® P R A C T I C I N G O P H T H A L M O L O G I S T S C U R R I C U L U M 2 0 1 4 - 2 0 1 6 Uveitis *** Uveitis 2 © AAO 2014 - 2016 Practicing Ophthalmologists Curriculum Disclaimer and Limitation of Liability As a service to its members and American Board of Ophthalmology (ABO) diplomates, the American Academy of Ophthalmology has developed the Practicing Ophthalmologists Curriculum (POC) as a tool for members to prepare for the Maintenance of Certification (MOC) -related examinations. The Academy provides this material for educational purposes only. The POC should not be deemed inclusive of all proper methods of care or exclusive of other methods of care reasonably directed at obtaining the best results. The physician must make the ultimate judgment about the propriety of the care of a particular patient in light of all the circumstances presented by that patient. The Academy specifically disclaims any and all liability for injury or other damages of any kind, from negligence or otherwise, for any and all claims that may arise out of the use of any information contained herein. References to certain drugs, instruments, and other products in the POC are made for illustrative purposes only and are not intended to constitute an endorsement of such. Such material may include information on applications that are not considered community standard, that reflect indications not included in approved FDA labeling, or that are approved for use only in restricted research settings. The FDA has stated that it is the responsibility of the physician to determine the FDA status of each drug or device he or she wishes to use, and to use them with appropriate patient consent in compliance with applicable law. The Practicing Ophthalmologists Curriculum is intended to be the basis for MOC examinations in 2014, 2015 and 2016. However, the Academy specifically disclaims any and all liability for any damages of any kind, for any and all claims that may arise out of the use of any information contained herein for the purposes of preparing for the examinations for MOC. THE AMERICAN ACADEMY OF OPHTHALMOLOGY DOES NOT WARRANT OR GUARANTEE THAT USE OF THESE MATERIALS WILL LEAD TO ANY PARTICULAR RESULT FOR INDIVIDUALS TAKING THE MOC EXAMINATIONS. THE AMERICAN ACADEMY OF OPHTHALMOLOGY DISCLAIMS ALL DAMAGES, DIRECT, INDIRECT OR CONSEQUENTIAL RELATED TO THE POC. Any questions or concerns related to the relevance and validity of questions on the MOC examinations should be directed to the American Board of Ophthalmology. COPYRIGHT © 2014 AMERICAN ACADEMY OF OPHTHALMOLOGY ALL RIGHTS RESERVED Uveitis 3 © AAO 2014 - 2016 Practicing Ophthalmologists Curriculum Authors and Financial Disclosures The Practicing Ophthalmologists Curriculum was developed by a group of dedicated ophthalmologists reflecting a diversity of background, training, practice type and geographic distribution. Jeffrey A. Nerad, M.D., American Academy of Ophthalmology Secretary for Knowledge Base Development, serves as the overall project director for the acquisition and review of the topic outlines. The Academy gratefully acknowledges the contributions of the American Uveitis Society. Practicing Ophthalmologists Curriculum Panel
 Ramana S. Moorthy, M.D., Chair Russell W. Read, M.D., Ph.D., Vice Chair Andrea D Birnbaum, M.D. Grace A Levy-Clarke, M.D. P. Kumar Rao, M.D. Jennifer E. Thorne, M.D Financial Disclosures The Academy’s Board of Trustees has determined that a financial relationship should not restrict expert scientific clinical or non-clinical presentation or publication, provided appropriate disclosure of such relationship is made. All contributors to Academy educational activities must disclose significant financial relationships (defined below) to the Academy annually. Contributors who have disclosed financial relationships: Grace A Levy-Clarke, M.D. Employee: Vistakon Johnson & Johnson Visioncare, Inc. P. Kumar Rao, M.D. Equity Owner: Pfizer, Inc. Grant Support: National Eye Institute, Regeneron Pharmaceuticals, Inc. Russell W. Read, M.D. Consultant/Advisor: Allergan, Eleven Biotherapeutics, Santen, Inc. Grant Support: Eyegate Pharmaceuticals, Inc., EyeSight Foundation of Alabama, International Retinal Research Foundation, Matthews Family Foundation, Research to Prevent Blindness Jennifer E. Thorne, M.D. Consultant/Advisor: Abbott Medical Optics Inc., Allergan, Gilead Sciences, Xoma Grant Support: Allergan, National Eye Institute, Research to Prevent Blindness Contributors who state they have no significant financial relationships to disclose: Andrea D Birnbaum, M.D Ramana S. Moorthy, M.D. Jeffrey A. Nerad, M.D. Uveitis 4 © AAO 2014 - 2016 Background on Maintenance of Certification (MOC) Developed according to standards established by the American Board of Medical Specialties (ABMS), the umbrella organization of 24 medical specialty boards, Maintenance of Certification (MOC) is designed as a series of requirements for practicing ophthalmologists to complete over a 10-year period. MOC is currently open to all Board Certified ophthalmologists on a voluntary basis; time-limited certificate holders (ophthalmologists who were Board Certified after July 1, 1992) are required to participate in this process. All medical specialties participate in a similar process. The roles of the American Board of Ophthalmology (ABO) and the American Academy of Ophthalmology relative to MOC follow their respective missions. The mission of the American Board of Ophthalmology is to serve the public by improving the quality of ophthalmic practice through a process of certification and maintenance of certification that fosters excellence and encourages continual learning. The mission of the American Academy of Ophthalmology is to advance the lifelong learning and professional interests of ophthalmologists to ensure that the public can obtain the best possible eye care. The role of the ABO in the MOC process is to evaluate and to certify. The role of the Academy in this process is to provide resources and to educate. Background on the Practicing Ophthalmologists Curriculum (POC) At the request of the ABO, the Academy developed the Practicing Ophthalmologists