Ocular Inflammatory
Diseases
Alexandre A.C.M.Ventura, MDa, Brandy C. Hayden, BSb,
MehranTaban, MDb, CareenY. Lowder, MD, PhDb,*
KEYWORDS
Uveitis Panuveitis Scleritis Toxocariasis
Orbital inflammatory disease
Ocular inflammatory diseases are an important group of entities that affect the eye and the adnexa. The incidence of ocular inflammatory diseases in the United States ranges from 11 to 38 per 100,000 persons per year. These diseases are responsible for up to 15% of all cases of blindness.1–3 Inflammation can be of infectious or noninfectious origin and can be localized or diffuse, involving the uvea, sclera, or retina, separately or simultaneously.4
Ultrasonography, optical coherence tomography, CT, and MRI are imaging modalities important in the diagnosis of inflammatory eye diseases. Optical coherence tomography, now in its fourth generation, has been available since 1991 and can process high-resolution cross-sectional imaging of ocular structures.5
Because of its ability to evaluate eyes using either clear or opaque media, ultrasonography has been a critical adjunct in ophthalmology for more than 50 years. This article provides an overview of the ultrasound characteristics observed in a variety of ocular inflammatory diseases.
UVEITIS
According to the Standardization of Uveitis Nomenclature Working Group6,7, the anatomic classification of uveitis is based on the site of inflammation as anterior uveitis, intermediate uveitis, posterior uveitis, or panuveitis (Table 1). Uveitis can be acute or chronic, with sudden or insidious onset, and infectious or noninfectious (Table 2).6
Anterior Uveitis
Anterior uveitis is the most common form of ophthalmic inflammation. The anterior chamber is the
primary site of inflammation and can be associated with involvement of the cornea (keratouveitis), iris (iritis), retrolental space and ciliary body (iridocyclitis), and the adjacent sclera (sclerouveitis). In the United States 91% of all cases of uveitis are anterior uveitis.8–14
Causes of anterior uveitis are numerous and include ankylosing spondylitis, Reiter’s syndrome, psoriatic arthritis, inflammatory bowel disease, juvenile rheumatoid arthritis–associated uveitis, herpetic uveitis, sarcoidosis, Fuchs’ heterochromic iridocyclitis, postsurgical inflammation, syphilis, tuberculosis, and Lyme disease, among others. The most common unilateral alternating acute anterior uveitis is associated with the HLAB27 antigen. Fifty percent of patients presenting to a uveitis specialist with severe pain, redness, and photophobia are HLA-B27 positive, and half of these patients are found to have a systemic disease. African Americans have more severe disease and may present with a hypopyon.8,9,12,14,15 The clinical features include unilateral or bilateral redness, pain, photophobia, excessive tearing, and blurring of vision associated with keratic precipitates and anterior chamber cells. However, patients who have juvenile rheumatoid arthritis associated uveitis often present with a quiet eye despite severe anterior chamber inflammation.
Ultrasound biomicroscopy (UBM) can be a valuable aid in the assessment of anterior uveitis pathologies (see the article by Pavlin and colleagues, elsewhere in this issue). Although direct visualization of iritis and iridocylitis usually is sufficient to provide a diagnosis, adjuvant use of UBM can aid or confirm the diagnosis and may lead to
aHospital de Olhos Santa Luzia & Santa Luzia Foundation Recife-PE, Brazil
bCole Eye Institute, Cleveland Clinic, 9500 Euclid Ave i-32, Cleveland, OH 44195, USA * Corresponding author.
E-mail address: lowderc@ccf.org (C.Y. Lowder).
Ultrasound Clin 3 (2008) 245–255 doi:10.1016/j.cult.2008.04.009
1556-858X/08/$ – see front matter ª 2008 Published by Elsevier Inc.
ultrasound.theclinics.com
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Table 1 |
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The Standardization of Uveitis NomenclatureWorking Group anatomic classification of uveitis |
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Type |
Primary Site of Inflammationa |
Clinical Manifestation |
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Anterior uveitis |
Anterior chamber |
Iritis |
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Iridocyclitis |
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Anterior cyclitis |
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Intermediate uveitis |
Vitreous |
Pars planitis |
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Posterior cyclitis |
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Hyalitis |
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Posterior uveitis |
Retina or choroid |
Focal, multifocal, or diffuse choroiditis |
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Chorioretinitis |
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Retinochoroiditis |
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Retinitis |
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Neuroretinitis |
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Panuveitis |
Anterior chamber, vitreous, |
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and retina or choroid |
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a As determined clinically. (Adapted from the International Uveitis Study Group anatomic classification. Bloch-Michel E, Nussenblatt RB. International Uveitis Study Group recommendations for the evaluation of intraocular inflammatory disease. Am J Ophthalmol 1987;103:234–5.)
