Chapter 161 – General Approach to the Uveitis Patient and Treatment Strategies
DAVID J. FORSTER
• Uveitis is any condition that involves inflammation of the uveal tract (iris, ciliary body, choroid) or adjacent structures.
• Inflammatory cells in the anterior chamber and/or vitreous cavity.
• Pain, redness, photophobia, blurred vision, floaters.
• Localized infiltration of inflammatory cells (e.g., keratic precipitates, iris nodules, retinochoroidal infiltrates).
• Anterior or posterior synechiae.
• Disc or macular edema, sheathing of retinal vessels.
• Secondary cataract or glaucoma.
Uveitis encompasses a myriad of conditions, all of which are characterized by inflammation of the uveal tract (iris, ciliary body, choroid), either directly or indirectly. The ophthalmologist’s goal in treating these potentially blinding conditions is to eliminate the inflammatory reaction within the eye while minimizing the potential risks of therapy to the patient. This is best achieved once an accurate diagnosis has been obtained. To do this most efficiently, an extensive history and ophthalmologic examination are required.
EPIDEMIOLOGY AND PATHOGENESIS
Numerous classification schemes have been used to categorize the various types of uveitis. These are based on such factors as follows:
• Location (e.g., anterior, posterior),
• Course (acute, chronic, recurrent),
• Pathology (granulomatous, nongranulomatous), and
• Causative factors (e.g., infectious, autoimmune, systemic, neoplastic diseases).
The classification scheme recommended by the International Uveitis Study Group is based on anatomical location ( Box 161-1 ).
Large series of uveitis patients show variation in terms of the relative prevalence of different forms of uveitis. In surveys of patients referred to tertiary centers, anterior uveitis has been shown to account for 28–66% of cases, intermediate uveitis for 5–15%, posterior uveitis for 19–51%, and panuveitis for 7–18%.  However, in a large community-based study, the vast majority of uveitis cases were anterior (71%), followed by posterior uveitis (5%) and intermediate and panuveitis (1% each). As regards specific entities associated with various types of uveitis, the numbers again vary from survey to survey. The most common causes of anterior uveitis are idiopathic (38–56%), the seronegative spondyloarthropathies (21–23%), juvenile rheumatoid arthritis (JRA; 9–11%), and herpetic keratouveitis (6–10%). The vast majority of cases of intermediate uveitis are idiopathic.
The Classification of Uveitis
Iritis, anterior cyclitis, iridocyclitis
INTERMEDIATE UVEITIS (FORMERLY KNOWN AS PARS PLANITIS)
Posterior cyclitis, hyalitis, basal retinochoroiditis
Focal, multifocal, or diffuse choroiditis, chorioretinitis, retinochoroiditis, or neurouveitis
Toxoplasmosis is the most common cause of posterior uveitis, and the most common causes of panuveitis are idiopathic (22–45%) and sarcoidosis (14–28%).   
Because uveitis is frequently associated with other systemic conditions, it is critical that a comprehensive history and review of systems be obtained for every patient who presents with intraocular inflammation (see Box 161-2 ). This is the first step in the process of appropriately classifying the type of uveitis present and is indispensable in helping to arrive at the correct diagnosis.
The clinical manifestations of uveitis vary depending on several factors—the primary site of involvement in the eye, the course of the inflammatory process (e.g., acute or chronic), and the presence of secondary complications arising from the uveitis itself.
The symptoms of acute anterior uveitis (e.g., human leukocyte antigen HLA-B27–related entities, such as ankylosing spondylitis) generally include pain, redness, photophobia, and blurred vision, which typically develop over a period of hours or days. On the other hand, patients who have chronic anterior uveitis, such as that seen with JRA or Fuchs’ heterochromic iridocyclitis, may present merely with blurring of vision or mild redness, with little pain or photophobia. Patients who have intermediate or posterior uveitis typically present with floaters or impaired vision secondary to cystoid macular edema or chorioretinal involvement. Patients who have panuveitis may present with any or all of these symptoms.
