2014-2015 Basic and Clinical Science Course (BCSC): Section 9: Intraocular Inflammation and Uvetis

385 Pages·2014·152.857 MB·English

by  Ramana S. Moorthy

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Visual Acuity Conversion Chart
Snellen Fraction
Decimal LogMAR
4-Meter Notation Visual Angle (Minimum Angle
Feet Meters Standard (Visus) Minute of Arc of Resolution)
20/10 6/3 4/2 2.00 0.50 -0.30
20/15 6/4.5 4/3 1.33 0.75 -0.12
20/20 6/6 4/4 1.00 1.00 0.00
20/25 6/7.5 4/5 0.80 1.25 0.10
20/30 6/9 4/6 0.67 1.50 0.18
20/40 6/12 4/8 0.50 2.00 0.30
20/50 6/15 4/10 0.40 2.50 0.40
20/60 6/18 4/12 0.33 3.00 0.48
20/80 6/24 4/16 0.25 4.00 0.60
20/100 6/30 4/20 0.20 5.00 0.70
20/120 6/36 4/24 0.17 6.00 0.78
20/150 6/45 4/30 0.13 7.50 0.88
20/200 6/60 4/40 0.10 10.00 1.00
20/400 6/120 4/80 0.05 20.00 1.30
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For discussion of this chart, see BCSC Section 3, Clinical Optics.
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lntraocular
Inflammation
and Uveitis
Section 9
2014-2015
(Last major revision 2011-2012)
l'.J�
AMERICAN ACADEMY"
\!!I
OF OPHTHALMOLOGY
The Eye M.D. Association
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The American Academy of Ophthalmology is accredited by the Accreditation Coun
cil for Continuing Medical Education to provide continuing medical education for
physicians.
The American Academy of Ophthalmology designates this enduring material for a
maximum of 10 AMA PRA Category 1 Credits™. Physicians should claim only the
credit commensurate with the extent of their participation in the activity.
Originally released June 20ll; reviewed for currency September 7, 2013; CME expira
tion date: June l, 2015. AMA PRA Category 1 Credits™ may be claimed only once dur
ing this period.
The BCSC is designed to increase the physician's ophthalmic knowledge through study
and review. Users of this activity are encouraged to read the text and then answer the
study questions provided at the back of the book.
To claim AMA PRA Category 1 Credits TM upon completion of this activity, learners must
demonstrate appropriate knowledge and participation in the activity by taking the post
test for Section 9 and achieving a score of 80% or higher. For further details, please see
the instructions for requesting CME credit at the back of the book.
The Academy provides this material for educational purposes only. It is not intended
to represent the only or best method or procedure in every case, nor to replace a physi
cian's own judgment or give specific advice for case management. Including all indica
tions, contraindications, side effects, and alternative agents for each drug or treatment
is beyond the scope of this material. All information and recommendations should be
verified, prior to use, with current information included in the manufacturers' package
inserts or other independent sources, and considered in light of the patient's condition
and history. Reference to certain drugs, instruments, and other products in this course
is made for illustrative purposes only and is not intended to constitute an endorsement
of such. Some material may include information on applications that are not considered
community standard, that reflect indications not included irl approved FDA labeling, or
that are approved for use only in restricted research settings. The FDA has stated that
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Cover image courtesy of E. Mitchel Opremcak, MD.
MIX
Paper from
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Copyright © 2014
American Academy of Ophthalmology
All rights reserved
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Basic and Clinical Science Course
Louis B. Cantor, MD, Indianapolis, Indiana, Senior Secretary for Clinical
Education
Christopher J. Rapuano, MD, Philadelphia, Pennsylvania, Secretary for
Ophthalmic Knowledge
George A. Cioffi, MD, New York, New York, BCSC Course Chair
Section 9
Faculty
Ramana S. Moorthy, MD, Chair, Indianapolis, Indiana
P. Kumar Rao, MD, St Louis, Missouri
Russell W Read, MD, PhD, Birmingham, Alabama
Russell N. Van Gelder, MD, PhD, Seattle, Washington
Albert T. Vitale, MD, Salt Lake City, Utah
Bahram Bodaghi, MD, PhD, Consultant, Paris, France
Carrie M. Parrish, MD, Nashville, Tennessee
Practicing Ophthalmologists Advisory Committee for Education
The Academy wishes to acknowledge Mary Lou Jackson, MD, Vision Rehabilitation Com
mittee, for her review of this edition.
The Academy wishes to acknowledge the American Uveitis Society for recommending
faculty members to the BCSC Section 9 committee.
Financial Disclosures
Academy staff members who contributed to the development of this product state that
within the past 12 months, they have had no financial interest in or other relationship with
any entity discussed in this course that produces, markets, resells, or distributes ophthal
mic health care goods or services consumed by or used in patients, or with any competing
commercial product or service.
