eMedicine Specialties > Ophthalmology > Iris & Ciliary Body

Uveitis, Anterior, Granulomatous

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

Updated: Sep 5, 2007

Introduction

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

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

Differential Diagnoses

Other Problems to Be Considered

Masquerade syndromes
Intraocular lymphoma
Chronic retinal detachment

Workup

Laboratory 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

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

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.

Corticosteroids

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

Prednisolone acetate 1% (Pred Forte, Econopred)

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

Dosing

Adult

1 gtt q1-2h initially; frequency based on severity of iritis

Pediatric

Administer as in adults

Interactions

None reported

Contraindications

Documented hypersensitivity; viral, fungal, or tubercular infections

Precautions

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Caution in hypertension; known to cause cataract formation with long-term use; suspect fungal invasion in any persistent corneal ulceration where a corticosteroid has been used or is in use (obtain fungal cultures when appropriate)

Prednisone (Meticorten, Deltasone, Orasone)

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.

Dosing

Adult

40-60 mg PO qd; taper over 2-4 wk after satisfactory response

Pediatric

0.5-1 mg/kg PO qd

Interactions

When used with digoxin may increase risk of digitalis toxicity; monitor for hypokalemia in patients taking diuretics

Contraindications

Documented hypersensitivity; avoid use in viral, fungal, or tubercular processes

Precautions

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

Abrupt discontinuation of glucocorticoids may cause adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, and infections may occur with glucocorticoid use

Cycloplegia

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

Cyclopentolate hydrochloride 1% (AK-Pentolate, Cyclogyl)

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.

Dosing

Adult

1 gtt qd/tid

Pediatric

Administer as in adults

Interactions

May antagonize antiglaucoma effects of ophthalmic cholinesterase inhibitors

Contraindications

Documented hypersensitivity

Precautions

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

May cause psychotic reaction in children; may produce reactions similar to those of other anticholinergics

Follow-up

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

Medicolegal 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

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

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.

References

1. 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].

2. 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].

3. Nussenblatt RB, Whitcup SM. Uveitis: Fundamentals and Clinical Practice. 3^rd ed. Mosby-Year Book; 2003.

4. Pepose JS, Holland GN, Wilhelmus KR. Ocular Infection and Immunity. Mosby-Year Book; 1996.

5. Rao NA, Cousins S, Forster D. Intraocular Inflammation and Uveitis: Basic and Clinical Science Course. 1999.

6. Rosenbaum JT, George RK. Uveitis. In: Current Ocular Therapy 5. 2000:519-21.

Keywords

iritis, iridocyclitis

Contributor Information and Disclosures

Author

Roger K George, MD, Director of Uveitis Service, Madigan Army Medical Center; Clinical Instructor, Department of Ophthalmology, Oregon Health and Sciences University
Roger K George, MD is a member of the following medical societies: American Uveitis Society
Disclosure: Nothing to disclose.

Medical Editor

Andrew A Dahl, MD, Residency Director, Ophthalmology, Kingston Hospital, Department of Ophthalmology, Assistant Professor of Surgery (Ophthalmology), Mid Hudson Family Practice Institute
Andrew A Dahl, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Ophthalmology, American College of Surgeons, American Medical Association, American Society of Cataract and Refractive Surgery, and Wilderness Medical Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Simon K Law, MD, PharmD, Assistant Professor of Ophthalmology, Jules Stein Eye Institute; Chief of Section of Ophthalmology Surgical Services, Department of Veterans Affairs Healthcare Center, West Los Angeles
Simon K Law, MD, PharmD is a member of the following medical societies: American Academy of Ophthalmology, American Glaucoma Society, and Association for Research in Vision and Ophthalmology
Disclosure: Nothing to disclose.

Managing Editor

R Christopher Walton, MD, Director of Uveitis and Ocular Inflammatory Diseases Service, Associate Professor, Department of Ophthalmology, University of Tennessee College of Medicine
R Christopher Walton, MD is a member of the following medical societies: American Academy of Ophthalmology and American Medical Association
Disclosure: Nothing to disclose.

CME Editor

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
Hampton Roy Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and Pan-American Association of Ophthalmology
Disclosure: Nothing to disclose.

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