On clinical examination, findings likewise vary depending on the location, course, and pathogenesis of the inflammation. Virtually any ocular tissue may yield findings that may be helpful in the diagnosis. The conjunctiva may show ciliary flush (perilimbal injection characteristic of anterior uveitis) or nodules (e.g., in sarcoidosis). The cornea should be examined for keratic precipitates, which are collections of inflammatory cells on the endothelial surface ( Fig. 161-1 ). Small keratic precipitates are usually seen in nongranulomatous types of uveitis, whereas larger (“mutton fat”) keratic precipitates are characteristic of granulomatous uveitis. The normal convection currents of the
History Taking in Uveitis**
Onset, course, symptoms, laterality, treatment
PAST OCULAR HISTORY
Previous episodes, past therapy and response, previous or antecedent ocular trauma or surgery
Systemic illnesses (especially sarcoidosis, juvenile rheumatoid arthritis, AIDS, tuberculosis, syphilis), prodromal syndromes, medications (especially immunosuppressive agents)
Dietary habits, sexual history, intravenous drug abuse
Age, race, sex
Birthplace, previous locations (e.g., Mississippi River valley for ocular histoplasmosis), foreign travel
Medical illnesses, contagious diseases (e.g., tuberculosis), maternal infections, history of uveitis
REVIEW OF SYSTEMS
General—fever, weight loss, malaise, night sweats
Rheumatologic—arthralgias, lower back pain, joint stiffness
Dermatologic—rashes, sores, alopecia, vitiligo, poliosis, tick/insect bites
Neurologic—tinnitus, headache, meningism, paresthesias, weakness/paralysis
Respiratory—shortness of breath, cough, sputum production
Gastrointestinal (GI)—diarrhea, bloody stools, oral aphthous ulcers
Genitourinary (GU—dysuria, discharge, genital ulcers, balanitis
* A comprehensive history and review of systems is critical in the evaluation of the uveitis patient.
aqueous humor result in keratic precipitates being typically concentrated on the inferior half of the cornea; a more diffuse pattern of keratic precipitates is frequently seen with Fuchs’ heterochromic iridocyclitis or herpetic keratouveitis. If they are new, keratic precipitates are usually white and become more pigmented or shrunken (“crenated”) as they age. The cornea may demonstrate epithelial dendrites, geographic ulcers, or stromal scarring in cases of herpetic keratouveitis. Chronic uveitis may result in the formation of band keratopathy, which is a deposition of calcium at the level of Bowman’s membrane.
Mechanisms of inflammation that occur at the cellular level (e.g., release of chemotactic factors and mediators that increase vascular permeability) result in the presence of cells and flare (protein) in the aqueous humor. Both of these are graded on a scale of zero to 4+. In severe cases of anterior uveitis, fibrin clot and/or hypopyon formation may be seen ( Fig. 161-2 ). With long-standing uveitis (e.g., that seen with JRA), eyes may have persistent flare from damage to the vasculature of the iris and ciliary body. This does not necessarily represent an active inflammatory process; the presence of cells in the anterior chamber is a more accurate marker of active inflammation.
The iris may show either anterior or posterior synechiae. If advanced, pupillary block, iris bombé, and/or angle-closure glaucoma may result. Iris nodules (Koeppe’s nodules at the pupillary border; Busacca’s nodules within the iris stroma) or actual granulomas may be seen in cases of granulomatous uveitis ( Fig. 161-3 ). Sectoral iris atrophy is characteristic of herpetic disease.
Cataract is a common complication of long-standing uveitis as well as chronic corticosteroid therapy. Most such cataracts are posterior subcapsular in location, but cortical opacities may also be seen (see Fig. 161-3 ). Patients who present with post-traumatic or postsurgical inflammation need to be checked carefully for capsular rupture, retained lens material, or intraocular lens (IOL) malposition.
