Uveitis, Anterior, Granulomatous

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Background

Iritis, also known as anterior uveitis, is the most common form of ocular inflammation and often causes a painful red eye.

Inflammation of the iris appropriately may be termed iritis. Inflammation of the iris and the ciliary body is called iridocyclitis. Iritis may be subdivided into 2 broad categories: granulomatous and nongranulomatous.

A granulomatous iritis has an increased likelihood of being part of a systemic disease process or a component of certain ocular syndromes. However, the diagnosis of granulomatous iritis does not definitively indicate that an underlying systemic granulomatous process is present.

Patients with a granulomatous iritis may present with an acutely painful eye or with chronic subclinical inflammation that is discovered only as part of a routine ocular examination.

Pathophysiology

The exact pathophysiology of granulomatous iritis is unknown. It may result from an autoimmune reaction or from the host's immune response to a systemic infectious process, such as syphilis, Lyme disease, tuberculosis (TB), or local reactivation of herpetic viral infection.

Not all cases classified as granulomatous are necessarily granulomatous upon histologic examination. Granuloma are found in certain infectious and autoimmune processes and even in inflammation secondary to foreign bodies; they represent an inflammatory response that implies chronic inflammation.

Epidemiology

Frequency

United States

Iritis, granulomatous and nongranulomatous, is the most frequent form of uveitis that ophthalmologists encounter. In one community-based study, anterior uveitis accounted for more than 90% of all cases of uveitis seen. The annual incidence is about 8 cases per 100,000 population.[1] However, these cases were predominantly nongranulomatous anterior uveitis.

International

No particular geographic distribution has been noted for granulomatous iritis. Although certain etiologies may be more common in certain parts of the world (eg, TB in endemic areas).

Mortality/Morbidity

Morbidity may arise from both the iritis and any associated systemic disease if present.

Race

Racial differences may exist, depending on the underlying cause of the iritis. For example, sarcoidosis is more likely to be diagnosed in the African American population than in other groups. Vogt-Koyanagi-Harada disease, although a rare cause of uveitis in the United States, is much more prevalent in persons of Mestizo, Asian, or American Indian ancestry.

Sex

No significant sex differences are reported.

Age

Granulomatous iritis may develop in individuals of any age.

History

Inquire about the patient's complete medical history, to include all medical conditions, surgeries, medications, and ocular history (eg, history of iritis). Perform a detailed review of systems. This is critical, as the history and the review of systems in many cases will suggest a diagnosis.

Critical review questions include, but are not limited to, asking about arthritis, rashes, shortness of breath, swollen lymph nodes, recent headaches, hearing difficulties, hair loss, pigment changes in the skin, a history of ocular trauma, recent insect bites, sexually transmitted diseases (STDs), TB exposure, blood in stools, and recent travel.[2, 3, 4]

Inquire about the following symptoms:

Physical

A complete ocular examination is indicated.

Causes

Not all patients with a granulomatous-appearing iritis have a systemic granulomatous disease process. A differential for a patient who has either bilateral granulomatous iritis or unilateral granulomatous iritis is outlined below. Many of these are more likely to be associated with intermediate or panuveitis and not isolated anterior uveitis. Alternatively, as in Vogt-Koyanagi-Harada disease, the chronic anterior uveitis may occur as the main clinical feature later in the course of the disease, with little active posterior segment inflammation.

Laboratory Studies

Imaging Studies

Procedures

Histologic Findings

Medical Care

Treatment of inflammation of the anterior segment is as follows.

Surgical Care

Glaucoma surgery can be performed if medical treatment fails to control the intraocular pressure and after quieting the eye from inflammation. In case of cataract, the eye must be free of inflammation at least 3 months before the surgery. A peripheral iridectomy may be indicated for iris bombe; however, if the pupil is not entirely occluded or secluded, an iridectomy can precipitate iris bombe by diverting flow from the small area of the pupil that is not occluded. In that case, if there is an exacerbation of inflammation, the pupil can close as there is no flow and the iridectomy may become occluded as well.

Consultations

If a specific systemic diagnosis is suspected or is confirmed on the basis of laboratory and/or radiographic investigation, consultation with a subspecialist may be indicated.

Medication Summary

Topical corticosteroids and a cycloplegic agent should be started. If the eye does not adequately respond to topical therapy within 1 week or so, or if very severe, oral corticosteroids or a periocular injection of corticosteroids may be added to the treatment regimen.