From Jabs DA, Nussenblatt, RB, Rosenbaum JT. Standardization of uveitis nomenclature for reporting clinical data. Results of the First International Workshop. Am J Ophthalmol 2005;140:510; with permission.
modifications in the treatment plan (Fig. 1). UBM evaluation of the iris has proven an effective aid in the diagnosis of iritis, iridocylitis, and parasitic uveitis.16,17
Intermediate Uveitis
Intermediate uveitis refers to inflammation in the vitreous and may be associated with peripheral vascular sheathing and pars plana snowballs and snowbanking. Anterior chamber involvement may be present but is usually mild. Most cases are
bilateral but asymmetric in onset and severity. Intermediate uveitis is seen in patients who have multiple sclerosis, sarcoidosis, Lyme disease, and syphilis. The most common symptom is floaters. Patients have little discomfort and a quiet eye. Decreased vision usually is caused by cystoid macular edema or cataract. Direct visualization of the structures involved in intermediate uveitis may be limited in the presence of inadequate pupillary dilation or opaque media. UBM provides a reliable method of documenting associated
pathology.8,10,14,18–25
Table 2
Common causes of inflammatory ocular diseases
Type |
Causes |
Infectious |
Toxoplasmosis, syphilis, tuberculosis, herpes simplex, varicella |
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zoster, ocular leprosy, presumed ocular histoplasmosis |
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syndrome, candidiasis, toxocariasis, cysticercosis, diffuse |
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unilateral subacute neuroretinitis, HIV, ophthalmomyiasis, |
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ascariasis, rubella, schistosomiasis, ophthalmia nodosa, |
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pneumocystosis, endophthalmitis |
Noninfectious (autoimmune) |
Seronegative spondyloarthropathies, systemic lupus |
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erythematosus, giant cell arteritis, Adamantiades-Behc¸et |
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disease, Wegener’s granulomatosis, Fuchs’ heterochromic |
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iridocyclitis, sarcoidosis, multiple sclerosis, birdshot |
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chorioretinopathy, Vogt-Koyanagi-Harada syndrome, multiple |
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evanescent white dot syndrome, acute posterior multifocal |
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placoid pigment epitheliopathy, acute zonal occult outer |
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retinopathy, serpiginous choroiditis |
Fig. 1. Iritis and iridocyclitis. UBM demonstrating marked, irregular thickening of the iris (arrow) and adjacent ciliary body (arrowhead).
Pars Planitis
In the absence of a systemic disease, intermediate uveitis is known as ‘‘pars planitis.’’18,19 The incidence in adults may range from 4% to 15% of all cases of uveitis in referral centers and accounts for up to 25% in the pediatric popula- tion.8,10,14,18–21,26 In clear media, indirect ophthalmoscopy or slit-lamp biomicroscopy examination reveals inflammatory cells in the anterior vitreous and aggregates of inflammatory cells (snowballs) and white exudates (snowbanks) over the pars plana. Patients frequently develop cataract as a result of either uncontrolled inflammation or the corticosteroids used to treat the condition. In these cases, UBM imaging allows the evaluation of the pars plana to identify thickening and overlying hyperreflective inflammatory aggregates (snowbanks) (Fig. 2).25 The absence of these condensations can help differentiate Behcet’s uveitis from pars planitis when the clinical presentation of Behcet’s disease is mild.27 In intermediate
Fig. 2. Pars planitis. UBM demonstrating white exudates (arrow) over thickened pars plana.
Ocular Inflammatory Diseases |
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uveitis and in Behcet’s disease, retinal neovascularization can occur and can lead to vitreous hemorrhage. B-scan ultrasonography is needed to assess the retina in the presence of moderate or dense vitreous hemorrhage.
Hypotony and Uveitis
Under various conditions, hypotony can accompany uveitis. UBM is a valuable tool in determining underlying pathophysiology by showing the presence of ciliary body cysts, cyclitic membranes, ciliochoroidal effusion (Fig. 3), or atrophy of the ciliary processes (Fig. 4), information that can aid tremendously in management and follow-up.26,28–30 Hypotony in the presence of ciliary processes requires aggressive anti-inflammatory or immunosuppressive treatment in patients who have noninfectious uveitis.
Posterior Uveitis
Posterior uveitis is ocular inflammation that involves the choroid, the retina, or both. In the United States, 5% to 38% of all cases of uveitis are posterior uveitis.31–33 In contrast to anterior uveitis, which in most cases has a noninfectious cause, many posterior uveitis syndromes are the result of an infection. Infectious posterior uveitis syndromes include syphilis, toxoplasmosis, toxocariasis, herpetic necrotizing retinitis (as in acute retinal necrosis or progressive outer retinal necrosis), cytomegalovirus retinitis, ocular histoplasmosis syndrome, candidiasis, cysticercosis, and diffuse unilateral subacute neuroretinitis. The incidence and type vary depending on the region, climate, and population.8,14,34,35 Noninfectious posterior uveitis syndromes include sarcoidosis, Behc¸ et’s disease, and white dot syndromes, such as multifocal choroiditis, birdshot chorioretinopathy, multiple evanescent white dot syndrome, and acute posterior multifocal placoid pigment epitheliopathy.
The signs and symptoms of posterior uveitis depend on the location of inflammation and are worse when the lesions affect the posterior pole. Patients may complain of floaters, metamorphopsia, scotomas, and markedly decreased vision. The anterior segment may be quiet, although some anterior chamber reaction may be present. Vitreous inflammation may range from mild to severe in areas overlying the inflammatory foci. Direct fundus examination usually is sufficient in the evaluation of mild or moderate uveitis. In cases of severe posterior uveitis, however, diffuse inflammatory infiltrates in the vitreous often preclude evaluation of the posterior segment of the eye. In these cases, contact B-scan is the preferred