- •The Sclera
- •Preface
- •Contents
- •1.1 Introduction
- •1.2 Development of the Sclera
- •1.2.1.1 First Week
- •1.2.1.2 Second Week
- •1.2.1.3 Third Week
- •1.2.1.4 Fourth Week
- •1.2.1.5 Fifth Week
- •1.2.1.6 Sixth Week
- •1.2.1.7 Seventh Week
- •1.2.1.8 Ninth Week
- •1.2.1.9 Tenth Week
- •1.2.1.10 Thirteenth Week
- •1.2.1.11 Sixteenth Week
- •1.2.1.12 Twenty-Fourth Week
- •1.2.2.1 Collagens
- •1.2.2.2 Proteoglycans
- •1.2.2.3 Glycoproteins
- •1.3 Anatomy
- •1.3.1 Gross and Microscopic Anatomy
- •1.3.1.1 Scleral Foramina
- •Anterior Scleral Foramen
- •Posterior Scleral Foramen
- •1.3.1.2 Layers of the Sclera
- •Episclera
- •Scleral Stroma
- •Lamina Fusca
- •1.3.1.3 Blood Supply and Emissary Canals
- •Vascular Distribution
- •Circulatory Dynamics
- •1.3.1.4 Nerve Supply
- •1.3.2 Ultramicroscopic Anatomy
- •1.3.2.1 Sclera
- •1.3.2.2 Vessels
- •1.4 Biochemistry
- •1.5 Immunohistochemistry
- •1.6 Biomechanics
- •1.7 Molecular Structure
- •1.7.1 Collagen
- •1.7.2 Elastin
- •1.7.3 Proteoglycans
- •1.7.4 Glycoproteins
- •1.7.6 Fibroblast Growth Regulation
- •1.8 Summary
- •References
- •2.1 General Immune Response Considerations
- •2.1.1 Components of the Adaptive Immune Response
- •2.1.1.1 Lymphocytes
- •T Lymphocytes
- •B Lymphocytes
- •Third-Population Lymphocytes or Null Lymphocytes
- •2.1.1.2 Monocytes/Macrophages
- •Phagocytosis
- •Antigen-Presenting Cells
- •2.1.1.3 Polymorphonuclear Granulocytes
- •Neutrophils
- •Eosinophils
- •Basophils/Mast Cells
- •2.1.1.4 Platelets
- •2.1.2 Immunoregulation
- •2.1.2.1 Major Histocompatibility Complex
- •2.1.2.2 Humoral Mechanisms: Antibodies
- •2.1.2.3 Cellular Mechanisms
- •2.1.2.4 Summary
- •2.1.3 Abnormalities of the Immune Response
- •2.1.3.1 Hypersensitivity Reactions
- •Type III Hypersensitivity Reactions
- •Systemic Immune Complex Disease
- •Local Immune Complex Disease (Arthus Reaction)
- •Type IV Hypersensitivity Reactions
- •2.1.3.2 Autoimmunity
- •Mechanisms of Autoimmunity
- •2.2 Connective Tissue and the Immune Response
- •2.2.1 Fibroblast Functions and the Immune Response
- •2.3 The Sclera and the Immune Response: Scleritis
- •2.3.1 Immune Characteristics of the Sclera
- •2.3.2 The Susceptible Host: Immunogenetics
- •2.3.3 Etiology
- •2.3.3.1 Exogenous Agents
- •Viruses
- •Mycobacteria
- •2.3.3.2 Endogenous Substances
- •Glycosaminoglycans
- •Collagen
- •2.3.4 Pathogenesis
- •2.4 Summary
- •References
- •3.1 Investigation of the Illness
- •3.1.1 Major Complaint and History of Present Illness
- •3.1.2 Past History
- •3.1.3 Family History
- •3.1.4 Past and Present Therapy History
- •3.1.5 Review of Systems
- •3.1.6 Systemic Examination
- •3.1.6.1 Head
- •3.1.6.2 Extremities
- •3.1.7 Ocular Examination
- •3.1.7.1 Episcleral and Scleral Examination
- •External Examination of the Eye in Daylight
- •Slit-Lamp Examination
- •Diffuse Illumination
- •Slit-Lamp Illumination
- •Red-Free Illumination
- •3.1.7.2 General Eye Examination
- •Visual Acuity