Oral corticosteroids may be particularly useful in cases of bilateral noninfectious granulomatous iritis. Routine use of depot steroids in infectious uveitis, in known steroid responders, or in patients with a glaucoma or already elevated IOP should be considered carefully because of potential for severe or sight-threatening adverse effects.

In cases of severe granulomatous iritis, the treating clinician may elect to begin therapy with topical and oral corticosteroids. The tapering of steroid therapy is guided by the clinical response on follow-up examination. Topical or systemic nonsteroidal anti-inflammatory drugs (NSAIDs) are of little or no benefit in the treatment of granulomatous iritis.

Immunomodulatory and immunosuppressive medications may be useful in patients who are unresponsive to corticosteroids, in patients with chronic uveitis, or in patients who develop adverse effects of corticosteroid therapy.

A number of agents have been used, including methotrexate, azathioprine, cyclosporin A, mycophenolate mofetil, cyclophosphamide, and chlorambucil. Myelosuppression and secondary infection are among the most common adverse effects of these agents.

Tumor necrosis factor alpha (TNF-alpha) inhibitors may be useful in some cases of granulomatous anterior uveitis. They are effective in reducing the number of flares of anterior uveitis in patients with sarcoidosis.[7]

An internist or a rheumatologist should be involved in the management of patients treated with immunomodulatory agents.

Prednisolone acetate 1% (Pred Forte, Econopred)

Clinical Context:  Decreases inflammation by suppressing migration of polymorphonuclear leukocytes and by reversing increased capillary permeability.

Prednisone (Meticorten, Deltasone, Orasone)

Clinical Context:  Can be used if topical therapy inadequate to treat iritis (especially if bilateral). Decreases inflammation by suppressing migration of polymorphonuclear leukocytes and by reversing increased capillary permeability.

Class Summary

These agents are the mainstays of therapy for iritis, and they help to stabilize blood-aqueous barrier.

Cyclopentolate hydrochloride 1% (AK-Pentolate, Cyclogyl)

Clinical Context:  Prevents muscle of ciliary body, and sphincter muscle of iris, from responding to cholinergic stimulation. Induces mydriasis in 30-60 min and cycloplegia in 25-75 min.

Class Summary

These agents are used to help prevent or break posterior synechiae and to reduce ciliary body–induced pain.

Further Outpatient Care

Complications

Prognosis

Author

Ralph D Levinson, MD, Associate Professor of Ophthalmology, Jules Stein Eye Institute at the David Geffen School of Medicine at UCLA

Disclosure: Nothing to disclose.

Specialty Editors

Andrew A Dahl, MD, FACS, Assistant Professor of Surgery (Ophthalmology), New York College of Medicine (NYCOM); Director of Residency Ophthalmology Training, The Institute for Family Health and Mid-Hudson Family Practice Residency Program; Staff Ophthalmologist, Telluride Medical Center

Disclosure: Nothing to disclose.

Simon K Law, MD, PharmD, Clinical Professor of Health Sciences, Department of Ophthalmology, Jules Stein Eye Institute, University of California, Los Angeles, David Geffen School of Medicine

Disclosure: Nothing to disclose.

R Christopher Walton, MD, Professor, Director of Uveitis and Ocular Inflammatory Disease Service, Department of Ophthalmology, University of Tennessee College of Medicine

Disclosure: Nothing to disclose.

Lance L Brown, OD, MD, Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri

Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy Sr, MD, Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences

Disclosure: Nothing to disclose.

Additional Contributors

Abdullah Al-Fawaz, MD, FRCS Assistant Professor, Cornea and Uveitis Department, King Abdulaziz University Hospital, Department of Ophthalmology, King Saud University, Riyadh, Saudi Arabia

Abdullah Al-Fawaz, MD, FRCS is a member of the following medical societies: American Academy of Ophthalmology and Royal College of Physicians and Surgeons of Glasgow

Disclosure: Nothing to disclose.

Roger K George, MD, Director of Uveitis Service, Madigan Army Medical Center; Clinical Instructor, Department of Ophthalmology, Oregon Health and Sciences University

Disclosure: Nothing to disclose.

References

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Granulomatous anterior uveitis with mutton-fat keratic precipitates and Koeppe and Busacca nodules.

Granulomatous anterior uveitis with numerous Busacca nodules on the iris surface and a few mutton-fat keratic precipitates on the inferior aspect.

Granulomatous anterior uveitis with mutton-fat keratic precipitates and Koeppe and Busacca nodules.

Granulomatous anterior uveitis with numerous Busacca nodules on the iris surface and a few mutton-fat keratic precipitates on the inferior aspect.