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ESSENTIALS IN OPHTHALMOLOGY: Uveitis and Immunological Disorders.

U. Pleyer · B. Mondino (Eds.)

ESSENTIALS IN OPHTHALMOLOGY

Glaucoma

G. K. Krieglstein · R. N. Weinreb

Cataract and Refractive Surgery

Series Editors

 

 

Uveitis and Immunological Disorders

 

Vitreo-retinal Surgery

 

Medical Retina

 

Oculoplastics and Orbit

 

Paediatric Ophthalmology,

 

Neuro-ophthalmology, Genetics

 

Cornea and External Eye Disease

Editors Uwe Pleyer

Bartly Mondino

Uveitis and

Immunological

Disorders

With 73 Figures, Mostly in Colour,

and 26 Tables

123

Series Editors

Volume Editors

Günther K. Krieglstein, MD

Uwe Pleyer, MD

Professor and Chairman

Professor of Ophthalmology

Department of Ophthalmology

Augenklinik Charité

University of Cologne

Campus Virchow Klinikum

Joseph-Stelzmann-Straße 9

Augustenburger Platz 1

50931 Cologne

13353 Berlin

Germany

Germany

Robert N. Weinreb, MD

Bartly Mondino, MD

Professor and Director

Professor of Ophthalmology

Hamilton Glaucoma Center

Jules Stein Eye Institute

Department of Ophthalmology – 0946

100 Stein Plaza #2–142

University of California at San Diego

Los Angeles, CA 90095-7000

9500 Gilman Drive

USA

La Jolla, CA 92093-0946

 

USA

 

ISBN 3-540-20045-2

ISSN 1612-3212

Springer Verlag Berlin Heidelberg New York

 

Library of Congress Control Number: 2004106762

This work is subject to copyright. All rights are reserved, whether the whole or part of the material is concerned, specifically the rights of translation, reprinting, reuse of illustrations, recitation, broadcasting, reproduction on microfilm or in any other way, and storage in data banks. Duplication of this publication or parts thereof is permitted only under the provisions of the German Copyright Law of September 9, 1965, in its current version, and permission for use must always be obtained from Springer-Ver- lag. Violations are liable for prosecution under the German Copyright Law.

Springer is a part of Springer Science +

Business Media

springeronline.com

© Springer-Verlag Berlin Heidelberg 2005

Printed in Germany

Cover picture “Cataract and Refractive Surgery” from Kampik A, Grehn F (eds) Augenärztliche Therapie. Georg Thieme Verlag Stuttgart, with permission.

The use of general descriptive names, registered names, trademarks, etc. in this publication does not imply, even in the absence of a specific statement, that such names are exempt from the relevant protective laws and regulations and therefore free for general use.

Product liability: The publishers cannot guarantee the accuracy of any information about dosage and application contained in this book. In every individual case the user must check such information by consulting the relevant literature.

Editor: Marion Philipp, Heidelberg

Desk editor: Martina Himberger, Heidelberg Production: ProEdit GmbH, Heidelberg Cover design: Erich Kirchner, Heidelberg Typesetting and reproduction of the figures: AM-productions GmbH, Wiesloch

Printing: Saladruck GmbH, Berlin Binding: Stein & Lehmann, Berlin

Printed on acid-free paper

24/3150beu-göh

5 4 3 2 1 0

Foreword

Essentials in Ophthalmology is a new review series covering all of ophthalmology categorized in eight subspecialties. It will be published quarterly; thus each subspecialty will be reviewed biannually.

Given the multiplicity of medical publications already available, why is a new series needed? Consider that the half-life of medical knowledge is estimated to be around 5 years. Moreover, it can be as long as 8 years between the description of a medical innovation in a peer-reviewed scientific journal and publication in a medical textbook.A series that narrows this time span between journal and textbook would provide a more rapid and efficient transfer of medical knowledge into clinical practice, and enhance care of our patients.

For the series, each subspecialty volume comprises 10–20 chapters selected by two distinguished editors and written by internationally renowned specialists. The selection of these contributions is based more on recent and note-

worthy advances in the subspecialty than on systematic completeness. Each article is structured in a standardized format and length, with citations for additional reading and an appropriate number of illustrations to enhance important points. Since every subspecialty volume is issued in a recurring sequence during the 2-year cycle, the reader has the opportunity to focus on the progress in a particular subspecialty or to be updated on the whole field. The clinical relevance of all material presented will be well established, so application to clinical practice can be made with confidence.

