Scleritis

Key Concepts Scleritis is a painful and potentially sight-threatening inflammation of the sclera and is associated with immune or infectious causes. Treatment often requires systemic anti-inflammatory therapy, including corticosteroids and immunosuppressive drugs. Biologic agents, including the anti–tumor necrosis factor (TNF)-α medications, may be useful in treating scleritis. Episcleritis is an inflammatory disorder of the episclera and is often mild in severity, rarely impairs vision, and frequently…

Anterior Uveitis

Key Concepts Anterior uveitis is the most common form of uveitis, with an annual incidence of 8:100,000 population, and is the least likely to be referred to a uveitis specialist. Although anterior uveitis is usually the most easily managed form, associated complications, such as glaucoma, may result in severe visual loss. Many disorders that can cause panuveitis, such as sarcoidosis, Behçet disease, bacterial endophthalmitis, and ocular…

Postsurgical Uveitis

Key Concepts The causes of postoperative inflammation are divided into those occurring acutely and those occurring after 6 weeks. Bacterial infections are the most common, but fungal endophthalmitis may also occur sometime after surgery. Lens-induced uveitis may occur spontaneously in an eye with a hypermature lens or after ocular trauma or cataract surgery. Postoperative noninfectious inflammation that persists longer than 3 months occurs in a small…

Onchocerciasis and Other Parasitic Diseases

Key Concepts Onchocerciasis, or river blindness, is a disease that affects 17.7 million people worldwide, and some 123 million people live in regions where onchocerciasis is endemic; ivermectin is usually the drug of choice, but use of it has not eradicated the disease. Worms of two sizes have been described in eyes with diffuse unilateral subacute neuroretinitis (DUSN). The number of parasitic disorders affecting the eye…

Ocular Toxocariasis

Key Concepts Toxocariasis is a zoonotic infection caused by the roundworms Toxocara canis and Toxocara cati. Although ocular toxocariasis is relatively uncommon, it mostly affects young children and frequently causes permanent vision loss, including blindness. The diagnosis is predominantly based on clinical findings and exposure to puppies or kittens. Enzyme-linked immunosorbent assay (ELISA) is the most reliable and readily available test for the evaluation of antibodies…

Ocular Histoplasmosis

Key Concepts Presumed ocular histoplasmosis syndrome (POHS) is characterized by a triad of peripapillary pigment changes, white round chorioretinal macular and midperipheral scars, and absence of vitritis. Amphotericin therapy does not help. Lesions have a high possibility of choroidal neovascularization. Laser therapy and anti–vascular endothelial growth factor (VEGF) therapies have been used to treat neovascularization. Subretinal surgical removal of neovascular lesions is of minimal value. In…

Ocular Toxoplasmosis

Key Concepts Ocular disease can occur with both congenital and acquired diseases. Disease is often unilateral, and the majority of recurrences are seen as satellite lesions. Immunodeficient patients are at risk for acquired disease, reactivation of old disease, and bilateral and multifocal disease. Pregnancy and cataract surgery may be associated with increased risk for recurrence. Toxoplasmosis is a common disease in both mammals and birds. The…

Other Viral Diseases

Key Concepts Herpes simplex virus (HSV) is a common cause of inflammatory eye disease. Keratitis and anterior uveitis are frequent manifestations of HSV disease; secondary glaucoma is an important cause of vision loss. A number of randomized controlled trials have helped guide therapy for HSV-associated ocular disease. Topical antiviral therapy is indicated for HSV keratitis. Although oral acyclovir did not improve outcomes in patients with active…

Acute Retinal Necrosis and Progressive Outer Retinal Necrosis

Key Concepts Despite effective antiviral therapies, herpes infection of the eye can lead to severe uveitis with substantial vision loss. Because acute retinal necrosis (ARN) can often begin as anterior uveitis, examination of the peripheral retina is important in all patients with a new onset of anterior inflammation. Testing of the aqueous or vitreous humor with polymerase chain reaction (PCR) testing is useful in confirming the…