Figure 161-1 Keratic precipitates in anterior uveitis. These keratic precipitates are granulomatous in appearance, as would be expected with entities such as sarcoidosis, Vogt–Koyanagi–Harada syndrome, and sympathetic ophthalmia.
Figure 161-2 HLA B27–related acute anterior uveitis. This severe case of anterior uveitis demonstrates fibrin clot formation and hypopyon in the anterior chamber.
The vitreous may show cellular infiltration, “snowball opacities” (commonly seen in intermediate uveitis and sarcoidosis), fibrosis with resultant traction on the retina, or cyclitic membrane formation behind the lens. It is important to determine where cells are present in the vitreous cavity. In cases of iridocyclitis, cells in the vitreous cavity are found anteriorly, whereas with intermediate or posterior uveitis cells are distributed either throughout the vitreous or more posteriorly.
Posterior segment manifestations of uveitis may include the following:
• Disc eccema
• Macular edema
• Retinal vasculitis
• Perivascular exudates
• Focal or diffuse retinitis or choroiditis
• Pars plana exudates (“snowbanking”)
• Serous, tractional, or rhegmatogenous retinal detachment
• Retinochoroidal atrophy
• Choroidal and retinal neovascularization
Intraocular pressure (IOP) can be affected by uveitis in a number of ways. Frequently, patients have low IOP with acute iridocyclitis due to the inflammation-induced decrease in aqueous production. Long-standing uveitis or cyclitic membrane–induced ciliary body detachment may result in hypotony and eventual phthisis bulbi. Conversely, IOP may be elevated by several mechanisms:
Figure 161-3 Chronic granulomatous uveitis. This patient demonstrates several features of chronic granulomatous uveitis, as may be seen with sarcoidosis, including iris nodules, posterior synechiae, and cataract formation.
• Plugging of trabecular meshwork with inflammatory cells,
• Swelling of meshwork fibers (“trabeculitis”),
• Peripheral anterior synechiae formation,
• Pupillary block from extensive posterior synechiae, and
• Corticosteroid-induced IOP elevation.
Gonioscopy should be performed regularly in patients who have chronic uveitis to check for peripheral anterior synechiae formation and angle closure.
DIAGNOSIS AND ANCILLARY TESTING
Once a list of potential uveitic entities has been developed, ranked from most likely to least likely, a list of laboratory investigations can be formulated that will yield the most useful information with respect to arriving at the final diagnosis. Here, most authorities advocate a “tailored” approach, whereby tests are carried out only for the most likely entities.
Table 161-1 lists the most commonly used laboratory investigations in the work-up of uveitis patients as well as the diseases for which they are useful. These tests are discussed further in the respective chapters for individual uveitic entities.
Most of these uveitis tests are specialized laboratory investigations that should be ordered only when a reasonable chance exists that they will provide useful diagnostic information for a particular patient. No hard-and-fast rules govern when a laboratory work-up should be pursued in patients who have uveitis. Work-up may not be indicated in initial episodes of isolated anterior uveitis. On the other hand, patients who have granulomatous inflammation or posterior uveitis of unclear cause probably merit a work-up because there is more likelihood of finding an underlying disease. The key is to make maximal use of the history, review of systems, and physical examination to tailor the work-up for each patient.
Fluorescein angiography (to assess cystoid macular edema, serous retinal detachments, infiltration of the choroid, and vascular abnormalities) and ultrasonography (to rule out posterior segment pathology in eyes with media opacities) are useful ancillary tests in patients who have uveitis. Diagnostic vitrectomy and/or retinochoroidal biopsy can be of help in suspected cases of infectious uveitis and intraocular neoplasms. Specimens can be sent for culture, antibody testing, electron microscopy, or polymerase chain reaction (PCR) testing.