This new series will earn space on the bookshelves of those ophthalmologists who seek to maintain the timeliness and relevance of their clinical practice.

G. K. Krieglstein

R. N. Weinreb

Series Editors

Preface

Essentials in Ophthalmology is a publication series that includes eight volumes covering the following topics: Glaucoma, Cataract and Refractive Surgery, Uveitis and Immunological Disorders,Vitreo-Retinal Surgery, Medical Retina, Ocular Plastics and Orbit, Neuro-Ophthal- mology, Paediatric Ophthalmology, and Cornea and External Eye Disease. A volume on each topic will be published every 2 years with one being published each quarter. Every 2 years, each volume will be repeated in a new and updated version with different topics.

Each volume is designed to bridge the gap between primary research literature and daily practice. Although current and practical information is stressed in each volume, the latest in research information and directions is also highlighted. The series is designed to be user friendly with numerous tables and illustrations. The target audience includes ophthalmologists and optometrists in practice and training as well as libraries.

The third volume in this series is entitled

Uveitis and Immunological Disorders. Our knowledge and understanding of these diseases has increased exponentially over the past decade, especially in the areas of immunopathogenesis and immunogenetics. This volume will provide the practitioner with practical information on how to diagnose and treat these difficult and, in some cases, blinding disorders. In addition,

there are important discussions of mechanisms underlying these conditions that incorporate the most recent, up-to-date research material available. A “Summary for the Clinician” as well as “Core Messages” enhance the value of the chapters. These aids will help the reader to focus on the important messages in each chapter.

The scope of chapters ranges from diseases that are relatively common and usually require only topical therapy such as ocular allergy and dry eye to diseases that may result in blindness without systemic therapy such as ocular cicatricial pemphigoid, scleritis and some forms of uveitis. Two of the chapters, “Immune Mechanisms in Uveitis” and “Uveitis and Genetics”, provide the reader with the latest laboratory research in these areas.

This volume has information of interest to a wide range of ophthalmic subspecialists. For example, the anterior segment subspecialist will find an interest in subjects such as ocular allergy,dry eye,cicatricial pemphigoid,corneal transplantation, scleritis and herpes viruses. Retina and uveitis specialists will have a special interest in the chapters dealing with uveitis and its mechanisms and therapy, including medical and surgical. Lastly, the chapter covering uveitis in children provides important and useful information for paediatric ophthalmologists.

Uwe Pleyer

Bartly Mondino

Contents

Chapter 1

 

Chapter 3

 

Targets in Ocular Allergy

 

Ocular Cicatricial Pemphigoid

 

Alessandra Micera, Sergio Bonini,

 

Ahmed Muna, C. Stephen Foster

 

Alessandro Lambiase, Roberto Sgrulletta,

 

 

 

Stefano Bonini

 

3.1

Introduction . . . . . . . . . . . . . . . . . . . .

25

 

 

 

3.2

Pathophysiology . . . . . . . . . . . . . . . . .

26

1.1

Introduction . . . . . . . . . . . . . . . . . . . .

1

3.3

Epidemiology . . . . . . . . . . . . . . . . . . .

26

1.2

Chronic Allergic Eye Diseases . . . . . .

2

3.4

Clinical History . . . . . . . . . . . . . . . . . .

26

1.3

Effector Cells and Cytokine Release.

2

3.5

Clinical Signs . . . . . . . . . . . . . . . . . . . .

26

1.4

Chemokines

 

3.6

Staging . . . . . . . . . . . . . . . . . . . . . . . . .

27

 

and Adhesion Molecules . . . . . . . . . .

3

3.7

Diagnosis . . . . . . . . . . . . . . . . . . . . . . .

28

1.5

Neuropeptide

 

3.7.1

Laboratory Tests . . . . . . . . . . . . . . . . .

28

 

and Growth Factor Involvement . . . .

4

3.7.2

Conjunctival Biopsy . . . . . . . . . . . . . .

