- •Uveitis
- •Foreword
- •Preface
- •Dedication
- •Acknowledgments
- •Elements of the Immune System and Concepts of Intraocular Inflammatory Disease Pathogenesis
- •Elements of the immune system
- •Macrophages/monocytes
- •Dendritic cells
- •T cells
- •Major subsets of T cells
- •Cytokines
- •T-cell subsets
- •T-regulatory cells
- •T-cell receptor
- •Chemokines
- •Thymic expression and central immune tolerance
- •B cells
- •Classes of Immunoglobulin
- •Other cells
- •Mast Cells
- •Eosinophils
- •Neutrophils
- •Resident Ocular Cells
- •Complement system
- •Cellular interactions: hypersensitivity reactions
- •Classic immune hypersensitivity reactions
- •Type I
- •Type II
- •Type III
- •Type IV
- •Type V
- •Concepts of disease pathogenesis
- •Immune characteristics of the eye
- •Absence of lymphatic drainage
- •Intraocular microenvironment
- •Anterior Chamber-Associated Immune Deviation (ACAID)
- •Fas-Fas Ligand Interactions and Programmed Cell Death (Apoptosis)
- •Resident Ocular Cells and Immune System
- •Cytokines and Chemokines and the Eye
- •Oral Tolerance
- •Choroidal circulation and anatomy
- •Retina
- •Immunogenetics
- •Class I antigens
- •Class II and class III antigens
- •Histocompatibility lymphocyte antigens
- •Single-nucleotide polymorphisms (SNPs)
- •Epigenetics
- •Immune complex-mediated disease
- •Gene expression profiling
- •Tissue damage in the eye
- •T-cell responses and autoimmunity
- •T-cell receptor and the expression of disease
- •Ocular autoimmunity
- •Uveitogenic antigens
- •Retinal S-Antigen (Arrestin)
- •Interphotoreceptor Retinoid-Binding Protein
- •Recoverin
- •Bovine Melanin Protein
- •Rhodopsin
- •Phosducin
- •Tyrosinase
- •Other Antigens
- •Endotoxin and Other Bacterial Antigens
- •Importance of Antigen Studies
- •Cell adhesion molecules and their role in lymphocyte homing and in disease
- •Immune responses to invading viruses and parasites
- •Suggested Readings
- •References
- •Medical History in the Patient with Uveitis
- •References
- •Sample Uveitis Questionnaire
- •FAMILY HISTORY
- •SOCIAL HISTORY
- •PERSONAL MEDICAL HISTORY
- •MEDICAL HISTORY
- •Examination of the Patient with Uveitis
- •Visual acuity
- •External examination
- •Pupils and extraocular muscles
- •Intraocular pressure measurement
- •Slit-lamp biomicroscopy
- •Conjunctiva
- •Cornea
- •Keratic Precipitates
- •Other Corneal Findings
- •Anterior chamber
- •Iris
- •Anterior chamber angle
- •Lens
- •Vitreous
- •Retina and choroid
- •Optic nerve
- •References
- •Development of a Differential Diagnosis
- •Forming a differential diagnosis
- •Classifying uveitis
- •Is the disease acute or chronic?
- •Is the inflammation granulomatous or nongranulomatous?
- •Is the disease unilateral or bilateral?
- •Where is the inflammation located in the eye?
- •What are the demographics of the patient?
- •What associated symptoms does the patient have?
- •What associated signs are present on physical examination?
- •What is the time course of the disease and response to previous therapy?
