Anatomic Classification of Uveitis



Anatomic Classification of Uveitis


Wendy M. Smith

Lisa J. Faia

Sunir J. Garg

H. Nida Sen



The uvea—made up of the iris, ciliary body, and choroid—is a pigmented, vascular structure of the eye. These anatomic components can be used to divide the uveal tract into anterior (iris and ciliary body), intermediate (ciliary body and pars plana), and posterior (choroid) locations. Inflammation of the uveal tract, or uveitis, may also involve the retina and the retinal vasculature.


CLASSIFICATION

In 2004, the First International Workshop on Standardization of Uveitis Nomenclature (SUN) endorsed an anatomic classification of uveitis based on the International Uveitis Study Group criteria.

Before these working groups, there were several grading systems in use. Standardization of classification criteria, inflammation grading schema, and outcomes allows comparisons of clinical research from different centers. The use of these criteria confers several advantages, including better definition of the clinical course of disease and more efficient evaluation of new therapies. The SUN classification contains several important features.

• The type of uveitis is determined by the predominant site(s) of uveal inflammation.



  • The anatomic localizations are anterior, intermediate, posterior, and panuveitis (Table 2-1).


  • Identifying the predominant site of inflammation also helps narrow the differential diagnosis (Table 2-2). Significant inflammation of the anterior chamber and vitreous is not panuveitis. (These should be classified as anterior and intermediate uveitis, respectively.)

• Anatomic classification is not influenced by the presence of structural complications.



  • For example, the presence of macular edema or optic disc edema alone is not enough to classify an eye as “posterior uveitis.” Macular edema due to anterior chamber inflammation would be correctly categorized as anterior uveitis.


  • Vitritis plus peripheral vascular sheathing or macular edema is defined as intermediate uveitis because this is the predominant site of inflammation.

• Pars planitis is a specific, idiopathic disease entity defined by the presence of snowball or

snowbank formation in the absence of an associated infection or systemic disease; otherwise, the correct term is intermediate uveitis. The SUN working group criteria currently have a few limitations:








TABLE 2-1. SUN Working Group Anatomic Classification of Uveitis





























Type


Primary Site of Inflammation


Includes


Anterior uveitis


Anterior chamber


Iritis, iridocyclitis, anterior cyclitis


Intermediate uveitis


Vitreous


Pars planitis, posterior cyclitis, hyalitis


Posterior uveitis


Retina or choroid


Focal, multifocal, or diffuse choroiditis; chorioretinitis, retinochoroiditis, retinitis, neuroretinitis


Panuveitis


Involves all compartments of the eye without one location predominating



SUN, Standardization of Uveitis Nomenclature.


Adapted from Jabs DA, Nussenblatt RB, Rosenbaum JT; Standardization of Uveitis Nomenclature (SUN) Working Group.


Standardization of uveitis nomenclature for reporting clinical data. Results of the First International Workshop. Am J Ophthalmol. 2005;140(3):509-516.









TABLE 2-2. Differential Diagnosis of the Major Causes of Uveitis



























































































































































































































ANTERIOR UVEITIS*



Granulomatous


Nongranulomatous



Sarcoidosis


Human leukocyte antigen (HLA) B27-associated (including ankylosing spondylitis, reactive arthritis, inflammatory bowel disease, and psoriatic arthritis)



Syphilis



Tuberculosis



Herpes simplex


Juvenile rheumatoid arthritis



Leptospirosis


Fuchs heterochromic iridocyclitis



Brucellosis


Posner-Schlossman (glaucomatocyclitic crisis)



Lens associated


Masquerade syndromes



Idiopathic


Uveitis-glaucoma-hyphema syndrome




Trauma




Kawasaki disease




Drug induced (rifabutin, cidofovir)


INTERMEDIATE UVEITIS



Sarcoid,




syphilis




Inflammatory bowel disease




Multiple sclerosis




Pars planitis (idiopathic)




Lymphoma, lyme




Other (tuberculosis, Behçet disease, Vogt-Koyanagi-Harada disease, Whipple disease, toxoplasmosis, endophthalmitis)



POSTERIOR UVEITIS



Focal Retinitis


Multifocal Retinitis



Toxoplasmosis


Syphilis



Onchocerciasis


Herpes simplex virus (acute retinal necrosis)



Cysticercosis


Cytomegalovirus



Masquerade syndromes


Sarcoidosis




Masquerade syndromes




Candidiasis




Progressive outer retinal necrosis




Eales disease




Diffuse unilateral subacute neuroretinitis


PANUVEITIS



Sympathetic ophthalmia




Vogt-Koyanagi-Harada




Behçet disease




Endophthalmitis




Sarcoidosis




Lens associated




Lyme disease




Masquerade syndromes




Toxoplasmosis




Syphilis




Tuberculosis



CHOROIDITIS



Focal Choroiditis


Multifocal Choroiditis



Tuberculosis


Histoplasmosis



Toxocariasis


Pneumocystis choroiditis



Nocardia


Serpiginous choroiditis



Candidiasis


Birdshot



Masquerade syndromes


Lymphoma




Acute posterior multifocal placoid pigment epitheliopathy




Multifocal choroiditis/punctate inner choroiditis




Masquerade syndromes




Cryptococcus




Mycobacterium


* This category is usually divided into granulomatous (mutton fat keratic precipitates) and nongranulomatous uveitis. All of the granulomatous entities can look nongranulomatous (especially with chronicity), but the nongranulomatous ones do not look granulomatous.


Pearl: Sarcoidosis, syphilis, tuberculosis, lyme, and lymphoma can look like anything.

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May 5, 2019 | Posted by in OPHTHALMOLOGY | Comments Off on Anatomic Classification of Uveitis
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