- •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
Infectious Scleritis |
7 |
|
Although systemic immune-mediated diseases are the main possibilities in the differential diagnosis of scleritis, other unusual etiologies, such as infectious diseases, must also be considered. Infectious scleritis, either endogenous or exogenous, may be caused either by a direct invasion of organisms that cause the systemic and local signs, or by an immune response induced by the infectious agent.
All classes of microbial organisms can infect the sclera, including bacteria, fungi, viruses, and parasites (Table 7.1). As the scleritis caused by these conditions may be identical to that caused by immune-mediated diseases, the challenge for the ophthalmologist is to distinguish infectious scleritis from other inßammatory conditions of the sclera. The differential diagnosis between both groups of diseases is important because infectious etiologies are usually treatable with speciÞc therapy, and because corticosteroid therapy or immunosuppressive therapy, often used in scleritis associated with immune-mediated diseases, is contraindicated in active infection; if topical or systemic corticosteroids are started because it is thought the scleritis has an immunologic basis, scleral destruction, and extension of the microbial process may progress.
In evaluating a patient with scleritis, it is important to take a history not only for evidence of underlying systemic diseases, but also for trauma, contact lens effects, past ocular conditions, topical therapy, and surgical procedures.
There are no speciÞc clinical signs that conÞrm scleral infection. When the diagnosis is suspected, laboratory studies are required to establish the causative agent. Appropriate therapy is initiated on the basis of clinical suspicion, the results of initial laboratory studies, and knowledge of the most likely responsible organisms responsible. The plan may be modiÞed later, depending on the clinical response and laboratory results. With advanced infection or with a severe host inßammatory response, devastating complications can occur, resulting in structural alterations, such as thinning, perforation, or extension to adjacent structures.
In theory, any infectious agent that induces an immune response can cause vasculitis. Bacteria, such as Pseudomonas, Streptococcus, Staphylococcus, or viruses such as herpes simplex virus (HSV) or Varicella-zoster virus (VZV) may be associated with small-sized vessel vasculitis. Syphilis and tuberculosis may cause large-sized vessel vasculitis (aortitis). Vascular damage is commonly incurred by direct invasion or the vessel by the organism, or by embolization, both of which result in an inßammatory response and immune complex formation and deposition.
This chapter focuses on the infectious diseases that may be associated with scleritis. Recommendations for an approach to the management and therapy of infectious scleritis are presented.
M. Sainz de la Maza et al., The Sclera, DOI 10.1007/978-1-4419-6502-8_7, |
241 |
© Springer Science+Business Media, LLC 2012 |
|