Once a complete history and physical examination have been performed, the next step in the evaluation of the uveitis patient is to develop a detailed list of differential diagnoses. This process begins by first giving the particular type of inflammation present as specific a descriptor as possible, using the previously mentioned classification schemes. For instance, instead of merely naming the entity “anterior uveitis,” a much more helpful and descriptive term is one such as “acute nongranulomatous anterior uveitis.” The “naming-meshing” system described by Smith and Nozik utilizes this approach to help formulate a list of differential diagnoses. Once the entity is appropriately named, the pattern of uveitis exhibited is matched or meshed with a list of potential types of uveitis that have similar clinical characteristics (see Table 161-1 ). In the preceding example, the most likely diagnoses would be an HLA-B27–related iridocyclitis, Behçet’s syndrome, and herpetic uveitis, whereas diagnoses such as sarcoidosis, JRA, and Fuchs’ heterochromic iridocyclitis fall much lower on the list.
The ultimate goal of the practitioner with respect to the management of the patient with uveitis is to treat effectively the inflammatory process within the eyes while minimizing the complications of both the disease process and the therapeutic regimen selected. To achieve this, the natural history of the particular uveitic entity and its expected impact on visual function need to be fully understood. Certain conditions require the prompt institution of specific therapy (e.g., intravenous acyclovir for the acute retinal necrosis syndrome) or the early institution of immunosuppressive agents (e.g., for Behçet’s syndrome) for optimal control of the inflammatory process. On the other hand, instances appear in which the treatment risks may outweigh the benefits (e.g., a patient who has a small peripheral focus of toxoplasmic retinochoroiditis, or patients with Fuchs’ heterochromic iridocyclitis, in which chronic corticosteroid therapy may have little impact on the degree of intraocular inflammation but may hasten the formation of cataract or further elevate IOP).
The most common anti-inflammatory medications used in the treatment of uveitis are outlined in Table 161-2 . Subsequent chapters detail agents used for specific types of uveitis (e.g., antimicrobial therapy for toxoplasmosis, intravenous acyclovir for acute retinal necrosis syndrome) (see Chapters 169 and 172 ).
Mydriatic and Cycloplegic Agents
These topical medications are used to treat the ciliary spasm that frequently occurs with acute anterior uveitis and to break recently formed posterior synechiae and/or prevent the development of new synechiae. Longer acting agents, such as homatropine, scopolamine (hyoscine), or atropine, are utilized to relieve ciliary spasm, whereas the shorter acting agents (tropicamide or cyclopentolate) may play a role in preventing new posterior synechiae formation in patients who have chronic iridocyclitis (e.g., secondary to JRA) and minimal photophobia in whom the pupil should be kept relatively mobile.
Corticosteroids represent the primary therapeutic modality in patients who have uveitis. These medications produce a broad suppression of the immune system and achieve their anti-inflammatory effect by a number of mechanisms ( Table 161-2 ). Corticosteroids are given topically, by periocular injection, by intravitreal injection, or systemically.
The topical route is useful primarily in patients who have anterior uveitis, as topically applied medications penetrate the posterior segment poorly (unless the patient is pseudophakic or aphakic, in which case topical corticosteroids may have somewhat more effect posteriorly). Prednisolone acetate is generally regarded as the most effective topical corticosteroid for treating anterior uveitis; generic versions may have significantly less anti-inflammatory
TABLE 161-1 — DIFFERENTIAL DIAGNOSIS AND LABORATORY INVESTIGATIONS FOR UVEITIS
Type of Uveitis
Most Common Etiologies
HLA-B27, sacroiliac films
Herpetic (HSV, VZV)
Juvenile rheumatoid arthritis
Antinuclear antibodies (ANAs)
Angiotensin-converting enzyme (ACE), chest x-ray/CT, gallium scan, biopsy
Fuchs’ heterochromic iridocyclitis
Rapid plasma reagin (RPR), FTA-ABS (if RPR positive)
PPD, chest x-ray
MRI of brain
Viral (HSV, VZV, CMV, acute retinal necrosis [ARN])
ELISA, vitrectomy/biopsy for PCR
Fluorescein angiography (FA), lumbar puncture
See subsequent chapters for detailed description of each entity.