- •Case 4-1
- •Case 4-2
- •References
- •Diagnostic Testing
- •Pretest likelihood of disease
- •Receiver operating characteristic (ROC) curve
- •Diagnostic tests for uveitis
- •Laboratory tests
- •Image analysis
- •Skin testing
- •Tissue samples
- •Ancillary ophthalmic tests
- •Electrophysiology
- •Laser interferometry
- •Fluorescein angiography
- •Indocyanine green
- •Laser flare photometry
- •Optical coherence tomography
- •High-frequency ultrasound biomicroscopy and multifrequency ultrasound
- •Fundus autofluorescence
- •Other diagnostic tests
- •Polymerase chain reaction (PCR)
- •Rapid tests for herpes simplex and herpes zoster
- •Bone mineral density studies
- •Genetic testing for steroid-induced glaucoma
- •Neurologic tests
- •References
- •Evidence-Based Medicine in Uveitis
- •Study design
- •Clinical trials in uveitis
- •References
- •Philosophy, Goals, and Approaches to Medical Therapy
- •Goals and philosophy
- •Pain, photophobia, and discomfort
- •Degree and location of inflammatory disease
- •Evaluation of visual acuity and prospect of reversibility
- •Follow-up procedures and standardization of observations
- •General health and age of patient
- •Patient reliability, preferences, and understanding
- •Nonsurgical therapeutic options
- •Corticosteroids
- •Mode of Action
- •Preparations, Dosage Schedules, and Complications
- •Ozurdex.
- •Secondary Effects
- •Cytotoxic agents
- •Alkylating agents
- •Mode of Action
- •Indications and Dosages
- •Secondary Effects
- •Antimetabolites
- •Azathioprine
- •Mode of Action
- •Indications and Dosages
- •Secondary Effects
- •Mycophenolate mofetil
- •Methotrexate
- •Mode of Action
- •Indications and Dosages
- •Secondary Effects
- •Ciclosporin
- •Mode of Action
- •Dosages and Indications
- •Secondary Effects
- •Tacrolimus
- •Mode of Action
- •Indications and Dosages
- •Secondary Effects
- •Lx 211 (Voclosporin)
- •Rapamycin
- •Mode of Action
- •Indications and Dosages
- •Toxicity
- •Antibodies and monoclonal antibodies
- •Daclizumab
- •Etanercept
- •Infliximab (Remicade)
- •Adalimumab (Humira)
- •Efalizumab (Raptiva)
- •Rituximab (Rituxan)
- •Anakinra (Kineret)
- •Alemtuzumab (Campath-1H)
- •Abatacept (Orencia)
- •Intravenous immunoglobulin therapy
- •Oral tolerance
- •Interferon-α
- •Antiviral therapy
- •Aciclovir
- •Ganciclovir
- •Valaciclovir
- •Famciclovir
- •Foscarnet
- •Combined ganciclovir and foscarnet
- •Cidofovir
- •Fomivirsen
- •Colchicine
- •Mode of Action
- •Indications and Dosages
- •Secondary Effects
- •Mydriatic and cycloplegic agents
- •Antitoxoplasmosis therapy
- •Other therapeutic approaches
- •Immunostimulators
- •Plasmapheresis
- •Nonsteroidal antiinflammatory agents
- •References
- •Role of Surgery in the Patient with Uveitis
- •Considerations
- •Removal of band keratopathy
- •Corneal transplantation
- •Cataract surgery
- •Glaucoma surgery
- •Treatment of vitreoretinal disease
- •Laser treatment
- •Photodynamic therapy
- •Diagnostic surgery
- •Anterior chamber paracentesis
- •Chorioretinal biopsy
- •Subretinal surgery
- •Case 8-1
- •References
- •Bacterial and Fungal Diseases
- •Introduction
- •Leprosy
- •Clinical findings
- •Immunology and pathology
- •Therapy
- •Tuberculosis
- •Systemic disease
- •Ocular disease
- •Diagnosis
- •Therapy
- •Other bacterial infections
- •Brucellosis
- •Whipple’s disease
- •Treatment and prognosis
- •Chronic granulomatous disease
- •Fungal disease
- •Neuroretinitis
- •References
- •Spirochetal Diseases
- •Spirochetal infections and the eye
- •Spirochetes
- •Definition
- •Venereal treponemal diseases
- •Syphilis
- •Etiology and Epidemiology
- •Clinical Manifestations
- •Primary syphilis.
- •Secondary syphilis.
- •Latent syphilis.
- •Tertiary syphilis.
- •Benign tertiary syphilis.
- •Cardiovascular syphilis.
- •Neurosyphilis.
- •Congenital syphilis.
