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The American College of Rheumatology 1990 criteria for the classification of vasculitisSummary.

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1135
THE AMERICAN COLLEGE OF RHEUMATOLOGY
1990 CRITERIA FOR THE CLASSIFICATION
OF VASCULITIS
Summary
JAMES F. FRIES,GENE G. HUNDER, DANIEL A. BLOCH, BEAT A. MICHEL, WILLIAM P. AREND,
LEONARD H. CALABRESE, ANTHONY S. FAUCI, RAND1 Y. LEAVITT, J. T. LIE,
ROBERT W. LIGHTFOOT, JR., ALFONSE T. MASI, DENNIS J. McSHANE, JOHN A. MILLS,
MARY BETTY STEVENS, STANLEY L. WALLACE, and NATHAN J. ZVAIFLER
The studies described in the preceding pages
regarding the classification of vasculitis are some of
the first to be performed through the analysis of patient
data collected in a prospective manner. The aim of this
project was to develop classification criteria that
would promote the more uniform description of the
patients when various research endeavors are reported.
The large number of patients that were included
(n = 1,000) and the inclusion of data from multiple
centers (n = 47) are major strengths of these studies,
helping to reduce referral bias by individual physicians
and centers. The information on the patient data forms
was reviewed carefully by the subcommittee members
From the American College of Rheumatology Subcommittee on Classification of Vasculitis (Diagnostic and Therapeutic
Criteria Committee of the Council on Research).
James F. Fries, MD: Stanford University, Stanford, CA;
Gene G. Hunder, MD: Mayo Clinic, Rochester, MN, and Chair,
Subcommittee on Classification of Vasculitis; Daniel A. Bloch.
PhD Stanford University, Stanford, CA; Beat A. Michel. MD.
Rheumaklinik Universitiitsspital. Zurich, Switzerland; William P.
Arend, MD: University of Colorado Health Science Center, Denver, CO; Leonard H. Calabrese, DO: Cleveland Clinic Foundation,
Cleveland, OH; Anthony S. Fauci. MD: NIAID. NIH, Bethesda,
MD; Randi Y. Leavitt, MD, PhD: NIAID, NIH, Bethesda, MD;
J. T. Lie, MD: Mayo Clinic, Rochester, MN; Robert W. Lightfoot,
Jr., MD: University of Kentucky, Lexington, KY; Alfonse T. Masi,
MD, DrPH: University of Illinois College of Medicine, Peoria, 1L;
Dennis J. McShane, MD: Stanford University, Stanford. CA; John
A. Mills, M D Massachusetts General Hospital, Boston, MA: Mary
Betty Stevens. MD: Johns Hopkins University, Baltimore, MD;
Stanley L.Wallace. MD: SUNY Downstate Medical Center, Brooklyn, NY (Dr. Wallace is deceased); Nathan J. Zvaifler, MD: University of California, San Diego, San Diego, CA.
Address reprint requests to the American College of Rheumatology, 17 Executive Park Drive NE, Suite 480, Atlanta, GA
30329.
Submitted for publication October 2 , 1989; accepted in
revised form April 3, 1990.
Arthritis and Rheumatism, Vol. 33, No. 8 (August 1990)
before and after the data were coded and entered into
the computer; these steps also enhanced the accuracy.
Statistical analyses utilized ARAMIS (Arthritis, Rheumatism, and Aging Medical Information System) procedures and staff experienced in prior efforts of criteria
development.
