In order to manage AU properly, you must first classify it correctly:

Location (anterior, intermediate, posterior, panuveitis)
Course (acute, chronic, recurrent)
Unilateral vs. bilateral
Nongranulomatous vs. granulomatous
Masquerade syndromes

	URL: http://www.opt.indiana.edu/ce/antuve/class.htm Revised: March 31, 2006
	IU Optometry home page: http://www.opt.indiana.edu/ Comments (content): Dr. Julie Torbit at jtorbit@indiana.edu Comments: Web Administrator Page design and coding: Terri Greene Copyright © 2006, The Trustees of Indiana University

Nongranulomatous

• Acute or chronic (see table below), painful
• Circumlimbal flush
• Moderate to severe presentation
• Normal keratic precipitates (KPs)
• More cell, less flare
• Mild to moderate posterior synechiae
• Choroidal involvement rare

Acute Nongranulomatous

Idiopathic Ankylosing spondylitis Spondyloarthropathies Group of rheumatoid negative diseases that are characterized by patients with a gene marker HLA-B27. Ankylosing spondylitis Reactive arthritis (formerly Reiter's syndrome) Psoriatic arthritis Inflammatory bowel disease (IBD) Undifferentiated spondyloarthropathy Whipple's disease Within the normal American population, 6 to 8 percent of the people are HLA-B27 positive [4]. If you suspect these conditions, ask about lower back pain, pain on urination, peripheral arthritis, diarrhea, abdominal pain, and psoriasis. Anklylosing Spondylitis (AS) Occurs in up to 1% of the Caucasian population [3] The cause of AS is unknown (viral or bacterial infections might trigger the disease) Strong association with HLA-B27 (about 90% of patients with AS are HLA-B27 positive) Typically observed in young men Peak age of onset is mid to late 20s Females tend to have a milder course of AS Used to be thought men were affected 10 times more frequently than women; however, newer studies put the male to female ratio anywhere from 1:1 to 5:1 [3] Symptom: Chronic pain of the lower spine and buttock areas characterized by morning stiffness that lessens as the day progresses Pain improves with exercise, hot bath, NSAIDs There is no specific diagnostic test for AS. Lab tests do not give a definitive diagnosis. Anklylosing spondylitis is diagnosed from history, clinical presentation, x-rays, and serologic test results. Uveitis occurs in about 25 percent of patients with AS [2]. Anklylosing spondylitis is the most frequently identified systemic disease associated with recurrent or acute anterior uveitis [5]. Often times the uveitis can present years before the skeletal signs. Tests to run if you suspect AS: X-ray of lower spine HLA-B27 CRP (C reactive protein) ESR (erythrocyte sedimentation rate) ANA (antinuclear antibody) RF (rheumatoid factor) Typically the sacroiliac joint is affected in AS patients. X-rays can detect sclerosis, fusion, or calcification of the vertebral column and sacroiliac joints. Consider anklylosing spondylitis if you have an acute, recurrent, unilateral anterior uveitis. Crohn's disease Reiter's syndrome Reiter's Syndrome (Reactive Arthritis) Classic triad (although Reiter's does not always present with classic triad): Arthritis Conjunctivitis Urethritis The most common agents that cause Reiter's syndrome or reactive arthritis are Salmonella, Shigella, and Chlamydia. Tests to run: Joint X-rays CBC (complete blood count) ESR C-reactive protein HLA-B27 RF ANA Serum creatinine Psoriatic arthritis Rheumatoid arthritis Behçet's disease Behçet's Disease Behçet's disease is a rare, chronic autoimmune condition that affects the inner lining of the mouth, the genitals, and the small blood vessels throughout the body. It typically causes painful mouth, skin, and genital ulcers/lesions. The posterior portion of the eye may be affected and a retinal vasculitis, papillitis, or macular edema may be present. Arthritis is associated with the disease. In the US, females are affected more than males (5:1) [6]. Highest prevalence occurs in the Middle East, Turkey, and Japan. Behçet's disease is associated with HLA-B5. Lab tests to run: ESR C-reactive protein HLA-B5 HLA-B51 Lyme disease Herpes simplex or zoster HIV/AIDS Lens-induced uveitis UGH (uveitis-glaucoma-hyphema) Postoperative Trauma Glaucomatocyclitic crisis (Posner-Schlossman syndrome) Glaucomatocyclitic Crisis (Posner-Schlossman Syndrome) Consider this diagnosis when you have a mildly symptomatic red eye with high IOP (30-60 mmHg). The KPs will be distributed mostly over the inferior cornea. A mild anterior chamber reaction (may only be a cell or two) can be seen as well as corneal edema. Elevated IOP level is disproportional to amount of inflammation. Inflammatory signs can precede elevated IOP. There is a strong association with primary open angle glaucoma (POAG) in these patients. The condition is self limiting and will spontaneously resolve. Be sure to treat the mild anterior uveitis as well as the elevated pressure--avoiding miotics and prostaglandin analogs. The cause of glaucomatocyclitic crisis is unknown. Recent research suggests that the Herpes virus and/or cyctomeglovirus (CMV) may be causing a trabeculitis [7]. Drug use: Ribabutrin and Cidoforvir Interstitial nephritis Autoimmune disease Lupus Polychrondiritis Wegener's granulomatosis Rare Causes Mumps, flu, adenovirus, measles, chlamydia Tight contact lens Kawasaki's disease Leptospirosis Richettsial disease