28

1.6

Tissue Remodelling

 

3.7.3

Histology . . . . . . . . . . . . . . . . . . . . . . .

29

 

and the Contribution of Fibroblasts

5

3.8

Treatment . . . . . . . . . . . . . . . . . . . . . .

29

1.7

Metabolism of Extracellular Matrix .

6

3.8.1

Medical . . . . . . . . . . . . . . . . . . . . . . . . .

29

1.8

Conclusions . . . . . . . . . . . . . . . . . . . . .

7

3.8.2

Surgery . . . . . . . . . . . . . . . . . . . . . . . . .

31

References . . . . . . . . . . . . . . . . . . . . . . . . . . . .

8

3.9

Collaborative Care . . . . . . . . . . . . . . .

33

 

 

 

3.10

Prognosis . . . . . . . . . . . . . . . . . . . . . . .

33

 

 

 

References . . . . . . . . . . . . . . . . . . . . . . . . . . . .

34

Chapter 2

Dry Eye: Inflammation

of the Lacrimal Functional Unit

Stephen C. Pflugfelder, Michael E. Stern

2.1 Introduction . . . . . . . . . . . . . . . . . . . . 11

2.1.1 Basics . . . . . . . . . . . . . . . . . . . . . . . . . . 11

2.1.2 Lacrimal Functional Unit . . . . . . . . . 12

2.2Specific Pathologies

of the Lacrimal Functional Unit . . . . 14

2.2.1Dysfunction of the Afferent System . 14

2.2.2Dysfunction of the Efferent System . 15

2.2.3 Glandular Dysfunction . . . . . . . . . . .

15

2.3Lacrimal Keratoconjunctivitis

Inflammation . . . . . . . . . . . . . . . . . . . 16

2.3.1 Corneal Epithelial Disease . . . . . . . . 17

2.3.2 Conjunctival Epithelial Disease . . . . 17

2.3.3 Inflammation . . . . . . . . . . . . . . . . . . . 18

2.4 Diagnosis . . . . . . . . . . . . . . . . . . . . . . . 19

2.5 Therapies . . . . . . . . . . . . . . . . . . . . . . . 21

References . . . . . . . . . . . . . . . . . . . . . . . . . . . . 22

Chapter 4

Immunomodulation

for Corneal Transplantation

Douglas J. Coster, Keryn A. Williams

4.1

Introduction . . . . . . . . . . . . . . . . . . . .

35

4.2Why Has Corneal Transplantation Fallen Behind Solid Organ

Grafting? . . . . . . . . . . . . . . . . . . . . . . . 36

4.3Immunomodulation

for Corneal Transplantation . . . . . . . 36

4.4Actuarial Survival Data

 

on Corneal Transplantation . . . . . . .

37

4.5

Options for Immunomodulation . . .

38

4.6

Corneal Privilege . . . . . . . . . . . . . . . .

38

4.7Mechanisms

of Corneal Allograft Rejection . . . . . 39

XContents

4.8

The Afferent Limb:

 

Chapter 6

 

 

Sensitization to Corneal Alloantigens

39

Clinical Aspects of MALT

 

4.9

The Efferent Limb:

 

Erich Knop, Nadja Knop, Uwe Pleyer

 

 

Histological Correlates . . . . . . . . . . .

40

 

 

 

4.10

Current Approaches

 

6.1

Introduction . . . . . . . . . . . . . . . . . . . .

67

 

to Immunomodulation

 

6.2

Structure and Function of MALT . . .

68

 

for Corneal Transplantation . . . . . . .

41

6.2.1

Structure of MALT . . . . . . . . . . . . . . .

68

4.10.1

Anti-inflammatory Measures . . . . . .

41

6.2.2

Eye-Associated Lymphoid Tissue

 

4.10.2

Atraumatic Microsurgical Technique

41

 

(EALT) . . . . . . . . . . . . . . . . . . . . . . . . .

71

4.10.3

Topical Corticosteroids . . . . . . . . . . .

41

6.2.3

Basic Functions of MALT . . . . . . . . .

74

4.10.4 HLA Matching for Class I

 

6.3

Dry Eye Disease . . . . . . . . . . . . . . . . .

76

 

and Class II Antigens . . . . . . . . . . . . .