CMV, Cytomegalovirus; ELISA, enzyme-linked immunosorbent assay; FTA-ABS, fluorescent treponemal antibody absorption; HLA, human leukocyte antigen; HSV, herpes simplex virus; MRI, magnetic resonance imaging; PPD, purified protein derivative; VKH, Vogt-Koyanagi-Harada; VZV, varicella-zoster virus.
effect. Rimexolone and loteprednol etabonate, while probably not as potent in terms of anti-inflammatory effect, tend to elevate IOP less than prednisolone.
The periocular route is effective for administering corticosteroids to patients who have intermediate uveitis, posterior uveitis, or cystoid macular edema, particularly if unilateral. It may also be beneficial in patients who have severe anterior uveitis unresponsive to topical therapy. Injection is usually performed by a sub–Tenon’s capsule or trans-septal approach, using only topical anesthesia. The longer acting agents (e.g., triamcinolone acetonide or methylprednisolone acetate) are preferred and are usually given every 3–4 weeks until the desired effect is achieved. Periocular injections of corticosteroids should not be used in cases of infectious uveitis (e.g., acute retinal necrosis syndrome, toxoplasmosis) and should be used with caution in patients who have a history of corticosteroid-induced IOP elevation.
The systemic route is used in cases of severe posterior uveitis or panuveitis, especially if bilateral, or in cases of severe anterior uveitis poorly responsive to topical and/or periocular corticosteroids. It is better to start at a higher dose (e.g., 1–2?mg/kg/day or 60–120?mg/day) and taper it as quickly as possible than to start at a low dose and have to increase it repeatedly because of poor response. These agents should be tapered gradually if the patient has been taking them for longer than 2–3 weeks.
Nonsteroidal Anti-Inflammatory Drugs
Nonsteroidal anti-inflammatory drugs (NSAIDs) do not play a primary role in the treatment of most types of uveitis. However, at times the adjunctive use of oral NSAIDs may allow maintenance of the patient on a lower dose of corticosteroids than would be the case otherwise (e.g., in chronic iridocyclitis secondary to JRA). Oral NSAIDs do have a role, however, in the treatment of certain types of scleritis.
TABLE 161-2 — ANTI-INFLAMMATORY THERAPY IN THE TREATMENT OF UVEITISM
Mechanism of Action
Inhibition of cyclo-oxygenase and lipoxygenase pathways
Topical—elevated IOP, cataract exacerbation of infection, corneal or scleral thinning/perforation
Decreases complement levels
Periocular—same as topical, as well as ptosis, scarring of Tenon’s capsule, scleral perforation, hemorrhage, abscess
Decreased migration of lymphocytes
Systemic—same as topical, as well as weight gain, fluid retention, electrolyte disturbances, peptic ulcer disease, osteoporosis, aseptic necrosis of hip, hypertension, impaired glucose tolerance, mental status changes, impaired wound healing, menstrual irregularities, others
Decreased production of vasoactive amines and interleukins
Decreased circulating monocytes
Decreased macrophage activity
Interfere with DNA synthesis and cellular replication
All:bone marrow suppression, teratogenicity, increased risk of infection
Folate analog;inhibits dihydrofolate reductase
Hepatotoxicity, GI upset, pneumonitis, stomatitis
Alters purine metabolism
GI upset, hepatitis
Inhibits purine synthesis
Lymphotoxicity, cross-links DNA
Hemorrhagic cystitis, sterility, increased risk of malignancy
Lymphotoxicity, cross-links DNA
Sterility, increased risk of malignancy
Inhibits T cells
Renal toxicity, hypertension, hirsutism, tremor
Inhibits T cells
Renal toxicity, hypertension, neurotoxicity, hepatitis, diabetes
As can be seen, the potential complications of such therapy are numerous and must be discussed fully with the patient prior to instituting therapy with these agents.
GI, Gastrointestinal; IOP, intraocular pressure.