- •Pupils and Extraocular Muscles
- •Cornea
- •Anterior Uvea
- •Lens
- •Fundus
- •Intraocular Pressure
- •3.2 Diagnostic Tests
- •3.2.1 Blood Tests
- •3.2.1.1 Rheumatoid Factor
- •3.2.1.2 Anticyclic Citrullinated Peptide Antibodies
- •3.2.1.3 Antinuclear Antibodies
- •3.2.1.4 Antineutrophil Cytoplasmic Antibodies
- •3.2.1.5 Circulating Immune Complexes
- •Fluid-Phase Binding Assays
- •C1q-Binding Assay
- •Cell-Binding Assays
- •Raji Cell-Binding Assay
- •3.2.1.6 Complement
- •Quantitation Tests
- •Functional Tests
- •3.2.1.7 HLA Typing
- •3.2.1.8 Antibody Titers Against Infectious Organisms
- •3.2.1.9 Interferon-Gamma Release Assays (IGRAs)
- •3.2.2 Anterior Chamber Polymerase Chain Reaction Testing
- •3.2.3 Smears and Cultures
- •3.2.4 Skin Testing
- •3.2.5 Radiologic Studies
- •3.2.6.1 Anterior Segment Fluorescein Angiography Techniques
- •3.2.6.2 Normal Anterior Segment Fluorescein Angiography
- •Arterial Phase
- •Capillary Phase
- •Venous Phase
- •3.2.7 Anterior Segment Indocyanine Green Angiography
- •3.2.8 Other Imaging Studies
- •3.2.8.1 Ultrasonography
- •A-Scan Ultrasonography
- •B-Scan Ultrasonography
- •High-Frequency Ultrasound Biomicroscopy
- •3.2.8.2 Optical Coherence Tomography
- •3.2.8.3 Computer Tomography Scanning
- •3.2.8.4 Magnetic Resonance Imaging
- •3.3 Biopsy
- •3.3.1 Biopsy for Suspected Systemic Vasculitic Disease
- •3.4 Data Integration: Diagnosis
- •3.5 Therapeutic Plan
- •3.6 Summary
- •References
- •4.1 Episcleritis
- •4.1.1 Introduction
- •4.1.2 Patient Characteristics
- •4.1.3 Clinical Manifestations
- •4.1.4.1 Simple Episcleritis
- •4.1.4.2 Nodular Episcleritis
- •4.1.5 Associated Diseases
- •4.1.6 Precipitating Factors
- •4.2 Scleritis
- •4.2.1 Introduction
- •4.2.2 Patient Characteristics
- •4.2.3 Clinical Manifestations
- •4.2.4.1 Diffuse Anterior Scleritis
- •4.2.4.2 Nodular Anterior Scleritis
- •Differential Diagnosis
- •Paralimbic Scleromalacia
- •Senile Scleral Hyaline Plaques
- •4.2.4.5 Posterior Scleritis
- •Symptoms and Signs
- •Fundus Findings
- •Choroidal Folds
- •Subretinal Mass
- •Disk Edema and Macular Edema
- •Annular Ciliochoroidal Detachment and Serous Retinal Detachment
- •Associated Diseases
- •Complications
- •Ancillary Tests
- •Ultrasonography
- •Computerized Tomography (CT) Scanning
- •Fluorescein Angiography
- •Differential Diagnosis
- •Proptosis, Chemosis, Lid Swelling, and Limitation of Ocular Movements
- •Subretinal Mass
- •Choroidal Folds
- •Annular Ciliochoroidal Detachment and/or Serous Retinal Detachment
- •Disk and Macular Edema
- •4.2.5 Associated Diseases
- •4.2.6 Complications of Scleritis
- •4.2.6.1 Keratopathy
- •Peripheral Corneal Thinning
- •Stromal Keratitis
- •Peripheral Ulcerative Keratitis
- •4.2.6.2 Uveitis
- •4.2.6.3 Glaucoma
- •Angle-Closure Glaucoma
- •Open-Angle Glaucoma
- •Neovascular Glaucoma
- •4.2.6.4 Cataract
- •4.3 Summary
- •References
- •5: Pathology in Scleritis
- •5.1.3 Fibrinoid Necrosis
- •5.2.1 Pathology of Episcleritis