41

6.3.1

Introduction . . . . . . . . . . . . . . . . . . . .

75

4.10.5 Systemic Immunosuppression . . . . .

42

6.3.2

Epidemiology, Definition

 

4.10.6 Systemic Corticosteroids . . . . . . . . . .

42

 

and Characteristics of Dry Eye . . . . .

76

4.10.7 Antiproliferative Agents . . . . . . . . . .

43

6.3.3

Novel Therapeutic Approaches

 

4.10.8 Calcineurin Blockers . . . . . . . . . . . . .

43

 

to Dry Eye Disease . . . . . . . . . . . . . . .

80

4.10.9 Combination Therapy . . . . . . . . . . . .

44

6.4

Ocular Allergy . . . . . . . . . . . . . . . . . . .

81

4.10.10Side Effects of Systemic

 

6.4.1

Introduction . . . . . . . . . . . . . . . . . . . .

81

 

Immunosuppression . . . . . . . . . . . . .

44

6.4.2

Epidemiology, Definition

 

4.11

Novel Approaches

 

 

and Characteristics

 

 

to Immunomodulation . . . . . . . . . . .

45

 

of Allergic Eye Disease . . . . . . . . . . .

81

4.11.1

Monoclonal Antibody Fragments . .

45

6.4.3

Course and Therapy Options

 

4.11.2

Gene Therapy . . . . . . . . . . . . . . . . . . .

45

 

in Allergic Ocular Disease . . . . . . . . .

84

4.12

Conclusion . . . . . . . . . . . . . . . . . . . . . .

46

6.5

Keratoplasty . . . . . . . . . . . . . . . . . . . . .

84

References . . . . . . . . . . . . . . . . . . . . . . . . . . . .

46

6.5.1

Introduction . . . . . . . . . . . . . . . . . . . .

84

 

 

 

6.5.2

Immunological Characteristics

 

 

 

 

 

of Keratoplasty . . . . . . . . . . . . . . . . . .

85

Chapter 5

 

References . . . . . . . . . . . . . . . . . . . . . . . . . . . .

87

Scleritis

 

 

 

 

Alisa Kim, Sean Dumars, Samir Shah,

 

 

 

 

Bartly Mondino

 

Chapter 7

 

 

 

 

Immunogenetics

 

5.1

Introduction . . . . . . . . . . . . . . . . . . . .

52

of Ocular Inflammatory Disease

 

5.1.1

Classification . . . . . . . . . . . . . . . . . . . .

52

Ralph D. Levinson

 

5.1.2

Anatomy . . . . . . . . . . . . . . . . . . . . . . .

55

 

 

 

5.1.3

Pathogenesis . . . . . . . . . . . . . . . . . . . .

55

7.1

Introduction . . . . . . . . . . . . . . . . . . . .

91

5.1.4

Histopathology . . . . . . . . . . . . . . . . . .

56

7.2

Human Leukocyte Antigens . . . . . . .

93

5.2

Clinical Presentation . . . . . . . . . . . . .

57

7.2.1

HLA Nomenclature . . . . . . . . . . . . . .

94

5.2.1

History: Ocular Symptoms . . . . . . . .

57

7.2.2

Class I HLA . . . . . . . . . . . . . . . . . . . . .

94

5.2.2

Physical Examination:

 

7.2.3

Class II HLA . . . . . . . . . . . . . . . . . . . .

95

 

Ocular Signs . . . . . . . . . . . . . . . . . . . .

58

7.2.4

HLA Haplotypes

 

5.2.3

Associated Ocular Manifestations . .

60

 

and Linkage Disequilibrium . . . . . . .

95

5.2.4

Systemic Diseases:

 

7.2.5

HLA and Disease Pathogenesis . . . .

95

 

Clinical Evaluation . . . . . . . . . . . . . . .

61

7.3

HLA Associations

 

5.3

Diagnostic Tools . . . . . . . . . . . . . . . . .

62

 

with Ocular Inflammatory Disease .

96

5.4

Medical Management . . . . . . . . . . . . .

63

7.3.1

Class I HLA Associations

 

5.5

Surgical Management . . . . . . . . . . . .

64

 

with Ocular Inflammatory Disease .