Immunosuppressive medications have generally been reserved for severe, sight-threatening uveitis that has not responded adequately to corticosteroids or for patients who experience severe side effects from corticosteroid therapy. However, evidence suggests that these agents can play an important part in the treatment regimen for uveitis, with potentially less morbidity than long-term corticosteroid use. Although several entities exist for which the early use of immunosuppressive agents is indicated (Behçet’s syndrome, Vogt-Koyanagi-Harada (VKH) syndrome, sympathetic ophthalmia, and necrotizing sclerouveitis), they should also be considered in patients who require chronic (e.g., longer than 6 months) corticosteroid therapy at doses greater than 10?mg/day.
Several classes of immunosuppressive medications exist. The commonly used agents, their mode of action, and their potential side effects are summarized in Table 161-2 . Antimetabolites include methotrexate, azathioprine, and mycophenolate. Methotrexate in particular has been used to treat many types of chronic noninfectious uveitis, including JRA-associated iridocyclitis, sarcoidosis, panuveitis, and scleritis.      It is given as a weekly dose of 7.5–25?mg, either orally, subcutaneously, or intramuscularly, and is generally well tolerated. Azathioprine, which is usually given at a dose of 1–3?mg/kg/day, has been shown to be beneficial in patients who have sympathetic ophthalmia, VKH syndrome, intermediate uveitis, and Behçet’s syndrome.  Mycophenolate is usually used at a dose of 1?g twice daily and may be an alternative for patients who are intolerant of methotrexate or azathioprine.
Alkylating agents include cyclophosphamide and chlorambucil and have been used in the treatment of Behçet’s syndrome, sympathetic ophthalmia, and intermediate uveitis.  Cyclophosphamide is usually given at a dose of 1–3?mg/kg/day and chlorambucil at 0.1–0.2?mg/kg/day.
Cyclosporine and tacrolimus are examples of T-cell inhibitors. The primary effect of these medications is inhibition of T-cell activation and recruitment, although the exact mechanism by which this occurs is debated. Cyclosporine has been shown to be effective in various types of posterior uveitis as well as intermediate uveitis.  It is usually begun at a dose of 2–5?mg/kg/day, usually in combination with corticosteroids. The primary side effects are renal toxicity and hypertension, which occur in up to 75% and 25% of patients, respectively. Unlike cytotoxic agents, which can actually kill the cells responsible for inflammatory disease, cyclosporine is cytostatic and merely suppresses the inflammatory cells. Thus, inflammation may recur when the medication is tapered. However, the treatment may be effective long enough to allow the inflammatory activity to subside by the time the medication is withdrawn.
Immunosuppressive medications may have serious and potentially life-threatening side effects (see Table 161-2 ). Most worrisome are renal or hepatic toxicity, bone marrow suppression, and, particularly with the alkylating agents, the potential for future malignancies (e.g., leukemia or lymphoma). These agents are teratogenic, and therefore patients should take precautions against becoming pregnant while taking them. Patients must be informed of such risks. Regular blood monitoring (complete blood count, hepatic function tests) needs to be performed, and most such patients should be observed closely by an internist or other physician familiar with the potential side effects of these medications.
Cytokine inhibitors such as etanercept and infliximab are being studied for treatment of various types of uveitis. In one study, a single intravenous infusion of infliximab resulted in a rapid decrease in intraocular inflammation in patients who have Behçet’s
syndrome. Intravenous immune globulin and interferon alfa-2b were shown in small studies to have beneficial effects in some patients with uveitis.  Also, a multicenter trial is ongoing using a sustained-release intravitreal implant that delivers the corticosteroid fluocinolone acetonide directly into the eye. This therapy has the potential advantage of sustained, consistent intraocular therapy without systemic side effects.
