the human leukocyte antigen (HLA)-linked disorders. For example, there is a strong genetic influence in HLA-B27-associated anterior uveitis and spondyloarthropathies. Chronic anterior uveitis is more common in patients with a family history of inflammatory bowel disease than in the general population.