96

References . . . . . . . . . . . . . . . . . . . . . . . . . . . .

65

7.3.2

Class II HLA Associations

 

 

 

 

 

with Ocular Inflammatory Disease .

98

 

 

 

 

 

Contents

XI

 

 

 

 

 

 

 

7.3.3

HLA Associations

 

9.9

Prognostic Factors for Uveitis. . .

. . . 131

 

 

with Ocular Infections . . . . . . . . . . . .

100

9.9.1

Gender . . . . . . . . . . . . . . . . . . . . . .

. . . 132

 

7.3.4

HLA Associations

 

9.9.2

Type of Arthritis . . . . . . . . . . . . . .

. . . 132

 

 

with External Ocular Inflammation. 100

9.9.3

Onset of Arthritis . . . . . . . . . . . . .

. . . 132

 

7.3.5

HLA Associations

 

9.9.4

Severity of Uveitis at Onset . . . . .

. . . 132

 

 

with Extraocular Disease . . . . . . . . . .

100

9.10

Correlation of Uveitis

 

 

7.4

Non-HLA Genes Associated

 

 

and Arthritis Activity . . . . . . . . . .

. . . 133

 

 

with Ocular Inflammatory Disease .

101

9.11

Treatment of Uveitis in JCA. . . . .

. . . 133

 

7.5

Other Ocular Diseases . . . . . . . . . . . .

102

9.11.1

Topical Steroid Treatment . . . . . .

. . . 133

 

7.6

Conclusion . . . . . . . . . . . . . . . . . . . . . .

102

9.11.2

Nonsteroidal Anti-inflammatory

 

 

References . . . . . . . . . . . . . . . . . . . . . . . . . . . .

102

 

Drugs (NSAID) . . . . . . . . . . . . . . .

. . . 134

 

 

 

 

9.11.3

Systemic Immunomodulatory

 

 

 

 

 

 

Agents . . . . . . . . . . . . . . . . . . . . . . .

. . . 134

 

Chapter 8

 

9.12

Recommendations

 

 

Immune Mechanisms in Uveitis

 

 

for Ocular Screening in JCA . . . .

. . . 136

 

Ling Chen, Lynn K. Gordon

 

References . . . . . . . . . . . . . . . . . . . . . . . . .

. . . 136

 

8.1

Introduction . . . . . . . . . . . . . . . . . . . .

109

 

 

 

 

8.2

Animal Uveitis Models . . . . . . . . . . .

110

Chapter 10

 

 

8.2.1

Experimental Autoimmune

 

Herpes Viruses in Ocular Inflammation

 

 

Uveitis (EAU). . . . . . . . . . . . . . . . . . . .

110

Bahram Bodaghi, Phuc LeHoang

 

 

8.2.2

Endotoxin-Induced Uveitis (EIU) . .

116

 

 

 

 

8.2.3

Experimental Melanin-Protein-

 

10.1

Introduction . . . . . . . . . . . . . . . . .

. . . 141

 

 

Induced Uveitis (EMIU) . . . . . . . . . .

116

10.2

Fundamental Virology . . . . . . . . .

. . . 143

 

8.2.4

Experimental Autoimmune Pigment

 

10.3

Epidemiology . . . . . . . . . . . . . . . .

. . . 143

 

 

Epithelial Membrane Protein-

 

10.4

Pathophysiology . . . . . . . . . . . . . .

. . . 144

 

 

Induced Uveitis (EAPU). . . . . . . . . . .

117

10.4.1

Experimental Viral Uveitis . . . . .

. . . 144

 

8.3

Immune Mechanisms

 

10.4.2

Experimental Models

 

 

 

in Human Uveitis . . . . . . . . . . . . . . . .

117

 

to Study Viral Retinitis . . . . . . . .

. . . 145

 

8.3.1

HLA-Associated Human Uveitis . . .

117

10.5

Clinical Features . . . . . . . . . . . . . .

. . . 145

 

8.3.2

Behçet’s Disease . . . . . . . . . . . . . . . . .

119

10.5.1

Anterior Uveitis . . . . . . . . . . . . . .

. . . 145

 

8.3.3

Vogt-Koyanagi-Harada Syndrome . .