Curriculum (POC), a knowledge base that identifies and defines areas of knowledge important to the delivery of quality eye care as a basis for the content of examinations for the MOC process. The content in the POC is comprised of the information deemed as the most relevant clinical information for a practicing ophthalmologist. The ABO has agreed that their Periodic Ophthalmic Review Test (PORT) and closed-book Demonstration of Ophthalmic Cognitive Knowledge (DOCK) examinations will be based on the POC. The ABO is solely responsible for creating the PORT and DOCK exams and for certifying MOC candidates. The Academy has developed study tools based on the POC to assist doctors preparing to meet these MOC requirements. Organization of the POC The Practicing Ophthalmologists Curriculum comprises 10 practice emphasis areas (PEA), plus Core Ophthalmic Knowledge. The ABO has designated the following as practice emphasis areas: • Comprehensive Ophthalmology • Cataract/Anterior Segment • Cornea/External Disease • Glaucoma • Neuro-Ophthalmology and Orbit • Oculoplastics and Orbit • Pediatric Ophthalmology/Strabismus • Refractive Management/Intervention • Retina/Vitreous • Uveitis Uveitis 5 © AAO 2014 - 2016 In addition to two practice emphasis areas of choice, every diplomate sitting for the DOCK examination will be tested on Core Ophthalmic Knowledge. Core Ophthalmic Knowledge is defined as the fundamental knowledge every practicing ophthalmologist must have whatever their area of practice. Each PEA is categorized into topics presented in an outline format for easier reading and understanding of the relevant information points by the reader. These outlines are based on a standard clinical diagnosis and treatment approach found in the Academy’s Preferred Practice Patterns. For each topic, there are Additional Resources that may contain journal citations and reference to textbooks. These resources are supplemental to the topic outline, and should not be necessary for MOC exam preparation purposes. Creation of the POC The POC was developed by panels of practicing ophthalmologists in each of the ten practice emphasis areas. The panels reflect a diversity of background, training, practice type and geographic distribution, with more than 90 percent of the panel members being time-limited certificate holders. The panels ranked clinical topics (diseases and procedures) in terms of clinical relevance to the subspecialist or comprehensive ophthalmologist. The panelists created outlines for the topics deemed Most Relevant, based on what an ophthalmologist in a specific practice emphasis area needs to know to provide competent, quality eye care (i.e., directly related to patient care). These outlines were reviewed by subspecialty societies and the American Board of Ophthalmology. Revision Process The POC is intended to be revised every three years. The POC panels will consider new evidence in the peer-reviewed literature, as well as input from the subspecialty societies, the American Board of Ophthalmology and the Academy’s Self-Assessment Committee, in revising and updating the POC. Prior to a scheduled review the POC may be changed only under the following circumstances: • A Level I (highest level of scientific evidence) randomized controlled trial indicates a major new therapeutic strategy • The FDA issues a drug/device warning • Industry issues a warning Uveitis 6 © AAO 2014 - 2016 Uveitis Basic concepts in immunology Basic concepts in immunology 1. Basic concepts in immunology: effector cells and the innate immune response ......................... 11 Diagnostic Tests/Procedures Diagnostic Tests/Procedures 2. Lab testing in uveitis ...................................................................................................................... 15 3. Optical coherence tomography (OCT) - in uveitis .......................................................................... 31 4. Posterior segment angiography: fluorescein angiography ............................................................ 36 5. Posterior segment angiography: indocyanine green angiography (ICG) ....................................... 38 6. Conjunctival biopsy/pathology ...................................................................................................... 41 7. Diagnostic vitreoretinal procedures in uveitis: vitreous biopsy .................................................... 44 8. Diagnostic vitreoretinal procedures in uveitis: chorioretinal biopsy ............................................. 47 Non infectious (Autoimmune) and other Uveitis Anterior Uveitis 9. Acute anterior uveitis ..................................................................................................................... 51 10. Lens-associated uveitis ................................................................................................................ 56 11. Persistent (chronic) iridocyclitis ................................................................................................... 60 12. Juvenile idiopathic arthritis .......................................................................................................... 65 13. Fuchs heterochromic iridocyclitis ................................................................................................ 71 Intermediate Uveitis 14. Intermediate uveitis, including pars planitis ................................................................................ 74 Posterior Uveitis 15. Acute posterior multifocal placoid pigment epitheliopathy ........................................................ 80 16. Birdshot retinochoroidopathy ..................................................................................................... 83 Uveitis 7 © AAO 2014 - 2016 17. Multiple evanescent white dot syndrome (MEWDS) .................................................................. 87 18. Multifocal choroiditis with panuveitis ......................................................................................... 90 19. Serpiginous choroidopathy .......................................................................................................... 