9/8/2017 Allergan Pharmaceuticals Speaker’s Bureau Bio-Tissue BioDLogics, LLC Katena/IOP Seed Biotech Johnson and Johnson Vision Care, Inc. COA Monterey Symposium 2017 Shire Pharmaceuticals Nicholas Colatrella, OD, FAAO, Dipl AAO, ABO, ABCMO Jeffrey R. Varanelli, OD, FAAO, Dipl ABO, ABCMO Nicholas Colatrella, OD, FAAO, Dipl AAO, ABO, ABCMO Jeffrey Varanelli, OD, FAAO, Dipl ABO, ABCMO Uveitis is the third leading cause of blindness in developed nations and accounts for 10-20% of blindness worldwide Annual incidence of uveitis varies between 17 to 52 cases per 100,000 persons per year Prevalence data suggest that uveitis may be found in up to 714 per 100,000 persons While all age groups are affected, the peak onset occurs between 20 and 40 years of age, among working individuals potentially greater personal and economic impact from visual loss than that of many age-related diseases Uveitis may be infectious or non-infectious, and is often Uveitis was initially considered a single disease entity associated with underlying systemic conditions Approach to treatment varied little Specific diagnosis is of critical importance so that antimicrobial therapy Immunologic and microbiologic testing became more is initiated when appropriate sophisticated Neoplastic disease is excluded or appropriately referred Though some diseases are local ocular immune phenomena, Anti-inflammatory therapy is commenced for both ocular and systemic majority are systemic diseases with ocular manifestations inflammatory disease Pathogenesis of uveitis ranges from autoimmunity to neoplasia to viruses 1 9/8/2017 Several diseases are clinical diagnoses and require little Laboratory testing for ocular inflammatory disease is frequently laboratory analysis a challenge Laboratory tests are rarely useful as screening tools The myriad of tests available, the complexities of their interpretation, and the underlying concern that one may miss an Knowledge of pretest probability is helpful important systemic disease all play a role Avoids false positive results Laboratory testing, although important, is not a substitute for a Avoids costly and unnecessary tests thorough history and physical examination of any patient with Diagnostic test is only useful if it can confidently rule in or rule ocular inflammation out a disease Obtaining an accurate and detailed history is the single most valuable tool in establishing a diagnosis of uveitis No amount of laboratory testing can compensate for an incomplete or inaccurate history Purpose of lab testing is to identify etiology in order to direct In 2005, the world’s major uveitis societies initiated a specific treatment for the patient’s condition standardization of nomenclature process i.e. Tuberculous uveitis should not be treated with corticosteroids alone This project, termed SUN (Standardization of Uveitis Identify associated systemic disease Nomenclature), established language for describing the such as the patient with tubulointerstitial nephritis and uveitis with presentation, chronicity, anatomic location, and severity of elevated urinary β-2 microglobulin levels uveitis and its response to treatment Provide valuable prognostic information even when treatment is The Standardization of Uveitis Nomenclature (SUN) Working Group. Standardization of Uveitis Nomenclature for not influenced by the diagnosis Reporting Clinical Data. Results of the First International Workshop. Am J Ophthalmol. 2005;140(3):511 knowing that a patient has HLA-B27–associated uveitis allows the clinician to counsel the patient about the likelihood of recurrent disease and vision loss Category Descriptor Disease is classified based on onset, duration, and course Sudden Onset of inflammation is considered either sudden or insidious Onset Duration is divided into limited (≤3 months) and persistent (>3 Insidious Limited = < 3 months months) Duration Disease course can be described as acute, recurrent, or chronic Persistent = > 3 months Acute - episode characterized by Acute anterior uveitis refers to an episode of sudden onset and limited sudden onset and limited duration duration Course Recurrent - repeated episodes separated Recurrent uveitis describes repeated episodes of uveitis with periods by periods of inactivity without treatment of quiescence off all treatment for more than 3 months ≥3 months in duration In chronic uveitis, a patient is not free of inflammation for longer than Chronic - persistent uveitis with relapse in 3 months while off treatment <3 months after discontinuing treatment 2 9/8/2017 The most common form of uveitis Accounts for approximately 90% of all uveitis cases seen by general OD/MD Type Primary Site of Inflammation Includes May present with wide range of symptoms Their severity may vary with the underlying