120

10.5.2

Viral Retinopathies . . . . . . . . . . .

. . . 147

 

References . . . . . . . . . . . . . . . . . . . . . . . . . . . .

121

10.5.3

Differential Diagnosis . . . . . . . . .

. . . 149

 

 

 

 

10.5.4

Putative Viral-Associated Uveitis

. . . 150

 

 

 

 

10.6

Diagnosis . . . . . . . . . . . . . . . . . . . .

. . . 150

 

Chapter 9

 

10.6.1

Obtention of Ocular Fluids . . . . .

. . . 151

 

The Tip of the Iceberg: Current Knowledge

 

10.6.2

Herpes Viruses . . . . . . . . . . . . . . .

. . . 151

 

of Uveitis in Juvenile Arthritis

 

10.6.3 Search for Antiviral Antibodies .

. . . 152

 

Uwe Pleyer, Claudia Sengler,

 

10.7

Treatment . . . . . . . . . . . . . . . . . . .

. . . 153

 

Natasa Orlic, Rolf Keitzer

 

10.7.1

Anterior Uveitis . . . . . . . . . . . . . .

. . . 153

 

 

 

 

10.7.2

Viral Retinopathies . . . . . . . . . . .

. . . 154

 

9.1

Introduction . . . . . . . . . . . . . . . . . . . .

125

References . . . . . . . . . . . . . . . . . . . . . . . . .

. . . 157

 

9.2

Classification of Arthritis . . . . . . . . .

126

 

 

 

 

9.3

Epidemiology . . . . . . . . . . . . . . . . . . .

126

 

 

 

 

9.4

Pathogenesis . . . . . . . . . . . . . . . . . . . .

127

Chapter 11

 

 

9.5

Risk Factors for the Development

 

Cytomegalovirus and the Eye

 

 

 

of Uveitis in JRA . . . . . . . . . . . . . . . . .

128

Marc D. de Smet

 

 

9.6

Clinical Features of Uveitis in JCA . .

128

 

 

 

 

9.7

Complications of Uveitis in JCA . . . .

129

11.1

Introduction . . . . . . . . . . . . . . . . .

. . . 161

 

9.7.1

Macula Edema . . . . . . . . . . . . . . . . . .

130

11.2

Populations at Risk . . . . . . . . . . .

. . . 161

 

9.8

Differential Diagnosis . . . . . . . . . . . .

130

 

 

 

 

XII Contents

11.2.1

HIV Associated

 

 

Immunosuppression . . . . . . . . . . . . .

161

11.2.2

Immunosuppression

 

 

in Non-HIV Settings. . . . . . . . . . . . . .

162

11.3

Appearance and Course . . . . . . . . . . .

163

11.3.1

Manifestation Prior

 

 

to Initial Treatment . . . . . . . . . . . . . .

163

11.3.2

Manifestations Under Therapy . . . . .

164

11.3.3

Differential Diagnosis . . . . . . . . . . . .

165

11.3.4

Investigations . . . . . . . . . . . . . . . . . . .

166

11.4

Therapeutic Agents . . . . . . . . . . . . . .

166

11.4.1

Ganciclovir. . . . . . . . . . . . . . . . . . . . . .

166

11.4.2

Valganciclovir . . . . . . . . . . . . . . . . . . .

167

11.4.3

Foscarnet . . . . . . . . . . . . . . . . . . . . . . .

167

11.4.4

Other Agents . . . . . . . . . . . . . . . . . . . .

167

11.5Screening and Treatment

Algorithms . . . . . . . . . . . . . . . . . . . . . 167

11.5.1 CMV in HIV Infected Patients . . . . . 168

11.5.2 CMV in Other Immunosuppressive

States . . . . . . . . . . . . . . . . . . . . . . . . . . 168

11.6 Management of Complications . . . . . 168

11.6.1 Immune Recovery . . . . . . . . . . . . . . . 168

11.6.2 Retinal Detachment . . . . . . . . . . . . . . 168

References . . . . . . . . . . . . . . . . . . . . . . . . . . . . 169

Chapter 12

Behçet’s Disease

Manfred Zierhut, Nicole Stübiger,

Christoph Deuter, Ina Kötter

12.1

Introduction . . . . . . . . . . . . . . . . . . . .

