AUTHOR AND EDITOR INFORMATION

Section 1 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

INTRODUCTION

Section 2 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

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.

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.

International

No particular geographic distribution has been noted for granulomatous iritis.

Mortality/Morbidity

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

• Patients may have anterior and posterior synechiae. Extensive posterior synechiae can lead to a secluded pupil that can result in angle-closure glaucoma. In addition, trabecular obstruction can lead to secondary glaucoma.
• The eye with a granulomatous iritis is likely to have uveitis involving other structures of the eye, including the posterior segment. This may result in an increased risk of substantial visual impairment.
• Associated ocular complications (eg, cataracts, corneal decompensation, glaucoma, chronic cystoid macular edema) may result in severe vision loss.

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 Asian or American Indian ancestry.

Sex

No significant sex differences are reported.

Age

Granulomatous iritis may develop in individuals of any age.

CLINICAL

Section 3 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

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.

Inquire about the following symptoms:

• Pain: Some patients have no ocular pain, or they may describe a foreign body sensation. Other patients describe an abrupt onset of dull, aching eye pain. This pain may be ocular, or it may be referred to the periorbital region or temple.
• Photosensitivity: Light, especially sunlight, worsens the discomfort.
• Redness: Patients may describe having an injected eye. A discharge is usually not present.
• Vision: If the posterior segment of the eye is involved, the patient may have reduced visual acuity and may complain of floaters.
• Granulomatous iritis is more likely to be a bilateral process (except in herpetic iridocyclitis), whereas nongranulomatous iritis is typically unilateral.

Physical

A complete ocular examination is indicated.

• Vision: Visual acuity may range from normal to significantly reduced, depending on the extent of the ocular inflammation.
• Intraocular pressure (IOP): IOP is usually reduced in the eye with iritis due to decreased aqueous production by the inflamed ciliary body. Occasionally, IOP is elevated as a result of altered or obstructed aqueous outflow.
• External findings: Examine the patient for enlarged lacrimal glands, as this may suggest sarcoidosis.
• Conjunctiva: Generalized redness of the bulbar conjunctiva may be present. The eye may have a perilimbal injection termed ciliary flush. Carefully examine the patient for small nodules that, if sampled during biopsy, may help in determining the underlying cause of the iritis.
• Cornea: Keratic precipitates (KPs) are found on the endothelium. KPs are clusters of WBCs. Mutton-fat KPs are large and have a greasy appearance. They are usually located over the lower half of the cornea. Corneal edema may be present.
• Anterior chamber: Flares and cells are usually present.
• • A flare, resulting from extra protein in the aqueous, is usually present and can be graded as follows:
  • • 0 = Completely absent
    • 1+ = Barely present
    • 2+ = Moderate
    • 3+ = Marked (iris and lens detail hazy)
    • 4+ = Intense (formed fibrin in aqueous)
  • Cells, the hallmark of iritis, are present in the aqueous. They should be graded by severity under high-magnification slit lamp examination in a 1 X 3-mm field of light, as follows:
  • • Trace = 1-5 cells
    • 1+ = 6-15 cells
    • 2+ = 16-25 cells
    • 3+ = 26-50 cells
    • 4+ = More than 50 cells
• Iris: Peripheral anterior synechiae and posterior synechiae may be present. Inflammatory nodules (Koeppe and Busacca) are usually tan in color and represent accumulations of inflammatory cells. Koeppe nodules are found at the pupillary border. Busacca nodules are located on the surface of the iris. If Busacca nodules are present, then the underlying etiology is almost always a granulomatous process.
• Lens and vitreous: Lenticular precipitates may be present on the anterior lens capsule. Posterior subcapsular cataracts may be present if the patient has had repeated episodes of iritis or ongoing chronic inflammation. Carefully examine the vitreous for inflammatory cells; if present, they imply a more extensive uveitic syndrome.
• Posterior segment: Carefully examine the posterior segment for evidence of optic nerve edema, for vasculitis, and for focal retinal and/or choroidal lesions. A patient with a granulomatous-appearing iritis is likely to have a more extensive uveitis.

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.

• Sarcoidosis
• Syphilis
• Vogt-Koyanagi-Harada disease
• Sympathetic ophthalmia
• Multiple sclerosis
• Lyme disease
• Tuberculosis
• Herpes zoster
• Coccidioidomycosis
• Leprosy
• Toxoplasmosis
• Brucellosis
• Idiopathic

DIFFERENTIALS

Section 4 of 11  

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Other Problems to be Considered

Masquerade syndromes
Intraocular lymphoma
Chronic retinal detachment

WORKUP

Section 5 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Lab Studies

• Findings from the physical examination, a comprehensive review of the patient's medical history, and the review of systems should guide the laboratory evaluation. The workup should be tailored accordingly.
• All patients who present with a granulomatous iritis should receive a diagnostic evaluation, even if it is their first episode of uveitis.
• Laboratory tests that may be requested are outlined below. At the least, chest radiography and fluorescent treponemal antibody absorption (FTA-ABS) should be ordered.
  • Chest radiograph for sarcoidosis and TB
  • FTA-ABS test for syphilis
  • Purified protein derivative (PPD) test for TB
  • CBC with differential
  • Angiotensin-converting enzyme (ACE) test for sarcoidosis
  • Gallium scan for sarcoidosis.
  • Anergy evaluation for sarcoidosis
  • Lyme serology
  • Toxoplasmosis enzyme-linked immunosorbent assay (ELISA)

Imaging Studies

• MRI of the head may help in suspected cases of intraocular (CNS) lymphoma.
• In patients in whom sarcoidosis is suspected and in whom chest radiographs are negative for disease, consider chest CT to look for hilar adenopathy. Up to 10% of patients with sarcoidosis who have negative chest radiographs may exhibit hilar pathology on chest CT.