- •5.2.2 Pathology of Scleritis
- •5.2.2.1 Noninfectious Scleritis
- •Sclera
- •Cells
- •Extracellular Matrix
- •Vessels
- •Episclera
- •Conjunctiva
- •Iris, Ciliary Body, and Choroid
- •Cornea
- •Other Ocular Structures
- •Polyarteritis Nodosa
- •Allergic Granulomatous Angiitis (Churg–Strauss Syndrome)
- •Granulomatosis with Polyangiitis (Wegener)
- •Connective Tissue Diseases
- •Clinicopathological Correlates in Infectious Scleritis
- •Systemic Infections
- •Local Infections
- •5.3 Biopsy
- •5.3.1 Noninfectious Necrotizing Scleritis
- •5.3.2 Noninfectious Recurrent Diffuse or Nodular (Nonnecrotizing) Scleritis
- •5.3.3 Infectious Scleritis (Diffuse, Nodular, or Necrotizing Scleritis)
- •5.3.4 Biopsy Technique
- •5.4 Summary
- •References
- •6: Noninfectious Scleritis
- •6.1.1 Adult Rheumatoid Arthritis
- •6.1.1.1 Epidemiology
- •Signs and Symptoms of Joint Involvement
- •Extraarticular Systemic Manifestations
- •6.1.1.2 Systemic Manifestations
- •Onset
- •Tegument
- •Vessels
- •Lung
- •Heart
- •Nervous System
- •Lymph Nodes
- •Larynx
- •Felty’s Syndrome
- •Amyloidosis
- •Miscellaneous
- •6.1.1.3 Ocular Manifestations
- •Keratoconjunctivitis Sicca
- •Scleritis
- •Keratitis
- •Anterior Uveitis
- •Glaucoma
- •Cataract
- •Retinal, Choroidal, and Optic Nerve Changes
- •Motility Disturbances
- •Episcleritis
- •6.1.1.4 Laboratory Findings
- •Rheumatoid Factor
- •Antibodies to Cyclic Citrullinated Polypeptides
- •Complete Blood Count
- •Acute-Phase Reactants
- •Synovial Fluid Analysis
- •Circulating Immune Complexes
- •Antinuclear Antibodies
- •Complement
- •Cryoglobulins
- •Radiographic Evaluation
- •Diagnosis
- •6.1.2 Systemic Lupus Erythematosus
- •6.1.2.1 Epidemiology
- •6.1.2.2 Systemic Manifestations
- •Musculoskeletal
- •Tegument
- •Vessels
- •Kidney
- •Hearth
- •Nervous System
- •Lung
- •Miscellaneous
- •6.1.2.3 Ocular Involvement
- •Scleritis
- •Episcleritis
- •Other Ocular Findings
- •6.1.2.4 Laboratory Findings
- •6.1.2.5 Diagnosis
- •6.1.3 Ankylosing Spondylitis
- •6.1.3.1 Epidemiology
- •6.1.3.2 Systemic Manifestations
- •Articular Involvement
- •Extraarticular Systemic Manifestations
- •6.1.3.3 Ocular Manifestations
- •Anterior Uveitis
- •Scleritis
- •Episcleritis
- •6.1.3.5 Diagnosis
- •6.1.4 Reactive Arthritis (Reiter)
- •6.1.4.1 Epidemiology
- •6.1.4.2 Systemic Manifestations
- •Articular Involvement
- •Extraarticular Systemic Manifestations
- •6.1.4.3 Ocular Manifestations
- •Conjunctivitis
- •Anterior Uveitis
- •Scleritis
- •Episcleritis
- •Other Ocular Findings
- •6.1.4.4 Laboratory and Radiographic Findings
- •6.1.4.5 Diagnosis
- •6.1.5 Psoriatic Arthritis
- •6.1.5.1 Epidemiology
- •6.1.5.2 Systemic Manifestations
- •Skin and Articular Involvement
- •6.1.5.3 Ocular Manifestations
- •Scleritis
- •Episcleritis
- •6.1.5.4 Laboratory and Radiographic Findings
- •6.1.5.5 Diagnosis
- •6.1.6.1 Epidemiology
- •6.1.6.2 Systemic Manifestations
- •Gastrointestinal and Articular Manifestations