- •Ocular Manifestations
- •Diagnosis
- •Prognosis
- •Treatment
- •General recommendations.
- •Approach to Syphilis in Patients with AIDS
- •Nonvenereal treponematoses
- •Endemic syphilis
- •Etiology and Epidemiology
- •Clinical Manifestations
- •Ocular Manifestations
- •Diagnosis
- •Prognosis
- •Treatment
- •Yaws and pinta
- •Ocular Manifestations
- •Diagnosis
- •Prognosis
- •Treatment
- •Borrelia infection
- •Lyme disease
- •Etiology and Epidemiology
- •Clinical Manifestations
- •Ocular Manifestations
- •Diagnosis
- •Prognosis
- •Treatment
- •Relapsing fever
- •Etiology and Epidemiology
- •Clinical Manifestations
- •Ocular Manifestations
- •Diagnosis
- •Prognosis
- •Treatment
- •Leptospirosis
- •Etiology and Epidemiology
- •Clinical Manifestations
- •Ocular Manifestations
- •Weil’s disease
- •Diagnosis
- •Prognosis
- •Treatment
- •Case 10-1
- •References
- •Acquired Immunodeficiency Syndrome
- •Human immunodeficiency virus
- •Epidemiology
- •Diagnosis
- •HIV disease
- •HIV therapy
- •Ocular manifestations of HIV infection
- •Ocular infection
- •Cytomegalovirus retinitis
- •Progression
- •CMV retinitis in the era of highly active antiretroviral therapy
- •Treatment
- •Intravitreal ganciclovir implant
- •Current therapeutic approach to CMV retinitis in the era of HAART
- •Retinal detachment
- •Prognosis
- •Immune recovery uveitis
- •Herpes zoster
- •Pneumocystis jirovecii choroiditis
- •Mycobacterium avium-intracellulare choroiditis
- •Other diseases
- •Drug-related ocular inflammation
- •Case 11-1
- •Case 11-2
- •References
- •Acute retinal necrosis
- •Epidemiology
- •Clinical features
- •Etiology
- •Differential diagnosis
- •Therapy
- •Progressive outer retinal necrosis
- •Diagnosis
- •Differential diagnosis
- •Etiology
- •Therapy
- •Case 12-1
- •Case 12-2
- •References
- •Other Viral Diseases
- •Herpes simplex virus kerititis and keratouveitis
- •Pathogenesis
- •Diagnosis
- •Treatment
- •Herpes zoster ophthalmicus
- •Treatment
- •West Nile virus
- •Epidemiology
- •Diagnosis
- •Clinical description
- •Ophthalmic manifestations
- •Treatment
- •Prognosis
- •Other viral infections
- •Human T-lymphotropic virus type I
- •Case 13-1
- •References
- •Ocular Toxoplasmosis
- •Organism
- •Clinical manifestations
- •Systemic
- •Ocular
- •Decreased Vision
- •Loss of Vision
- •Effects in immunocompromised host
- •Histopathology and immune factors
- •Immune response
- •Inflammatory response
- •Methods of diagnosis
- •Pregnancy
- •Other methods
- •Congenital versus acquired disease
- •Therapy
- •Additional therapeutic approaches
- •Case 14-1
- •Case 14-2
- •Case 14-3
- •Case 14-4
- •References
- •Ocular Histoplasmosis
- •Systemic findings
- •Ocular appearance
- •‘Histo’ spots
- •Maculopathy
- •Peripapillary pigment changes
- •Clear vitreous
- •Etiology and immunology
- •Nonsurgical therapies
- •Laser therapy
- •Subretinal surgery
- •References
- •Toxocara canis
- •Ocular manifestations
- •Histopathology and immune factors
- •Enzyme-linked immunoabsorbent assay
- •Treatment
- •Case 16-1
- •References
- •Onchocerciasis and Other Parasitic Diseases
- •Onchocerciasis
- •Clinical appearance
- •Immune characteristics
- •Therapy
- •Giardiasis
- •Ophthalmomyiasis
- •Cysticercosis