The authors state the following financial relationships:
Dr Bodaghi: Allergan, consultant; Bausch & Lomb Surgical, consultant; Lux Biosciences,
grant recipient; Novartis Pharmaceuticals, grant recipient
Dr Rao: National Eye Institute, grant recipient; Genentech, grant recipient
Dr Read: Alcon Laboratories, consultant; EyeSight Foundation of Alabama, grant recipi
ent; GlaxoSmithKline, consultant; International Retinal Research Foundation, grant re
cipient; Research to Prevent Blindness, grant recipient; Lux Biosciences, consultant
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D. yan Gelder: Alcon Laboratories, consultant, grant recipient; Novartis Pharmaceuti-
ca1 1 s, consultant; Photoswitch Therapeutics, grant recipient
Dr yitale: Bausch & Lomb Surgical, consultant; Aciont, consultant
The other authors state that they have no significant financial interest or other relation -
ship with the manufacturer of any commercial product discussed in the chapters that
they contributed to this course or with the manufacturer of any competing commercial
product.
Recent Past Faculty
Janet Davis, MD
C. Stephen Foster, MD
Careen Yen Lowder, MD, PhD
Nalini S. Bora, PhD
In addition, the Academy gratefully acknowledges the contributions of numerous past
faculty and advisory committee members who have played an important role in the devel
opment of previous editions of the Basic and Clinical Science Course.
American Academy of Ophthalmology Staff
Richard A. Zorab, Vice President, Ophthalmic Knowledge
Hal Straus, Director, Publications Department
Christine Arturo, Acquisitions Manager
Stephanie Tanaka, Publications Manager
D. Jean Ray, Production Manager
Ann McGuire, Medical Editor
Crissa M. Williams, Administrative Coordinator
~~ AMERICAN ACADEMY•
~ OF OPHTHALMOLOGY
The Eye M.D. Association
655 Beach Street
Box 7424
San Francisco, CA 94120-7424
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Contents
General Introduction .xi
Objectives . . .1
Introduction . .3
PART I Ocular Immunology. . .. ... ... . 5
Introduction to Ocular Immunology and
Immune-Mediated Eye Disease . . . . . . . . . . . 7
1 Basic Concepts in Immunology:
Effector Cells and the Innate Immune Response . 9
Definitions. . . . . . . . . . . . . 9
Components of the Immune System. . . . 9
Leukocytes . . . . . . . . . . . . . 9
Overview of the Innate Immune System . 12
Immunity Versus Inflammation . . . 13
Triggers of Innate Immunity . . . . . . 14
Bacteria-Derived Molecules That Trigger Innate Immunity . 14
Other Triggers or Modulators of Innate Immunity . . . 17
Innate Mechanisms for the Recruitment and Activation
ofNeutrophils . . . . . . . . . . . . . . . . . 17
Innate Mechanisms for the Recruitment and Activation
of Macrophages . . . . . . . . . . . . . 20
Mediator Systems That Amplify Immune Responses . 23
Plasma-Derived Enzyme Systems 23
Lipid Mediators. . . . . . . 25
Cytokines . . . . . . . . . 27
Reactive Oxygen Intermediates 30
Reactive Nitrogen Products. . 31
Neutrophil-Derived Granule Products . 31
2 Immunization and Adaptive Immunity:
The Immune Response Arc and Immune Effectors . 33
Overview of the Immune Response Arc 33
Phases of the Immune Response Arc 36
Afferent Phase . 36
Processing Phase 37
Effector Phase . 42
V
vi • Contents
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Immune Response Arc and Primary or Secondary Immune Response 43
Concept of Immunologic Memory. . . . . . . 43
Effector Reactivities of Adaptive Immunity. . . . . 44
Antibody-Mediated Immune Effector Responses 45
Lymphocyte-Mediated Effector Responses . . . 48
Combined Antibody and Cellular Effector Mechanisms 51
3 Ocular Immune Responses. . . . . . . . . . . 57
Regional Immunity and Immunologic Microenvironments . 57
Regional Immunity . . . . . . . . . . . . . 57
Immune Responses of the Conjunctiva . . . . . . 57
Features of the Immunologic Microenvironment 57
Immunoregulatory Systems. . . . . . . . . . 59
Immune Responses of the Anterior Chamber, Anterior Uvea, and Vitreous . 59
Features of the Immunologic Microenvironment 59
Immunoregulatory Systems. . . . . . . . . . 62
Immune Responses of the Cornea. . . . . . . . . 63
Features of the Immunologic Micro environment 63