- •6.1.6.3 Ocular Manifestations
- •Anterior Uveitis
- •Scleritis
- •Episcleritis
- •Keratitis
- •6.1.6.4 Laboratory and Joint Radiologic Findings
- •6.1.6.5 Diagnosis
- •6.1.7 Relapsing Polychondritis
- •6.1.7.1 Epidemiology
- •6.1.7.2 Systemic Manifestations
- •6.1.7.3 Ocular Manifestations
- •Scleritis
- •Episcleritis
- •6.1.7.4 Laboratory Findings
- •6.1.7.5 Diagnosis
- •6.1.8 Polyarteritis Nodosa
- •6.1.8.1 Epidemiology
- •6.1.8.2 Systemic Manifestations
- •6.1.8.3 Ocular Manifestations
- •Scleritis
- •Episcleritis
- •6.1.8.4 Laboratory and Angiographic Findings
- •6.1.8.5 Diagnosis
- •6.1.9.1 Epidemiology
- •6.1.9.2 Systemic Manifestations
- •6.1.9.3 Ocular Manifestations
- •6.1.9.4 Laboratory Findings
- •6.1.9.5 Diagnosis
- •6.1.10 Granulomatosis with Polyangiitis (Wegener)
- •6.1.10.1 Epidemiology
- •6.1.10.2 Clinical Manifestations
- •6.1.10.3 Ocular Manifestations
- •Scleritis
- •Episcleritis
- •6.1.10.4 Laboratory Findings
- •6.1.10.5 Diagnosis
- •6.1.11 Adamantiades–Behçet’s Disease
- •6.1.11.1 Epidemiology
- •6.1.11.2 Systemic Manifestations
- •6.1.11.3 Ocular Manifestations
- •Scleritis
- •Episcleritis
- •6.1.11.4 Laboratory Findings
- •6.1.11.5 Diagnosis
- •6.1.12 Giant-Cell Arteritis
- •6.1.12.1 Epidemiology
- •6.1.12.2 Systemic Manifestations
- •6.1.12.3 Ocular Manifestations
- •Scleritis
- •6.1.12.4 Laboratory Findings
- •6.1.12.5 Diagnosis
- •6.1.13 Cogan’s Syndrome
- •6.1.13.1 Clinical Manifestations
- •Scleritis
- •Episcleritis
- •6.1.13.2 Laboratory Findings
- •6.2.1 Rosacea
- •6.3.1 Gout
- •6.5 Chemical Injury-Associated Scleritis
- •6.6 Summary
- •References
- •7: Infectious Scleritis
- •7.1 Bacterial Scleritis
- •7.1.1.1 Pathogenesis
- •7.1.1.2 Organisms
- •7.1.1.3 Management
- •7.1.1.4 Therapy
- •7.1.1.5 Prognosis
- •7.1.1.6 Our Experience
- •7.1.2 Mycobacterial Scleritis
- •7.1.2.1 Atypical Mycobacterial Disease
- •7.1.2.2 Tuberculosis
- •7.1.2.3 Leprosy
- •7.1.3 Spirochetal Scleritis
- •7.1.3.1 Syphilis
- •Epidemiology
- •Pathogenesis and Clinical Features
- •Scleritis and Episcleritis
- •Diagnosis
- •Therapy
- •7.1.3.2 Lyme Disease
- •Epidemiology
- •Pathogenesis and Clinical Features
- •Scleritis and Episcleritis
- •Diagnosis
- •7.1.3.3 Treatment
- •7.1.4 Chlamydial Scleritis
- •7.1.5 Actinomycetic Scleritis
- •7.1.5.1 Nocardiosis
- •7.2 Fungal Scleritis
- •7.2.1 Filamentous and Dimorphic Fungal Scleritis
- •7.2.1.1 Pathogenesis
- •7.2.1.2 Organisms
- •7.2.1.3 Management
- •7.2.1.4 Therapy
- •7.2.1.5 Our Experience
- •7.3 Viral Scleritis
- •7.3.1 Herpes Scleritis
- •7.3.1.1 Herpes Zoster Scleritis
- •Epidemiology
- •Pathogenesis
- •Clinical Features
- •Scleritis
- •Episcleritis
- •Diagnosis
- •Treatment
- •7.3.1.2 Herpes Simplex Scleritis
- •Epidemiology
- •Pathogenesis
- •Clinical Features
- •Scleritis
- •Episcleritis
- •Diagnosis
- •Therapy
- •Our Experience
- •7.3.2 Mumps Scleritis
- •7.4 Parasitic Scleritis
- •7.4.1 Protozoal Scleritis