173

12.2

Definition and Epidemiology of BD

174

12.2.1

Definition . . . . . . . . . . . . . . . . . . . . . . .

174

12.2.2

History . . . . . . . . . . . . . . . . . . . . . . . . .

174

12.2.3

Epidemiology . . . . . . . . . . . . . . . . . . .

174

12.3

Genetics of BD . . . . . . . . . . . . . . . . . .

178

12.4

Immunology . . . . . . . . . . . . . . . . . . . .

182

12.4.1

T Cells . . . . . . . . . . . . . . . . . . . . . . . . .

182

12.4.2

NK Cells . . . . . . . . . . . . . . . . . . . . . . . .

183

12.4.3

Neutrophils . . . . . . . . . . . . . . . . . . . . .

183

12.4.4

Endothelial Dysfunction . . . . . . . . . .

183

12.4.5

Coagulation and Fibrinolytic

 

 

Pathway Abnormalities . . . . . . . . . . .

184

12.5General (Extraocular)

Manifestations of BD . . . . . . . . . . . . . 184

12.5.1 Oral Aphthous Ulcerations . . . . . . . . 184

12.5.2 Genital Ulcerations . . . . . . . . . . . . . . . 185

12.5.3 Skin Lesions . . . . . . . . . . . . . . . . . . . . . 185

12.5.4 Pathergy Phenomenon . . . . . . . . . . . 185

12.5.5 Skeletal System . . . . . . . . . . . . . . . . . . 185 12.5.6 Gastrointestinal Manifestations . . . . 186 12.5.7 Neurological Manifestations . . . . . . . 186 12.5.8 Vascular Manifestations . . . . . . . . . . . 186 12.5.9 Rare Manifestations . . . . . . . . . . . . . . 187 12.5.10Life Expectancy . . . . . . . . . . . . . . . . . . 187 12.6 Ocular Involvement . . . . . . . . . . . . . . 187 12.6.1 Anterior Segment Changes . . . . . . . . 188 12.6.2 Posterior Segment Changes . . . . . . . 188 12.6.3 Complications . . . . . . . . . . . . . . . . . . . 189 12.6.4 Diagnosis . . . . . . . . . . . . . . . . . . . . . . . 190 12.7 Therapy . . . . . . . . . . . . . . . . . . . . . . . . 190 12.7.1 Local Treatment . . . . . . . . . . . . . . . . . 191 12.7.2 Systemic Treatment. . . . . . . . . . . . . . . 191 12.7.3 Surgical Treatment . . . . . . . . . . . . . . . 194 12.7.4 Practical Treatment

Recommendations . . . . . . . . . . . . . . . 194 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . 195

Chapter 13

Choroiditis:

General Considerations and Classification

Carl P. Herbort

13.1 Introduction . . . . . . . . . . . . . . . . . . . . 202

13.2Indocyanine Green Angiography

(ICGA) . . . . . . . . . . . . . . . . . . . . . . . . . 202 13.2.1 Physicochemical Properties

of Indocyanine Green (ICG) . . . . . . . 202

13.2.2Standard ICG Angiographic Protocol for Inflammatory

Diseases . . . . . . . . . . . . . . . . . . . . . . . . 204 13.2.3 Principles for the Interpretation

of ICGA [9, 10] . . . . . . . . . . . . . . . . . . . 204

13.2.4Differences Between Fluorescein and Indocyanine Green

Angiography . . . . . . . . . . . . . . . . . . . . 205

13.2.5 Clinico-pathologic-angiographic

Correlations . . . . . . . . . . . . . . . . . . . . 205

13.2.6 Relevance of ICGA in Ocular

Inflammatory Diseases . . . . . . . . . . . 205

13.3The Concepts of Inflammatory

Choriocapillaropathy

and Stromal Choroiditis [12] . . . . . . . 206

13.3.1 Primary Inflammatory

Choriocapillaropathy (PICCP) . . . . . 206

13.3.2 Stromal Choroiditis . . . . . . . . . . . . . . 207

13.3.3 Secondary Inflammatory

Choriocapillaropathy . . . . . . . . . . . . . 207

References . . . . . . . . . . . . . . . . . . . . . . . . . . . . 207