Acquired Immunodeficiency Syndrome

Key Concepts Despite efforts at disease prevention, in 2017, the annual number of diagnoses of human immunodeficiency virus (HIV) infection in the United States was 11.8 per 100,000 population, and HIV infection remains a global epidemic. Vasculopathy with cotton-wool spots and retinal hemorrhage is the most common manifestation of HIV infection. HIV infection can be treated with combination antiretroviral therapy (cART), also known as highly active…

Spirochetal Diseases

Key Concepts Spirochetal diseases that cause uveitis include a gamut of historical and clinical infections, including syphilis, endemic syphilis, Lyme disease, relapsing fever, and leptospirosis. Syphilis remains a common and frequently missed, but treatable, cause of uveitis. Ocular findings can occur in primary, secondary, latent, and tertiary syphilis. The Venereal Disease Research Laboratory (VDRL) test can yield false–negative results, especially in the later stages of the…

Bacterial and Fungal Diseases

Key Concepts Infection remains a common cause of uveitis. Better diagnostic tests are helping to identify specific bacteria as the cause of a number of uveitic conditions, such as bartonellosis as the cause of cat scratch disease. Prompt diagnosis is critical so that appropriate anti-infective therapy can be started. Clinicians should know the sensitivity and specificity of the diagnostic tests ordered for the infectious causes of…

Role of Surgery in the Patient with Uveitis

Key Concepts Inflammatory disease needs to be clinically quiescent for at least 3 months before elective surgery is considered. Adequate immunosuppressive therapy perioperatively is very important. Surgery needs to be considered when there is something to save, not when the disease is at the end stage. Despite even optimal medical therapy, structural damage will develop in many eyes with chronic inflammation, which can be repaired only…

Approaches to Medical Therapy

Key Concepts Therapy is guided by the etiology of the inflammation (infectious versus noninfectious), severity, and anatomic location. Corticosteroid therapy can be used to quiet acute inflammation, but steroid-sparing therapies must be added if systemic corticosteroids cannot be tapered successfully by 3 months. It is important to establish goals of therapy and consider further workup or altering therapy if goals of therapy are not achieved. Patients…

Evidence-Based Medicine in Uveitis

Key Concepts All published literature does not have equal import. All medical literature, including this book, must be read critically. Whenever possible, treatment decisions should be based on evidence-based medicine. The practice of evidence-based medicine is defined as the conscientious, explicit, and judicious use of current best evidence in making decisions about the care of individual patients. The best evidence usually comes from well-conducted, randomized clinical…

Optical Coherence Tomography in Uveitis

Key Concepts Optical coherence tomography (OCT) using low-coherence, long-wavelength light can produce high-resolution images of all layers of the retina as well as the choroid and vitreous. Two types of OCT devices are most commonly used in ophthalmology today: spectral-domain OCT (SD-OCT) and swept-source OCT (SS-OCT). Current SS-OCT devices allow better imaging of deeper structures like the choroid. OCT is frequently used to detect, document, and…

Diagnostic Testing and Imaging

Key concepts Mistakes in ordering and interpreting diagnostic tests can lead to misdiagnosis and inappropriate therapy. Diagnostic tests, including laboratory tests and imaging studies, should be ordered to narrow down the differential diagnosis. Clinicians must know the sensitivity and specificity of the diagnostic test to avoid misinterpretation of the results. A few diagnostic tests are both highly sensitive and highly specific and may therefore be useful…

Development of a Differential Diagnosis

Key Concepts Diagnosis of uveitis is often challenging, and developing an erudite differential diagnosis is the key to success. Classification of uveitis is the first step and is guided by a series of questions that can be answered from both the medical history and clinical examination. Classification of uveitis and development of a list of potential diagnoses will help determine appropriate diagnostic testing, guide therapy, and…

Clinical Examination of the Patient with Uveitis

Key Concepts A thorough clinical ophthalmic examination is critical for both diagnosis and assessment of response to therapy. Use of standardized grading scales for assessing intraocular inflammation can improve patient management. Standard grading scales are available for assessment of anterior chamber cells and flare and vitreous cells and haze. A detailed examination of the peripheral retina can reveal pars plana exudates, signs of retinal vasculitis, Delen-Fuchs…