- •7.4.1.1 Acanthamoeba
- •7.4.1.2 Toxoplasmosis
- •7.4.2 Helminthic Scleritis
- •7.4.2.1 Toxocariasis
- •7.5 Summary
- •References
- •8.1 Scleral Deposits
- •8.1.1 Scleral Protein Deposition
- •8.1.1.1 Porphyria
- •8.1.1.2 Cystinosis
- •8.1.1.3 Alkaptonuria
- •8.1.1.4 Amyloidosis
- •8.1.2 Scleral Lipid Deposition
- •8.1.2.1 Familial Hypercholesterolemia and Histiocytosis X
- •8.1.2.2 Age-Related Degeneration
- •8.1.3 Scleral Carbohydrate Deposition
- •8.1.3.1 Mucopolysaccharidosis
- •8.1.4 Scleral Mineral Deposition: Calcium
- •8.1.4.1 Hyperparathyroidism
- •8.1.4.2 Other Causes of Hypercalcemia
- •8.1.4.3 Age-Related Degeneration
- •Senile Scleral Hyaline Plaques
- •8.1.5 Scleral Pigment Deposition: Bilirubin
- •8.1.5.1 Jaundice
- •8.2 Scleral Thinning (Blue Sclerae)
- •8.2.1 Scleral Thinning in Inherited or Congenital Diseases
- •8.2.1.1 Marfan’s Syndrome
- •8.2.1.2 Osteogenesis Imperfecta
- •8.2.1.3 Pseudoxanthoma Elasticum
- •8.2.1.4 Ehlers–Danlos Syndrome
- •8.2.1.5 Keratoconus
- •8.2.1.6 Buphthalmos
- •8.2.1.7 Coloboma
- •8.2.1.8 Myopia
- •8.2.2 Scleral Thinning in Acquired Diseases
- •8.2.2.2 Paralimbal Scleromalacia
- •8.3 Scleral Thickening
- •8.3.1 Nanophthalmos
- •8.3.2 Scleropachynsis
- •8.3.3 Phthisis Bulbi
- •8.4 Scleral Tumors
- •8.4.1 Dermoid Choristomas
- •8.4.2 Epithelial Tumors
- •8.4.2.1 Papillomas or Intraepithelial Epitheliomas
- •8.4.2.2 Squamous Cell Carcinoma
- •8.4.3 Dense Connective Tissue Tumors
- •8.4.3.1 Nodular Fasciitis
- •8.4.3.2 Fibroma
- •8.4.3.3 Fibrous Histiocytoma
- •8.4.3.4 Sarcomas
- •8.4.4 Vascular Tumors
- •8.4.4.1 Hemangiomas
- •8.4.4.2 Lymphangiomas
- •8.4.5 Blood Cell Tumors
- •8.4.5.1 Leukemia
- •8.4.5.2 Lymphoma and Lymphosarcoma
- •8.4.6 Nervous Tumors
- •8.4.6.2 Neurilemmoma (Schwannoma)
- •8.4.7 Pigmented Tumors
- •8.4.7.1 Nevus
- •8.4.7.2 Melanocytoma
- •8.4.8 Secondary Tumors
- •8.5 Summary
- •References
- •9.1 Treatment of Episcleritis
- •9.2 Treatment of Scleritis
- •9.2.1 Medical Treatment
- •9.2.1.1 Rheumatoid Arthritis
- •9.2.1.2 Systemic Lupus Erythematosus
- •9.2.1.3 Polyarteritis Nodosa
- •9.2.1.4 Granulomatosis with Polyangiitis (Wegener)
- •9.2.1.5 Relapsing Polychondritis
- •9.2.1.7 Posterior Scleritis
- •9.2.1.8 Infectious Scleritis
- •9.2.2 Ancillary Therapy
- •9.2.3 Drug Management Responsibility
- •9.2.4 Surgical Treatment
- •9.3 Summary
- •References
- •Index
3.1 Investigation of the Illness |
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Fig. 3.8 Characteristic rheumatoid joint disease with the ÒZÓ deformity of wrist and metacarpal joints
Fig. 3.9 Three vascular networks can be distinguished in the anterior segment of the eye: the conjunctival network, the superÞcial episcleral network, and the deep episcleral network
Fig. 3.10 Episcleritis. The edema is localized to the episcleral tissue; the superÞcial episcleral network is displaced forward because of underlying episcleral edema, and the deep episcleral network remains ßat