Chronic Nongranulomatous

Juvenile rheumatoid arthritis (JRA) Fuchs' heterochromatic iridocyclitis Fuchs' Heterochromic Iridocyclitis The etiology is unknown. Chorioretinal lesions reminiscent of Toxoplasmosis are often found in the fundus. Rubella antibodies are also high in patients with Fuchs' [8]. Typical triad is: Heterochromia (may be absent or subtle; dark eyes may not show heterochromia) Uveitis Cataract Transillumination defects of the iris (TIDs) may be present. KPs will be distributed over the ENTIRE endothelium (pathognomonic for this condition). Fuchs' does not respond well to topical steroids. (photo courtesy of Dr. Brad Sutton, IU School of Optometry) Autoimmune disease Lupus, polychrondritis, Wegener's Postoperative Trauma

Granulomatous

Chronic, insidious Minimal injection Mild presentation Mutton fat keratic precipitates (KPs) Less cell, more flare Heavy posterior synechiae Posterior involvement common Koeppe and Busacca nodules Rarely, if ever, idiopathic!

Mutton Fat KPs (photos courtesy of Dr. Brad Sutton)

Chronic Granulomatous Sarcoidosis The etiology of sarcoidosis is unknown. Sarcoidosis can affect any organ or system in the body. Peak incidence occurs in third and fourth decade; although, it can affect any age--even children. Sarcoidosis is slightly more common in females, and in the US population, it is more frequently seen among African Americans than Caucasians (3.2:1); however, worldwide 79 percent of patients with sarcoidosis are Caucasian [9]. It is also more common among the Scandinavian population. Diagnosis of sarcoidosis is one of exclusion and requires a tissue biopsy finding of a non-caseating (non-necrotizing) granuloma not caused by any other etiology. About one quarter of patients with sarcoidosis develop uveitis [9,10], and 80% of the time it is anterior in nature [9]. Tests for sarcoidosis are: Chest X-ray (typically shows bilateral hilar lymphadenopathy; around 90% of patients with sarcoidosis have lung disease [10]) Blood tests: Serum ACE (Angiotensin Converting Enzyme) level Sarcoid granulomas produce angiotensin converting enzyme; ACE levels are elevated in 50 to 80 percent of patients with sarcoidosis [11]. It does not, however, confirm sarcoidosis because it can be elevated in many conditions (e.g., DM, hyperthyroidism, TB, histoplasmosis, hepatitis). ACE levels are most helpful to follow the activity of the disease and evaluating treatment. Syphilis Syphilis is an uncommon cause of uveitis in the US. Syphilis itself is caused by a the bacteria Treponema pallidum. Uveitis associated with syphilis is typically granulomatous in nature, but might present as an acute nongranulomatous uveitis. Complete serologic testing is relatively sensitive and inexpensive [12]. It is wise to run testing for syphilis on all sexually active adolescents and adults with uveitis. You can cure this uveitis! Physical findings might include: Primary chancre at site of inoculation Maculopapular rash on the palms, soles of feet, and scalp Chorioretinitis Non-Treponemal lab tests are quick and nonspecific. They reflect the disease activity. They include: RPR (Rapid Plasma Reagin) VDRL (Venereal Disease Research Laboratory) A negative result does not rule out syphilis because the RPR or VDRL reverts back to negative with treatment. False positives can occur with viral illnesses, pregnancy, autoimmune disease, and drug use. Treponemal specific tests, on the other hand, are highly sensitive and specific. They are: FTA-ABS (Fluorescent Treponemal Antibody Absorption Test) MHA-TP (Micro-Hemagglutination Treponemal Pallidum Test) Once positive, remains so for life; remains positive with treatment. Cross-reactivity with Lyme disease can occur. Have a 20% false negative rate with primary syphilis [13]. All syphilis tests will be negative within the first 4-8 weeks. Interpretation of Tests RPR FTA-ABS Interpretation + + Active syphilis - + Adequate treatment + - False positive - - No exposure or recent exposure Typically the RPR or VDRL is used as a screening test and, if positive, confirmed with FTA-ABS or MHA-TP. Tuberculosis Tuberculosis is a rare cause of uveitis--even less common cause of uveitis than syphilis. Consider tuberculosis in patients with family history or travel exposure --OR-- history of fever, cough, or night sweats. Patients with uveitis and a (+) PPD result have only a 1 percent likelihood of having TB [12]. Tests: Chest x-ray; PPD (?) Approximately 15 percent of patients acutely ill with TB will have a negative PPD due to having other infections, age, medications, surgery, stress, other infections, or live virus vaccinations [11]. The definitive test for TB is an examination/culture of sputum.

Masquerade Syndromes

• Retinal detachment
• Retinoblastoma
• Malignant melanoma
• Leukemia, lymphoma
• Intraocular foreign body
• Spill-over from any posterior uveitis
• Ocular ischemic syndrome
• Juvenile xanthogranuloma

MUST dilate patients with anterior uveitis to aid with diagnosis and to ensure there is no posterior pole involvement.

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