Procedures

• Biopsy of any conjunctival nodules or the lacrimal gland may help in diagnosing sarcoidosis.
• Vitreous biopsy may be indicated if the diagnosis of intraocular (CNS) lymphoma is seriously considered or if a diagnostic dilemma exists in which a specific tissue diagnosis may alter or direct therapy.
• Lumbar puncture may be required to help rule out intraocular (CNS) lymphoma.
• If the patient presents with a secluded pupil from extensive posterior synechiae, iris bombe with angle-closure glaucoma may be present. Perform iridotomy as soon as possible.

TREATMENT

Section 6 of 11  

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• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Medical Care

Treatment for inflammation of the anterior segment is as follows.

• Cycloplegia: Use a long-acting cycloplegic agent, such as cyclopentolate or homatropine, to relieve both pain and photophobia (if present) and to prevent the formation of posterior synechiae.
• Corticosteroids: Topical corticosteroids are the mainstay of therapy and should be used aggressively during the initial phases of therapy.
• • If the patient poorly complies with topical therapy or if the iritis is not responding to topical corticosteroids, a subconjunctival injection of depot steroids (eg, Celestone) may be used.
  • Depot steroids should be avoided in cases of uveitis secondary to herpetic or toxoplasmosis because of their potentially severe adverse effects.
  • In severe cases of iritis, oral corticosteroids may be added to the treatment regimen.
• Aqueous suppressant: If the IOP is elevated, a topical aqueous suppressant should be used.

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

Section 7 of 11  

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• Follow-up
• Miscellaneous
• Multimedia
• References

Topical corticosteroids and a cycloplegic agent should be started immediately. If the eye does not adequately respond to topical therapy within 1 week or so, 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.

Drug Category: Corticosteroids

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

Drug Category: Cycloplegia

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

FOLLOW-UP

Section 8 of 11  

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• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Further Outpatient Care

• Patients should be observed closely, and steroids should be tapered as the inflammation resolves. It is prudent to reexamine the patient 2-3 weeks after all medications have been tapered to ensure that no residual inflammation is present.
• Consultations with other subspecialists should be arranged, if warranted by the patient's history and laboratory workup. Consultation with a uveitis subspecialist should be considered in unusual or difficult cases, cases not responding or progressing despite appropriate maximal therapy, or cases at risk for significant visual loss.

Complications

• Recurrent episodes of iritis and subsequent therapy may lead to cataract formation and to the development of glaucoma.

Prognosis

• Most patients will more than likely have a recurrence of their inflammatory process.
• The overall visual prognosis for patients with recurrent iritis is good in the absence of cataracts, glaucoma, or posterior uveitis.

Patient Education

• For excellent patient education resources, visit eMedicine's Eye and Vision Center. Also, see eMedicine's patient education articles Anatomy of the Eye and Iritis.

MISCELLANEOUS

Section 9 of 11  

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• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Medical/Legal Pitfalls

• Although the etiology of the anterior granulomatous uveitis may be difficult to identify, it is nevertheless important to attempt to rule out all possible associated systemic disease processes.
• Certain disease processes should be carefully considered (with important consequences if overlooked) when these patients are evaluated. Examples are intraocular lymphoma or multiple sclerosis.
• Ensure that all possibilities in the differential diagnosis list have been adequately explored in the history and workup.

MULTIMEDIA

Section 10 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Media file 1:  Granulomatous anterior uveitis with mutton-fat keratic precipitates and Koeppe and Busacca nodules.
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Media type:  Photo

Media file 2:  Granulomatous anterior uveitis with numerous Busacca nodules on the iris surface and a few mutton-fat keratic precipitates on the inferior aspect.
	View Full Size Image
Media type:  Photo

REFERENCES

Section 11 of 11

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

• Lobo A, Barton K, Minassian D, du Bois RM, Lightman S. Visual loss in sarcoid-related uveitis. Clin Experiment Ophthalmol. Aug 2003;31(4):310-6. [Medline].
• McCannel CA, Holland GN, Helm CJ, Cornell PJ, Winston JV, Rimmer TG. Causes of uveitis in the general practice of ophthalmology. UCLA Community-Based Uveitis Study Group. Am J Ophthalmol. Jan 1996;121(1):35-46. [Medline].
• Nussenblatt RB, Whitcup SM. Uveitis: Fundamentals and Clinical Practice. 3^rd ed. Mosby-Year Book; 2003.
• Pepose JS, Holland GN, Wilhelmus KR. Ocular Infection and Immunity. Mosby-Year Book; 1996.
• Rao NA, Cousins S, Forster D. Intraocular Inflammation and Uveitis: Basic and Clinical Science Course. 1999.
• Rosenbaum JT, George RK. Uveitis. In: Current Ocular Therapy 5. 2000:519-21.

Article Last Updated: Sep 5, 2007