Immunoregulatory Systems. . . . . . . . . . 64
Immune Responses of the Retina, RPE, Choriocapillaris, and Choroid . 64
Features of the Immunologic Microenvironment 64
Immunoregulatory Systems. . . . . . . . . 66
4 Special Topics in Ocular Immunology 69
Animal Models of Human Uveitis. . . 69
Experimental Autoimmune Uveitis 69
Experimental Immune Uveitis 69
Equine Recurrent Uveitis. 69
AIRE-Deficient Mice . . . . 69
HLA Associations and Disease . . 70
Normal Function ofHLA Molecules. 70
Allelic Variation. . . 70
Disease Associations. . . . . . . . 72
PART II lntraocular Inflammation and Uveitis 75
5
Clinical Approach to Uveitis . 77
Classification of Uveitis . 78
Anterior Uveitis. 79
Intermediate Uveitis . 79
Posterior Uveitis 82
Panuveitis 82
Categorization by Clinical Course . 83
Symptoms of Uveitis 84
Signs of Uveitis . 85
Anterior Segment . 85
Intermediate Segment . 89
Posterior Segment. 89
Contents • vii
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Review of the Patient's Health and Other Associated Factors 90
Differential Diagnosis of Uveitic Entities . 91
Epidemiology of Uveitis . •. . . . . 91
Laboratory and Medical Evaluation . 93
Therapy ............ . 98
Medical Management of Uveitis. . . 98
Mydriatic and Cycloplegic Agents . 99
N onsteroidal Anti-Inflammatory Drugs 99
Corticosteroids . . . . . . . . . . 100
Immunomodulatory Medications . . 106
Surgical Management of Uveitis . . . . 116
6 Noninfectious (Autoimmune) Ocular
Inflammatory Disease . . . . . 117
Noninfectious Scleritis . . . . . . . . . 117
Treatment of Noninfectious Scleritis . . 117
Anterior Uveitis . . . . . . . . . . . . 118
Acute Nongranulomatous Iritis and Iridocyclitis. . 119
Chronic Anterior Uveitis (Iridocyclitis) . 128
Intermediate Uveitis. . . 134
Pars Planitis . . . . 135
Multiple Sclerosis . . 139
Posterior Uveitis . . . . 140
Collagen Vascular Diseases . . 140
Susac Syndrome. . . . . . . 146
Inflammatory Chorioretinopathies of Unknown Etiology. . 147
Panuveitis . . . . . . . . . . 172
Sarcoidosis . . . . . . . . . . . . 172
Sympathetic Ophthalmia . . . . . . 178
Vogt-Koyanagi-Harada Syndrome. . 183
Beh<;:et Disease . . . . . . . . . . 190
7 Infectious Ocular Inflammatory Disease. 197
Viral Uveitis . . . . . . . 197
Herpesviridae Family . . . . . . . . 197
Rubella ............ . . 209
Lymphocytic Choriomeningitis Virus . 211
Measles (Rubeola). . 212
West Nile Virus ......... . . 214
Rift Valley Fever . . . . . . . . . . 217
Human T-Cell Lymphotropic Virus Type 1 . 218
Dengue Fever. . . . . 219
Chikungunya Fever . . 220
Other Viral Diseases. . 220
Fungal Uveitis . . . . . . 221
Ocular Histoplasmosis Syndrome . . 221
Protozoal Uveitis . . 226
Toxoplasmosis . . . . . . . . . . 226
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Helminthic Uveitis . 235
Toxocariasis . . 235
Cysticercosis . . 237
Diffuse Unilateral Subacute Neuroretinitis . 239
Onchocerciasis . 241
Bacterial Uveitis . . 242
Syphilis ... . 242
Lyme Disease . . 250
Leptospirosis . . 254
Ocular Nocardiosis . 256
Tuberculosis . . . . 256
Ocular Bartonellosis . . 262
Whipple Disease . 265
Infectious Scleritis . . 266
Etiology .... . 266
Clinical Features . 266
Diagnostic Workup . 266
Treatment . 267
Prognosis .... . 267
8 Endophthalmitis 269
Chronic Postoperative Endophthalmitis . 269
Endogenous Endophthalmitis . . . . . 271
Endogenous Bacterial Endophthalmitis . 271
Endogenous Fungal Endophthalmitis . 273
9 Masquerade Syndromes . . . . 281
Neoplastic Masquerade Syndromes . . . . 281
Primary Central Nervous System Lymphoma . . 281
Neoplastic Masquerade Syndromes Secondary
to Systemic Lymphoma . . . . . . . . . . 284
Neoplastic Masquerade Syndromes Secondary to Leukemia. . 285
Neoplastic Masquerade Syndromes Secondary
to Uveal Lymphoid Proliferations . . 285
Nonlymphoid Malignancies . . . . . . . . . 285
Metastatic Tumors. . . . . . . . . . . . . . 286
Bilateral Diffuse Uveal Melanocytic Proliferation . 287
Nonneoplastic Masquerade Syndromes . 287
Retinitis Pigmentosa. . . . . . . . . . . . . . 287
Ocular Ischemic Syndrome . . . . . . . . . . . 287
Chronic Peripheral Rhegmatogenous Retinal Detachment . 288
Intraocular Foreign Bodies . . . 288
Pigment Dispersion Syndrome . 289
Other Syndromes . . . . . . . 289