- •Caterpillar hairs
- •Amebiasis
- •Diffuse unilateral subacute neuroretinitis (DUSN)
- •Malaria
- •Seasonal hyperacute panuveitis (SHAPU)
- •References
- •Postsurgical Uveitis
- •Acute bacterial endophthalmitis
- •Chronic bacterial endophthalmitis
- •Fungal endophthalmitis
- •Endogenous endophthalmitis
- •Lens-induced uveitis
- •Toxic anterior segment syndrome (TASS)
- •Laser-induced uveitis
- •Case 18-1
- •References
- •Anterior Uveitis
- •Epidemiology
- •Clinical description
- •Idiopathic anterior uveitis
- •Diagnostic workup
- •Treatment
- •HLA-B27–associated anterior uveitis
- •Epidemiology
- •Demographics and clinical findings
- •Etiology
- •HLA-B27–associated anterior uveitis with systemic disease
- •Ankylosing spondylitis
- •Etiology
- •Treatment
- •Reactive arthritis (Reiter’s syndrome)
- •Juvenile idiopathic arthritis
- •Diagnosis
- •Pathology
- •Differential diagnosis
- •Treatment and prognosis
- •Psoriatic arthropathy
- •Inflammatory bowel disease
- •Whipple’s disease
- •Disease associations
- •Fuchs’ heterochromic iridocyclitis
- •Etiology
- •Treatment and prognosis
- •Kawasaki disease
- •Tubulointerstitial nephritis and uveitis syndrome (TINU)
- •Pathogenesis
- •Glaucomatous cyclitic crisis
- •Schwartz syndrome
- •Anterior segment ischemia
- •Lens-induced uveitis
- •Anterior uveitis associated with AIDS
- •Other disease associations
- •References
- •Scleritis
- •Episcleritis
- •Scleritis
- •Disease associations
- •Other causes of scleritis
- •Diagnostic testing
- •Pathogenesis
- •Differential diagnosis
- •Treatment
- •References
- •Intermediate Uveitis
- •Epidemiology
- •Clinical manifestations
- •Prognosis
- •Differential diagnosis
- •Multiple sclerosis
- •Etiology
- •Treatment
- •Corticosteroids
- •Immunosuppressive agents
- •Surgery
- •Case 21-1
- •Case 21-2
- •References
- •Sarcoidosis
- •Epidemiology
- •Etiology
- •Clinical manifestations
- •Anterior uveitis
- •Posterior segment findings
- •Systemic involvement
- •Pathology
- •Diagnosis
- •Treatment
- •Case 22-1
- •References
- •Sympathetic Ophthalmia
- •Clinical appearance and prevalence
- •Classic presentation
- •Sequelae
- •Tests and immunologic characteristics
- •Dalen–fuchs nodules
- •Preservation of the choriocapillaris
- •Therapy
- •Corticosteroids
- •Immunosuppressive agents
- •Case 23-1
- •Case 23-2
- •References
- •Vogt–Koyanagi–Harada Syndrome
- •Clinical aspects
- •Systemic findings
- •Ocular findings
- •Course of disease
- •Laboratory tests, etiology, and histopathology
- •Antigen-specific and immune responses
- •Vogt–Koyanagi–Harada syndrome versus sympathetic ophthalmia
- •Therapy
- •Cataract extraction
- •Case 24-1
- •Case 24-2
- •References
- •Birdshot Retinochoroidopathy
- •Clinical manifestations
- •Ocular examination and ancillary clinical tests
- •Tests, histology and etiology
- •Therapy
- •Case 25-1
- •Case 25-2
- •References
- •Behçet’s Disease
- •Clinical manifestations
- •Oral aphthous ulcers
- •Skin lesions
- •Genital ulcers
- •Ocular disease
- •Retinal disease
- •Complications
- •Minor criteria
- •Arthritis
- •Vascular alterations
- •Neurologic involvement (neuro-Behçet’s disease)
- •Immunologic and histologic considerations
- •Role of T cells (but other cells count too!)