93 20. Systemic vasculitis - intraocular manifestations .......................................................................... 98 Panuveitis 21. Sarcoidosis panuveitis .................................................................................................................. 106 22. Sympathetic ophthalmia .............................................................................................................. 111 23. Vogt-Koyanagi-Harada disease .................................................................................................... 115 24. Behçet disease ............................................................................................................................. 120 Scleritis-Non infectious 25. Scleritis associated with systemic autoimmune diseases ............................................................ 124 Infectious Uveitis Viral Uveitis 26. Herpetic anterior uveitis .............................................................................................................. 131 27. Necrotizing herpetic retinitis: acute retinal necrosis and progressive outer retinal necrosis ..... 137 28. Cytomegalovirus retinitis ............................................................................................................. 141 29. Other rare infections: Human T-cell lymphotrophic virus ........................................................... 147 Fungal uveitis 30. Ocular histoplasmosis syndrome ................................................................................................. 151 Protozoal Uveitis 31. Toxoplasmic retinochoroiditis...................................................................................................... 155 Helminthic Uveitis 32. Toxocariasis posterior uveitis ...................................................................................................... 162 33. Other infectious uveitis: cysticercosis .......................................................................................... 166 34. Diffuse unilateral subacute neuroretinitis (DUSN) ...................................................................... 168 Uveitis 8 © AAO 2014 - 2016 Bacterial Uveitis 35. Delayed-onset pseudophakic uveitis ........................................................................................... 172 36. Syphilitic panuveitis ..................................................................................................................... 175 37. Lyme disease ................................................................................................................................ 182 38. Leptospiral uveitis ........................................................................................................................ 187 39. Tuberculous uveitis ...................................................................................................................... 191 40. Ocular bartonellosis ..................................................................................................................... 199 41. Brucellar uveitis............................................................................................................................ 203 Scleritis-Infectious 42. Post-surgical and traumatic infectious scleritis ........................................................................... 207 Endophthalmitis Endophthalmitis 43. Candida and Aspergillus endophthalmitis ................................................................................... 211 Masquerade Syndromes 44. Masquerade syndromes .............................................................................................................. 217 45. Cancer-associated retinopathy (CAR) and melanoma-associated retinopathy (MAR)................ 229 Acquired Immune Deficiency Syndrome related disease Acquired Immune Deficiency Syndrome related disease 46. Ocular manifestations of acquired immunodeficiency syndrome ............................................... 233 Medical Therapy Medical Therapy 47. Treatment guidelines for non-infectious ocular inflammatory disease ...................................... 248 48. Cycloplegics .................................................................................................................................. 252 49. Nonsteroidal anti-inflammatory drugs ........................................................................................ 254 50. Corticosteroids ............................................................................................................................. 258 Uveitis 9 © AAO 2014 - 2016 51. Methotrexate ............................................................................................................................... 264 52. Azathioprine ................................................................................................................................. 269 53. Mycophenolate ............................................................................................................................ 273 54. Cyclophosphamide and chlorambucil .......................................................................................... 277 55. Cyclosporine ................................................................................................................................. 282 56. Biologic response modifiers ......................................................................................................... 287 57. Intravitreal antiviral therapy ........................................................................................................ 293 Complications of uveitis and their management Complications of uveitis and their management 58. Cataract extraction for uveitis patients ....................................................................................... 296 59. Glaucoma surgery for uveitis patients ......................................................................................... 301 60. Complications of uveitis ............................................................................................................... 306 Uveitis 10 © AAO 2014 - 2016