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● PURPOSE: To begin a process of standardizing the J Ophthalmol 2005;140:509 –516. © 2005 by Elsevier
methods for reporting clinical data in the field of uveitis. Inc. All rights reserved.)
● DESIGN: Consensus workshop.
T
● METHODS: Members of an international working group HE FIELD OF UVEITIS DEALS WITH MULTIPLE DISEASE
were surveyed about diagnostic terminology, inflamma- entities, some of which are caused directly by
tion grading schema, and outcome measures, and the infectious agents and others of which appear to be
results used to develop a series of proposals to better immune-mediated. Many uveitic entities are associated
standardize the use of these entities. Small groups em- with systemic immune-mediated diseases, such as sarcoid-
ployed nominal group techniques to achieve consensus osis, the HLA-B27-associated spondyloarthropathies, and
on several of these issues. Behçet’s disease, whereas others are limited to the eye.
● RESULTS: The group affirmed that an anatomic classi- Although attempts have been made to standardize some
fication of uveitis should be used as a framework for aspects of uveitis,1 in general there is limited standardiza-
subsequent work on diagnostic criteria for specific uveitic tion of classification criteria, inflammation grading
syndromes and that the classification of uveitis entities schema, and outcomes.2 Standardization would enhance
should be on the basis of the location of the inflammation greatly the comparability of clinical research from different
and not on the presence of structural complications. centers, permit meta-analyses, and assist in the develop-
Issues regarding the use of the terms “intermediate ment of a more complete and meaningful picture of the
uveitis,” “pars planitis,” “panuveitis,” and descriptors of clinical course of these diseases and their response to
the onset and course of the uveitis were addressed. The treatment.
following were adopted: standardized grading schema for The American College of Rheumatology (ACR) has
anterior chamber cells, anterior chamber flare, and for developed classification criteria for many of the rheumatic
vitreous haze; standardized methods of recording struc- diseases such as rheumatoid arthritis and systemic lupus
tural complications of uveitis; standardized definitions of erythematosus.3,4 These criteria have been developed
outcomes, including “inactive” inflammation, “improve- through a standard process and validated against large
ment” and “worsening” of the inflammation, and “corti- databases, in an effort to maximize sensitivity and speci-
costeroid sparing,” and standardized guidelines for ficity. In the field of uveitis, provisional criteria have been
reporting visual acuity outcomes. developed for a limited number of disorders (acute retinal
● CONCLUSIONS: A process of standardizing the ap- necrosis, progressive outer retinal necrosis, Vogt-Koyanagi-
proach to reporting clinical data in uveitis research has Harada disease, and tubulointerstitial nephritis with uve-
begun, and several terms have been standardized. (Am itis),5– 8 and they still await validation. Additionally, there
are criteria for the systemic portion of three diseases in
Accepted for publication Mar 17, 2005. which uveitis is an important feature (ankylosing spondy-
A listing of members of The Standardization of Uveitis Nomenclature
(SUN) Working Group appears in the Appendix. litis, juvenile idiopathic arthritis, and Behçet’s dis-
Supported by the American Uveitis Society and by an unrestricted ease),9 –11 but not for the uveitis in these diseases. Like the
grant from Centocor, Inc, Malvern, Pennsylvania, USA. ACR classifications of arthritis and vasculitis, which are on
Inquiries to Douglas A. Jabs, MD, MBA, The Wilmer Eye Institute,
550 North Broadway, Suite 700, Baltimore, MD 21205; fax: 410-955- the basis of the anatomic pattern of the disease, the most
0629djabs@jhmi.edu widely used classification of uveitis is the one devised by
Term Definition
treatment can be reported, but because of the variability including inactive disease on treatment, inactive disease
among practitioners in indications for treatment, it should after discontinuing treatment, and inactive disease for a
not be used as the only criterion for reporting elevated specified duration after discontinuing treatment. Because
intraocular pressure. chronic uveitis may be a life-long problem, which can be
controlled by treatment but relapses promptly after discon-
● OUTCOMES AND RESULTS REPORTING: The activity tinuing treatment, there was consensus that the term
of anterior chamber inflammation should be on the basis of remission should be reserved for inactive disease for at least
the cells in the anterior chamber. High-speed optical 3 months after discontinuing all treatments for eye disease.
coherence tomography of the anterior chamber has dem- Clinical studies of immunosuppressive drugs and bio-
onstrated that a rare cell (but less than 1 per field on logic agents for severe uveitis can evaluate either the
standard slit-lamp examination) may be present in the response of active uveitis to the drug being evaluated or
anterior chamber of normal individuals.26 Therefore, it was the ability of the drug being evaluated to maintain inactive
agreed that for reporting purposes, inactive anterior uveitis disease in the face of tapering other drugs, such as systemic
should be defined as rare cells or less. The presence of one corticosteroids. Although global scoring systems for a
cell in every field is indicative of 0.5⫹ cells (or in some reduction in the total corticosteroid and immunosuppres-
systems “trace cells”) and, for reporting purposes, should sive drug regimen may be a desirable goal for research
not be considered inactive uveitis. As with the inability to studies, in clinical practice a reduction in the prednisone
reach consensus on a grading system for vitreous cells, no dose for adults to 10 mg per day or less is a primary goal of
consensus could be reached on a definition of inactive immunosuppressive drug therapy.20 Therefore, there was
vitritis on the basis of vitreous cells. consensus that for reporting purposes in studies of adult
Although the goal of treatment of uveitis is to suppress patients, reduction in the dose of prednisone to 10 mg per
the inflammation completely (“inactive” disease), for the day or less (or its equivalent for other corticosteroids)
short-term evaluation of new therapies, it may be appro- while maintaining inactive uveitis be considered the pri-
priate to determine whether the inflammation has im- mary outcome for successful corticosteroid sparing. Although
proved or worsened (Table 5). Given the semiquantitative other outcomes also may be reported (for example, discon-
nature of the grading systems (for example, for anterior tinuation of prednisone), in studies where corticosteroid
chamber cells, vitreous haze), it was agreed that at least a sparing is an outcome, reduction in the dose of prednisone
two-step decrease in the level of inflammation for improve- to 10 mg per day or less should be reported and should be
ment and at least a two-step increase in the level of the primary measure of this outcome.
inflammation for worsening were better criteria than one- Data from clinical series should use accepted statisti-
step changes. However, because of floor and ceiling effects cal methods and should not report events or outcomes as
(that is, 3⫹ only can increase by one step and 0.5⫹ only the proportion of a population when there is variable
can decrease by one step), the definition of improvement follow-up.27 Instead, the proportion with the outcome at
should include a decrease in inflammation from 0.5⫹ to presentation (or study entry) should be reported and the
inactive, and the definition of worsening should include an event rate during follow-up should be reported for
increase from 3⫹ to the maximum grade. Hence, improve- longitudinal studies. In those series with complete or
ment in the inflammation will be defined as either a nearly complete follow-up, the distribution of outcomes
two-step decrease in the level of inflammation or a at defined time point(s) after presentation (or study
decrease to “inactive,” and worsening of the inflammation entry) can be reported. “Final visual acuity,” defined as
will be defined as either a two-step increase in the level of the last measured acuity in a series of patients with
inflammation or an increase to the maximum grade. The variable follow-up, should not be reported, because it is
group considered several definitions of the term remission, a flawed concept that may introduce uncontrolled bias