Flexibility was provided by the development of
criteria by 2 different methods: the traditional format
and the classification tree. The criteria in both formats
are presented in relatively simple terms and should
prove easy to use. The clinical information needed to
fulfill the criteria variables can be obtained from the
patient’s medical history, the physical examination,
and established laboratory tests that can be performed
in most centers. Data regarding less common tests
were not reported on the forms in all instances;
however, we examined trends and found that such
factors as immune complexes, reduced complement
levels, or other immunologic data did not indicate that
these might have been important discriminators had
the information been available for all patients. Yet,
further studies may show that other tests are important. One such possibility, the test for antineutrophil
cytoplasmic antibodies, which was recently reported
in Wegener’s granulomatosis, was described only after
data had been collected from many patients (Abbott F,
Jones S, Lockwood CM,Rees AJ: Autoantibodies to
glomerular antigens in patients with Wegener’s granulomatosis. Nephrol Dial Transplant 4: 1-8, 1989).
Biopsy verified the presence of vasculitis in the
majority of cases. It appeared that patients with negative biopsies or those who had not undergone biopsy
were not different from those whose biopsy findings
were positive. Many patients can be classified without
FRIES ET AL
1136
Table 1. Summary of the sensitivity and specificity of the American College of Rheumatology 1990 classification of vasculitis
Vasculitis type
Polyarteritis nodosa
Churg-Strauss
syndrome
Wegener’s
granulornatosis
Hypersensitivity
vasculitis
Henoch-Schdnlein
PUrPUra
Giant cell (temporal)
arteritis
Takayasu arteritis
Classification
method
Sensitivity
Specificity
(%I
(%)
Traditional
Tree
Traditional
Tree
Traditional
Tree
Traditional
Tree
Traditional
Tree
Traditional
Tree
Traditional
Tree
82.2
87.3
85.0
95.0
88.2
87.1
71.0
78.5
87.1
89.4
93.5
95.3
90.5
92.1
86.6
89.3
99.7
99.2
92.0
93.6
83.9
78.7
87.7
88.1
91.2
90.7
97.8
97.0
biopsy data. From a clinical standpoint, however, a
biopsy should be obtained when possible.
Several limitations also should be noted. The
studies were not global in the sense that criteria were
established for only the most common types of vasculitis. Criteria for each type of vasculitis were developed by comparing findings in patients with that type
of vasculitis with findings in the remaining group of
patients with other forms of vasculitis; this served the
general question of differential diagnosis. Comparisons of the criteria sets between individual types of
vasculitis were not extensive.
The sensitivity and specificity rates for the
vasculitides studied varied considerably, from approximately 70% to more than 99% (Table 1). In most
instances, and when possible, we selected criteria sets
that were reasonably balanced between sensitivity and
specificity values. Rates of over 90% were found in
Churg-Strauss syndrome, giant cell (temporal) arteritis, and Takayasu arteritis. These conditions are fairly
well defined from a clinical standpoint, although they
have a broad range of manifestations. Rates below
80% were found for hypersensitivity vasculitis. This
syndrome has been difficult to define, and its definition
has varied widely from one author to another.
A specific formula to guide the investigator
toward the proper criteria set to use when classifying a
patient was not developed. If the diagnosis is known,
that criteria set should, of course, be used. In other
instances, the physician needs to make a judgment
regarding the patient’s most likely diagnosis, and then,
use the appropriate criteria set. When the diagnosis is
unknown, it may be useful to examine more than one
set of criteria. Experience with using the criteria will
provide the best guidance. Either the traditional format or the tree classification can be applied; however,
uniformity should be maintained throughout a given
study.
These criteria sets may be used to describe the
patients included in studies of vasculitis. Their use
should enhance uniformity of reporting. A brief perusal of the literature reveals that a wide variety of
descriptions (or lack of descriptions) of patients have
been used in reporting these conditions. As new
information becomes available about these syndromes
and their specific causes, it is expected that it may
become necessary to revise these sets of classification
criteria. Despite these caveats, which are unavoidable
given current knowledge of these syndromes and their
largely descriptive nature, experience with classification criteria in other areas of rheumatic disease has
shown that criteria can be extremely useful. They help
standardize our clinical vocabularies, aid in estimating
prognosis, encourage more systematic definition of
study patients, and provide a conceptual base against
which to assess the merits of new information.
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