DIAGNOSIS AND MANAGEMENT OF ANTERIOR UVEITIS POA SPRING CONFERENCE - 2009 Classification of Anterior Uveitis BERNARD H. BLAUSTEIN, O.D., F.A.A.O. A. Anatomic location 1. Iritis Cells and flare mostly in anterior chamber with some spill over into the retrolentil space 2. Cyclitis Cells and flare mostly in the retrolentil space 3. Iridocyclitis Cells and flare equally dispersed in the anterior chamber and retolentil space 4. Intermediate uveitis (pars planitis) Cellular inflammatory material in the vitreous over the ora serrata and pars plana B. Chronicity 1. Acute evolves rapidly, is symptomatic, has profound signs, and lasts 8 weeks or less with treatment 2. Chronic evolves slowly, is mildly symptomatic or non-symptomatic, may have a minimal A/C reaction, and may persist for months Characteristics of Anterior Uveitis A. Circumcorneal flush - violet in color, represents dilation of the deep episcleral limbal plexus B. IOP usually reduced; but may be elevated if trabecular meshwork is clogged or synechiae block aqueous outflow C. Relative miosis in the affected eye D. Flare represents leakage of plasma proteins; proteins may be small causing a foggy appearance to the aqueous; proteins may be large and viscous and may congeal (plastic iritis) E. Cells represent a more profound inflammatory response; float in the aqueous convection currents 1. Nongranulomatous lymphocytes and plasma cells that appear as fine glistening dots 2. Granulomatous clumps of lymphocytes and macrophages that are fused together forming large cellular deposits; may form white-yellow nodules on the pupil border (Koeppe nodules) or on the body of the iris (Busaca nodules) 3. Hypopyon Certain acute non-granulomatous inflammations and infectious corneal ulcers cause a WBC layer in the A/C F. Keratitic percipitates (KPs) cells deposit on the endothelium of the cornea; in granulomatous inflammations the KPs have a greasy, mutton-fat appearance
-2- Masqueraders of anterior uveitis A. Rhegmatogenous retinal detachment (RD) RD secondary to a retinal break; may result in cells in the A/C and vitreous B. Choroidal melanoma may result in cells and pigment in the A/C and vitreous C. Large cell lymphoma massive vitreous cells and debris with a mild A/C reaction in patients over 50 D. Ocular ischemia - may result in cells and flare in A/C in elderly patients with carotid insufficiency Potential Morbidity of Anterior Uveitis A. Synechiae 1. Peripheral anterior synechiae adhesions between iris and cornea 2. Posterior synechiae adhesions between pupil border and anterior lens capsule B. Band keratopathy calcium salts deposit in the corneal epithelium and Bowman s membrane in chronic anterior uveitis C. Iris neovascularization new blood vessel growth around the pupil in chronic anterior uveitis D. Iris atrophy loss of iris stroma in chronic anterior uveitis E. Secondary cataract occurs in chronic anterior uveitis F. Cystoid macular edema accumulation of intraretinal fluid in the perifoveal in chronic anterior uveitis Treatment of Anterior Uveitis A. Cycloplegia, i.e. 1% cyclopentolate QID if uveitis is mild; 5% homatropine or.25% scopolamine TID if uveitis is moderate; atropine 1% BID if uveitis is severe B. Topical steroids, e.g. prednisolone acetate, with dosage varying from QID to QH depending upon severity C. Non- responsive severe anterior uveitis - subtenons repository depot of methylprenisolone D. Systemic steroids E. Immunosupressants F. Treat systemic etiology Etiology of Anterior Uveitis A. Idiopathic in 30% of cases B. Trauma to the anterior segment often results in flare without cells C. Collagen vascular autoimmune disease 1. Juvenile rheumatoid arthritis bilateral, nongranulomatous anterior uveitis; girls under age 16; fewer than 5 joints; usually, the knee is affected.