COURSE AND OUTCOME
Uveitis is a potentially blinding condition that can result in serious complications in the eyes. Cataracts are one of the most common causes of visual loss in patients who have uveitis. Cataract extraction should ideally be performed only after uveitis has been quiescent for 3–4 months. Phacoemulsification or nuclear expression (NE) extracapsular cataract extraction (ECCE) may be performed, although phacoemulsification is probably associated with fewer complications than NE. Cases associated with vitritis (e.g., intermediate uveitis) often benefit from combined cataract extraction and pars plana vitrectomy.  With the exception of patients who have JRA-associated uveitis, most patients tolerate a posterior chamber IOL well, provided their inflammation is aggressively treated during the perioperative period.  However, patients who have inflammation centered around the pars plana region tend to have a higher complication rate than those with other types of uveitis. Acrylic or all-polymethylmethacrylate lenses seem to incite less inflammatory response than lenses containing silicone or polypropylene.
Glaucoma is also a common complication of chronic uveitis. The various mechanisms for the development of glaucoma in uveitic eyes were discussed earlier. Medical therapy is used initially, although miotics should be avoided as they may exacerbate ciliary spasm and predispose to posterior synechiae formation. Also, prostaglandin analogs have been associated with anterior uveitis and cystoid macular edema. In cases of pupillary block, iridectomy should be performed. Laser iridectomies frequently close off with continued inflammation; in such cases surgical iridectomy should be performed. If filtering surgery is necessary, the use of antimetabolites (e.g., mitomycin) or aqueous drainage devices greatly increases the success rate in these patients. 
Patients who have uveitis may also have cystoid macular edema, which, if long-standing, can result in irreversible visual loss. Periocular or systemic corticosteroids are usually successful, but, if not, immunosuppressive agents may be helpful. Therapeutic vitrectomy may also be beneficial in some cases as it debulks the vitreous cavity of inflammatory cells and mediators, which may be responsible for chronic vascular leakage.
Retinal detachment can be either serous (e.g., with VKH syndrome), rhegmatogenous (e.g., acute retinal necrosis syndrome), or tractional. Serous detachments usually respond to corticosteroids (either periocular or systemic); rhegmatogenous or tractional detachments may require the use of silicone oil tamponade (especially in cases such as acute retinal necrosis syndrome).
Chronic uveitis may lead to aqueous hyposecretion with decreased nutrient supply to anterior segment structures or cyclitic membrane formation with ciliary body detachment and subsequent hypotony. Cyclitic membranes may be removed through a pars plana approach; such surgery may allow reattachment of the ciliary body and return of normal aqueous production. Chronic hypotony may result in phthisis bulbi.
1. Bloch-Michel E, Nussenblatt RB. International uveitis study group recommendations for the evaluation of intraocular inflammatory disease. Am J Ophthalmol. 1987;103:234–5.
2. Henderly DE, Genstler AJ, Smith RE, Rao NA. Changing patterns of uveitis. Am J Ophthalmol. 1987;103:131–6.
3. Rodriguez A, Calonge M, Pedroza-Seres M, et al. Referral patterns of uveitis in a tertiary eye care center. Arch Ophthalmol. 1996;114:593–9.
4. McCannel CA, Holland GN, Helm CJ, et al. Causes of uveitis in the general practice of ophthalmology. UCLA Community-Based Uveitis Study Group. Am J Ophthalmol. 1996;121:35–46.
5. Smith RE, Nozik RA. Uveitis: a clinical approach to diagnosis and management, 2nd ed. Baltimore: Williams & Wilkins; 1989:23–6.
6. The Loteprednol Etabonate US Uveitis Study Group. Controlled evaluation of loteprednol etabonate and prednisolone acetate in the treatment of acute anterior uveitis. Am J Ophthalmol. 1999;127:537–44.
7. Jabs DA, Rosenbaum JT, Foster CS, et al. Guidelines for the use of immunosuppressive drugs in patients with ocular inflammatory disorders: recommendations of an expert panel. Am J Ophthalmol. 2000;130:492–513.
8. Holz FG, Krastel H, Breitbart A, et al. Low-dose methotrexate treatment in noninfectious uveitis resistant to corticosteroids. Geriatr J Ophthalmol. 1992;1:142–4.