etiology, abruptness of onset, and tolerance of the Iritis patient Anterior Uveitis Anterior Chamber Anterior cyclitis Pain is often localized to the eye, but also can be referred to the periorbital region, forehead, Iridocyclitis or temple Blurred vision may be seen in cases of severe inflammation with fibrin or with reactive cystoid Pars planitis macular edema (CME) Intermediate Uveitis Vitreous Posterior cyclitis Hyalitis Classic presentation Pain, redness, photophobia Focal, multifocal, or diffuse Vision may be normal or slightly decreased Posterior Uveitis Retina or Choroid choroiditis Perilimbal injection Chorioretinitis KP may be present Retinochoroiditis Cell/flare Retinitis Hypopyon Neuroretinitis Pain is uncommon in chronic anterior uveitis. Instead, blurred vision and dull ache are the Anterior chamber, vitreous, and more common presenting symptoms PanUveitis retina or choroid Defined as intraocular inflammation that predominantly involves Has an association with several systemic disorders peripheral retina, pars plana, and vitreous Initial diagnostic evaluation should exclude masquerade syndromes Patients typically present with painless blurred vision and and infectious diseases floaters Diagnostic approach should focus on history and clinical exam Photophobia and redness are not common May have mild to moderate anterior segment inflammation Anterior vitreous cells are present White clumps of inflammatory cells (snowballs) and whitish yellow exudate (snowbanking) The review of systems is essential in Recent contact with individuals with It is important to observe the patient’s overall health, noting in developing a differential diagnosis for known tubercular disease particular signs such as pallor and nutritional status uveitis and utilizes an extensive Diarrhea or blood in the stool review from a patient questionnaire, Examination of the skin, joints, and oral mucosa, as well as supplemented by direct questioning Skin rashes Items of particular importance Arthritis (axial or peripheral) auscultation of the lungs and heart can be valuable in include: High-risk sexual activities formulation of the differential diagnosis History of oral or genital ulcers Ingestion of game meats By the end of the initial interview, you should have a reasonably Tinnitus or hearing loss Undercooked meats, or tainted water Headaches supplies complete differential diagnosis in mind before examining the Malaise Presence and types of pets patient Chronic cough Shortness of breath Insect bites Recent weight loss or gain Recent foreign travel Fevers, chills, or night sweats 3 9/8/2017 Certain types of uveitis or more common in particular age groups Consideration of gender, race, and ethnicity also useful when considering < 5 Years 5-15 Years 16-35 Years 36-64 years >65 Years diagnosis Ankylosing spondylitis is more common in males Juvenile Juvenile HLA-B27 Pauciarticular juvenile idiopathic arthritis (JIA) occurs most frequently in females. idiopathic idiopathic Idiopathic Idiopathic associated Occupation may provide clues to infectious etiologies arthritis arthritis Slaughterhouse workers, butchers, veterinarians, and farmers may be exposed to HLA-B27 IOL-associated tissues or milk products infected with Brucella Toxocariasis Toxocariasis Herpetic associated uveitis Medical workers are at risk for tuberculosis, herpes simplex, HIV Post-viral Sarcoidosis Sarcoidosis Herpetic Herpetic Current and past residences and recent travel A history of tick bites or traveling in wooded areas, particularly in endemic regions Fuchs’ such as Connecticut or Wisconsin, raise possibility of Lyme disease Kawasaki’s Intraocular Retinoblastoma Toxoplasmosis heterochromic Individuals residing in the southwestern United States, Mexico, or Central and disease lymphoma iridocyclitis South America may be exposed to coccidioidomycosis Leprosy should be considered in immigrants from developing regions JXG Leukemia Behçet’s Sarcoidosis Ocular ischemia Onchocerciasis, or river blindness, is endemic in Africa and Central America Fuchs’ heterochromic iridocyclitis may be more common in patients from countries Leukemia Lyme Syphilis Toxoplasmosis without a rubella vaccination program Evaluate Anterior Segment for: Evaluate Posterior Segment for: Presence of scleritis Quality, quantity, and location of vitreous cells Presence of keratitis Optic nerve edema, hyperemia, pallor, and cupping Presence, distribution, and qualitative characteristics of KP Cystoid macular edema SUN scoring of anterior chamber cell and flare Choroidal neovascularization Anterior and posterior synechiae Presence, size, quality, and location of retinal and choroidal lesions Lens opacity or lens precipitates State of the peripheral retina (with scleral indentation) Vitreous haze score (standardized