-3-2. Ankylosing spondylitis nongranulomatous anterior uveitis; young men; low back pain; HLA B27 positive 3. Psoriatic arthritis nongranulomatous anterior uveitis; psoriasis and arthritis; HLA B27 positive 4. Behcet s disease nongranulomatous anterior uveitis with hypopyon; young Middle Eastern or Asian men; ulcers in the mouth and genitalia; arthritis 5. Reiter s syndrome (Reactive arthritis) nongranulomatous anterior uveitis; urethritis; tendonitis of Achilles tendon and wrist tendon; arthropathies; HLA B27 positive 6. Inflammatory bowel disease nongranulomaous anterior uveitis; ulcerative colitis or Crohn s disease; HLA B27 positive 7. Granulomatous infectious disease a. Syphilis granulomatous anterior uveitis b. Tuberculosis granulomatous anterior uveitis 8. Granulomatous noninfectious disease a. Sarcoidosis unilateral nongranulomatous or bilateral granulomatous anterior uveitis 9. Nongranulomatous infectious disease a. Herpes simplex b. Herpes zoster c. Lyme Workup of Anterior Uveitis A. Complete history, attempting to define etiology B. Complete dilated ocular health evaluation 1. Grade flare by determining clarity of the iris: Grade 1 - iris has trace haze Grade 2 - iris mildly hazy Grade 3 - iris moderately hazy; can barely see crypts Grade 4 iris not visible 2. Grade cells by cell count Grade 1-1-10 cells Grade 2-16-25 cells Grade 3-25-60 Grade 4 - greater than 60 cells 3. Evaluate vitreous for cells 4. Evaluate retina for detachment, optic nerve infiltration, and inflammation. C. Laboratory evaluation is indicated if anterior uveitis is bilateral, recurrent, granulomatous, or symptoms suggest systemic association. 1. Complete blood count with RBC indices, WBC differentials 2. Urinalysis 3. Blood chemistry (SMA12) a. Alkaline phosphatase g. Creatinine
-4- b. Aspartate transferase h. Glucose c. Bilirubin i. Lactic acid dehydrogenase d. Blood urea nitrogen (BUN) j. Phosphorous e. Calcium k. Total protein albumin f. Cholesterol l. Uric acid 4. Antinuclear antibody antigen (ANA) 5. Erythrocyte sedimentation rate (ESR) 6. Rapid plasma regain (RPR) 7. Fluorescent treponemal antibody absorption test (FTA-ABS) 8. Lyme titer 9. HLA panel (HLA) 10. Angiotensin converting enzyme (ACE) 11. Chest x-ray Normative Values Hematology RBC Male 4.6-6.2 million/ul Female 4.2-5.4 million/ul HCT Male 40-54% Female 38-47% Hgb Male 14-18 g/dl Female 12-16% MCV 82-98 cu microns MCH 27-31 pg MCHC 32-36% WBC Adult 4,500-11,000 WBC Differential Neutrophil 56% Lymphocyte 34% Monocyte 7.0% Eosinophil 2.7% Platelets 140,000-400,000/uL ESR Male Less than (1/2 age) mm/hr Female Less than ½ (age+10 mm/hr Clinical Significance of Blood Chemistry Panel Aspertate Aminotransferase Elevation implies liver disease or MI Alkaline Phosphatase Elevation implies liver disease and/or bone formation Bilirubin Elevation implies liver disease Blood Urea Nitrogen (BUN) Elevation implies kidney disease Calcium Elevation implies hyperthyroidism or sarcoidosis Cholesterol Elevation implies risk for CVA or MI Creatinine Elevation implies kidney disease Glucose Elevation implies diabetes mellitus Lactic Acid Dehydrogenase Elevation implies MI
-5- Phosphorous Protein/Albumin Uric Acid Elevation implies chronic kidney disease Elevation implies chronic liver disease Elevation implies kidney disease or gout Clinical Significance of Other Tests ACE ANA AntiDNA RPR FTA-ABS ESR HLA Panel Lyme Radiology Elevated in many granulomatous diseases, particularly sarcoid A screening test for collagen-vascular autoimmune diseases, particularly systemic lupus erythematosis (SLE) Specific for SLE A non-treponemal antibody test that screens for syphilis; has many false positives A treponemal antibody test that confirms syphilis; may be falsely positive in Lyme disease A nonspecific indicator for systemic inflammation Screens for a number of antigens that are associated with certain autoimmune diseases, e.g. HLA-B27 is positive in 90% of ankylosing spondylitis and 80% of Reiter s disease Lung x-ray diagnoses TB or sarcoid; gallium scan is specific for sarcoid SARCOIDOSIS A. A noninfectious, idiopathic, chronic multisystem disease that affects patients between ages 20-40 B. In US, blacks affected 10-17 times more often than whites C. No link to HLA antigens D. Characteristic lesion is a noncaseating granuloma, i.e. an accumulation of T- lymphocytes surrounding an aggregate of fused, enlarged macrophages with no caseation. E. Granulomas do not secrete proinflammatory cytokines. F. Granulomas disrupt function of organs by deranging and distorting normal architecture. G. Granulomas can be found in any organ; lung is involved in over 90%; thoracic lymph nodes involved in 75-90%; liver involved in 60-90%; eyes and skin involved in 25%; joints involved in 15-20%; heart, bone nervous system and kidney involve to a lesser degree H. 10% of patients die; 50% of patients resolve spontaneously; most patients suffer no significant morbidity. I. Systemic prednisone is the treatment of choice.
-6- Diagnosis of Sarcoidosis A. Biopsy of sarcoid nodules B. Lung x-ray reveals infiltrates. C. Gallium scan reveals uptake in hilar lymph nodes and in other sites. D. ACE is elevated. E. Calcium is often elevated. G. Serum lyzozyme is elevated Potential Eye Signs of Sarcoidosis A. Granulomatous or nongranulomatous anterior uveitis B. Lacrimal gland infiltration leading to keratoconjunctivits sicca C. Schlemm s canal and trabecular meshwork infiltration leading to glaucoma D. Vitritis (fluffy snowballs) E. Optic disc edema that can develop into optic neuritis and optic neuropathy F. Periphlebitis of peripheral venules (candle wax drippings) G. Peripheral venule occlusions leading to peripheral neovascularization