9. Shah SS, Lowder CY, Schmitt MA, et al. Low-dose methotrexate therapy for ocular inflammatory disease. Ophthalmology. 1992;99:1419–23.
10. Samson CM, Waheed N, Baltatzis S, Foster CS. Methotrexate therapy for chronic noninfectious uveitis. Ophthalmology. 2001;108:1134-9.
11. Dev S, McCallum RM, Jaffe GJ. Methotrexate treatment for sarcoid-associated panuveitis. Ophthalmology. 1999;106:111–8.
12. Yazici H, Pazarli H, Barnes CG, et al. A controlled trial of azathioprine in Behçet’s syndrome. N Engl J Med. 1990;322:281–5.
13. Tessler HH, Jennings T. High-dose short-term chlorambucil for intractable sympathetic ophthalmia and Behçet’s disease. Br J Ophthalmol. 1990;74:353–7.
14. Nussenblatt RB, Whitcup SM, Palestine AG. Uveitis, fundamentals and clinical practice, 2nd ed. St Louis: Mosby–Year Book; 1996:97–134.
15. Schreiber SL, Crabtree GR. The mechanism of action of cyclosporin A and FK506. Immunol Today. 1992;13:136–42.
16. Whitcup SM, Salvo EC Jr, Nussenblatt RB, et al. Combined cyclosporine and corticosteroid therapy for sight-threatening uveitis in Behçet’s disease. Am J Ophthalmol. 1994;118:39–45.
17. Sfikakis PP, Theodossiadis PG, Katsiari CG, et al. Effect of infliximab on sight-threatening panuveitis in Behçet’s disease. Lancet. 2001;358:295–6.
18. Rosenbaum JT, George R, Gordon C. The treatment of refractory uveitis with intravenous immunoglobulin. Am J Ophthalmol. 1999;127:545–9.
19. Demiroglu H, Ozcebe OI, Barista I, et al. Interferon alfa-2b, colchicines, and benzathine penicillin versus colchicines and benzathine penicillin in Behçet’s disease: a randomized trial. Lancet. 2000;355:605–9.
20. Smith RE. Pars planitis. In: Ryan SJ, ed. Retina, 2nd ed, Vol 2. St Louis: CV Mosby; 1994:1621–31.
21. Estafanous MFG, Lowder CY, Meisler DM, Chauhan R. Phacoemulsification cataract extraction and posterior chamber lens implantation in patients with uveitis. Am J Ophthalmol. 2001;131:620–5.
22. Walker J, Rao NA, Ober RR, et al. A combined anterior and posterior approach to cataract surgery in patients with chronic uveitis. Int Ophthalmol. 1993;17:63–9.
23. Foster RE, Lowder CY, Meisler DM, et al. Combined extracapsular cataract extraction, posterior chamber intraocular lens implantation, and pars plana vitrectomy. Ophthalmic Surg. 1993;24:446–52.
24. Krishna R, Meisler DM, Lowder CY, et al. Long-term follow-up of extracapsular cataract extraction and posterior chamber lens implantation in patients with uveitis. Ophthalmology. 1998;105:1765–9.
25. Foster CS, Stavrou P, Zafirakis P, et al. Intraocular lens removal from patients with uveitis. Am J Ophthalmol. 1999;128:31–7.
26. Hooper PL, Rao NA, Smith RE. Cataract extraction in uveitis patients. Surv Ophthalmol. 1990;35:120–44.
27. Warwar RE, Bullock JD, Ballal D. Cystoid macular edema and anterior uveitis associated with latanoprost use. Ophthalmology. 1998;105:263–8.
28. Forster DJ, Rao NA, Hill RA, et al. Incidence and management of glaucoma in Vogt-Koyanagi-Harada syndrome. Ophthalmology. 1993;100:613–18.
29. Heiligenhaus A, Bornfeld N, Foerster MH, Wessing A. Long-term results of pars plana vitrectomy in the management of complicated uveitis. Br J Ophthalmol. 1994;78:549–54.