- •HLA typing and single nucleotide polymorphisms (SNPs)
- •Therapy
- •Systemic corticosteroids
- •Cytotoxic and antimetabolic agents
- •Colchicine
- •Interferon-α
- •Ciclosporin and tacrolimus (FK506)
- •Anti-TNF therapy (infliximab)
- •Other approaches
- •Case 26-1
- •Case 26-2
- •Case 26-3
- •References
- •Retinal Vasculitis
- •Clinical characteristics
- •Ocular vasculitic disorders without systemic disease
- •Eales’ disease
- •Idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN syndrome)
- •Frosted branch angiitis
- •Scleritis
- •Ocular vasculitic disorders with systemic disease
- •Systemic lupus erythematosus
- •Polyarteritis nodosa
- •Wegener’s granulomatosis
- •Whipple’s disease
- •Inflammatory bowel disease
- •Autoantibodies to Sjögren’s syndrome A antigen
- •Retinal vein occlusion
- •Relapsing polychondritis
- •Viral diseases
- •Multiple sclerosis
- •Tuberculosis
- •Rheumatoid arthritis
- •Kikuchi–Fujimoto disease
- •Susac syndrome
- •Sweet syndrome
- •References
- •Serpiginous Choroidopathy
- •Clinical features
- •Pathology
- •Etiology
- •Differential diagnosis
- •Therapy
- •Case 28-1
- •Case 28-2
- •Case 28-3
- •References
- •White-Dot Syndromes
- •Multiple evanescent white-dot syndrome
- •Clinical findings
- •Laboratory findings
- •Therapy
- •Multifocal choroiditis and panuveitis
- •Clinical findings
- •Punctate inner choroidopathy
- •Laboratory findings
- •Therapy
- •Acute retinal pigment epitheliitis
- •Clinical findings
- •Laboratory findings
- •Therapy
- •Acute posterior multifocal placoid pigment epitheliopathy
- •Clinical findings
- •Etiology
- •Therapy
- •Subretinal fibrosis and uveitis syndrome
- •Clinical findings
- •Laboratory findings
- •Therapy
- •Acute zonal occult outer retinopathy (AZOOR) and the azoor complex diseases
- •Case 29-1
- •Case 29-2
- •Case 29-3
- •References
- •Masquerade Syndromes
- •Intraocular lymphoma
- •Non-Hodgkin’s lymphoma of central nervous system
- •Diagnosis
- •Treatment
- •Systemic Non-Hodgkin’s lymphoma metastatic to eye
- •Lymphoid hyperplasia of uvea
- •Other malignant processes manifesting as uveitis
- •Paraneoplastic syndromes
- •Multiple sclerosis
- •Other nonmalignant conditions
- •References
- •Introduction
- •Age-related macular degeneration
- •Animal work
- •Animal laser model
- •Ccl2 and Ccr2 knockout model
- •Ccl2 and Cx3cr1 double knockout model
- •CEP induced AMD-like disease
- •Human data
- •Autoimmunity
- •Gene associations
- •Macrophages and other cells
- •Histopathology
- •The downregulatory immune environment
- •Should we consider immunotherapy?
- •Diabetic retinopathy
- •Diabetes and the immune process
- •Animal work
- •Human observations
- •Can we begin to think about immune therapy for diabetes and diabetic retinopathy?
- •Glaucoma
- •Autoantibodies and glaucoma
- •Cellular immunity and glaucoma
- •Can immune intervention help alter the course of glaucoma?
- •References
- •Index
Case 22-1
A 25-year-old white woman with sarcoidosis developed floaters and blurred vision in one eye. Examination revealed granulomatous KPs, vitreous cells, fluff balls, and a choroidal granuloma in the posterior pole temporal to the macula. Vision was 20/200, primarily because of macular edema. The patient received a periocular depot steroid injection that resulted in a partial reduction in the inflammation but no visual
References
improvement. Four additional injections given 3 weeks apart resulted in total resolution of the inflammation and an improvement in visual acuity to 20/25. The disease remained quiet for 6 months, at which time the inflammation began to increase and vision dropped to 20/50. One periocular depot steroid injection resulted in resolution of the inflammation and a return of vision to 20/25. The patient has required one or two injections of periocular steroid every 6 months to maintain good vision in the affected eye. The other eye has remained normal.
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