to distinguish the benign, self-limiting, and frequently symptomless episcleritis from the much more severe, destructive, and often painful scleritis, which can lead to loss of vision and portend serious systemic disease. Early diagnosis may lead to early treatment of the ocular and general condition. Differentiation between the two entities and further characterization of each can be accomplished if the eye is examined methodically and thoroughly, following a strict routine of examination.
3.1.7.1 Episcleral and Scleral Examination
Three vascular networks can be distinguished in the anterior segment of the eye: a conjunctival network, a superÞcial episcleral network, and a deep episcleral network (Fig. 3.9). In episcleritis, maximum congestion is in the superÞcial episcleral vascular network, with no changes in the deep episcleral network. The edema is localized to the episcleral tissue; the superÞcial episcleral network is displaced forward because of underlying episcleral edema, and the deep episcleral network
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3 Diagnostic Approach of Episcleritis and Scleritis |
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Fig. 3.11 Scleritis. The edema is localized to the scleral and episcleral tissue; the deep and superÞcial episcleral networks are displaced forward because of underlying scleral and episcleral edema
Fig. 3.12 External examination in daylight may show an obvious diffuse, grayish blue tinge of the eye with scleritis; the presence of brownish areas indicates a necrotizing process
remains undisturbed, thereby separating the two networks compared to the normal relationship (Fig. 3.10). In scleritis, maximum congestion is in the deep episcleral network, although there is also some congestion in the superÞcial episcleral network. The edema is localized to the scleral and episcleral tissue; the deep episcleral network is displaced forward because of underlying scleral edema and the superÞcial episcleral network is also displaced forward because of underlying episcleral and scleral edema (Fig. 3.11).
External Examination of the Eye in Daylight
External evaluation of the eye in daylight is sometimes helpful in distinguishing episcleritis from scleritis because tungsten and ßuorescent lights do not disclose subtle color differences. In episcleritis, the eye appears bright red. In deep scleritis, the eye has a diffuse, grayish blue tinge; this is because after several attacks of scleral inßammation, the sclera may become more translucent and sometimes thinner, allowing the dark uvea to show through (Fig. 3.2).
A dark brown or black-tinged area surrounded by active scleral inßammation indicates that a necrotic process is taking place (Fig. 3.12). If tissue
Fig. 3.13 Necrotizing scleritis. Note the dark area surrounded by active inßammation, characteristic of a necrotizing process
Fig. 3.14 Necrotizing scleritis. The sequestrum is partially removed
