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New ACR EULAR guidelines for systemic sclerosis classification

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Curr Rheumatol Rep (2015) 17: 32
DOI 10.1007/s11926-015-0506-3
SCLERODERMA (J VARGA, SECTION EDITOR)
New ACR EULAR Guidelines for Systemic
Sclerosis Classification
Sindhu R. Johnson
Published online: 15 April 2015
# Springer Science+Business Media New York 2015
Abstract The American College of Rheumatology and
European League Against Rheumatism classification criteria
for systemic sclerosis are a significant advancement in the
field. This article describes the innovative, rigorous, criteria
development strategy that was used. The new criteria build
upon previous criteria by incorporating important elements
(proximal scleroderma, sclerodactyly, digital pits, pulmonary
fibrosis, Raynaud’s phenomenon, and scleroderma specific
autoantibodies). The new criteria add emphasis to the
vasculopathic manifestations, and include the early manifes-
tation of puffy fingers. Together, these enhancements have
resulted in a shift in the conceptual framework of the disease
for the next generation. The new criteria have improved sen-
sitivity and specifically, particularly among cases with early
disease, mild disease, or limited disease. The ability to classify
more cases, at an earlier stage, may confer the opportunity to
intervene and prevent disease progression. Undoubtedly, this
will lead to a paradigm shift in the conduct of clinical trials in
systemic sclerosis.
Keywords Systemic sclerosis . Scleroderma . Classification
criteria . Classification . Misclassification . Clinical
epidemiology . Cohort . Delphi . Nominal group technique .
Bayesian . Multicriteria decision analysis . Conjoint analysis .
Forced choice . 1000Minds . Mixed connective tissue disease
This article is part of the Topical Collection on Scleroderma
S. R. Johnson (*)
Toronto Scleroderma Program, Mount Sinai Hospital, Toronto
Western Hospital, Division of Rheumatology, Department of
Medicine, University of Toronto, Ground Floor, East Wing,
399 Bathurst Street, Toronto, ON, Canada M5T 2S8
e-mail: Sindhu.Johnson@uhn.ca
S. R. Johnson
Institute of Health Policy, Management and Evaluation,
University of Toronto, Toronto, ON, Canada
Introduction
Systemic sclerosis (also called scleroderma) is an autoim-
mune, multi-systemic, rheumatic disease that lacks a single
diagnostic test. A diagnosis is made based on recognition of
specific features and physician judgment. Classification
criteria are used to identify homogenous groups of patients
for inclusion into studies and can be used to inform the con-
cept of the disease. The new American College of
Rheumatology (ACR)–European League Against
Rheumatism (EULAR) classification criteria for systemic
sclerosis are an important advancement in this field. This ar-
ticle introduces the mosaic called systemic sclerosis, reviews
previous iterations of systemic sclerosis classification criteria,
discusses advances in the methodology of classification
criteria development, and highlights the development and per-
formance characteristics of the new criteria. This article dis-
cusses areas of controversy and the impact of the new criteria.
The Mosaic Called Systemic Sclerosis
Systemic sclerosis is likely a group of closely related condi-
tions that share features of autoimmunity, vasculopathy, and
fibrosis. How the disease manifests itself can vary consider-
ably. The disease’s pathognomonic feature, tightening of the
skin, can range from none at all (called systemic sclerosis sine
scleroderma), to mild involvement with puffy fingers, to
bound down skin affecting the extremities, face, and torso.
Other fibrotic manifestations may include pulmonary fibrosis,
flexion contractures, and tendon friction rubs. Systemic scle-
rosis is an inherently vasculopathic disease, and these mani-
festations can also vary [1••]. Raynaud’s phenomenon is pres-
ent in the majority of cases, such that the absence of
Raynaud’s phenomenon suggests consideration of an alterna-
tive diagnosis. Persistent digital hypoperfusion can lead to
32 Page 2 of 8 Curr Rheumatol Rep (2015) 17: 32

digital pitting, digital or extremity ulceration, and gangrene.
Other vasculopathic manifestations may include abnormal
nailfold capillaries, gastric antral vascular ectasia, scleroderma
renal crisis, and pulmonary arterial hypertension. Systemic scle-
rosis can vary in its initial presentation and course over time,
with differences in internal organ involvement and prognosis.
Previous Iterations of Systemic Sclerosis Classification
Given the absence of a single diagnostic test, it was recog-
nized that classification criteria were needed to distinguish
systemic sclerosis from other similar diseases, and to identify
more similar groups of patients for inclusion into research
studies. This would allow for more appropriate comparisons
across studies [2]. In 1968, a subcommittee of the American
Rheumatism Association Diagnostic and Therapeutic
Committee began efforts to develop what has come to be
known as the Preliminary Criteria for the Classification of
Systemic Sclerosis (Scleroderma) [2, 3]. The Scleroderma
Criteria Cooperative Study developed the SSc classification
criteria in 797 case and control subjects’ data, from 29 centers
in Canada, the United States, and Mexico. The presence of
either a major criterion (proximal scleroderma defined as
tightening, thickening, and non-pitting induration proximal
to the metacarpophalangeal or metatarsophalangeal joints) or
two or more minor criteria: (1) sclerodactyly, (2) digital pitting
scars of the fingertips or loss of the substance of the distal pad,
and (3) bilateral basilar pulmonary fibrosis were sufficient for
an individual to be classified as having systemic sclerosis [2]
(Table 1). Published in 1980, these criteria were quickly
adopted, highly cited [8], and served the rheumatology
community well for more than 30 years [9]. These criteria
were used in many of the landmark systemic sclerosis obser-
vational studies and clinical trials of the therapeutic interven-
tions. For medical educators, these criteria informed the con-
cept of systemic sclerosis for a generation.
It was later appreciated that this criteria set had some limi-
tations. Approximately 20 % of systemic sclerosis patients
diagnosed by expert clinicians did not fulfill the criteria [10].
These were patients with the limited subtype, early or mild
disease [11]. This resulted in their exclusion from participation
in clinical trials. Subsequently, alternative criteria sets and
modifications were proposed [4, 10] (Table 1). Lonzetti et al.
proposed that the addition of abnormal nailfold capillaries and
systemic sclerosis specific antibodies to the 1980 criteria could
improve the sensitivity for limited systemic sclerosis [10].
Nadashkevich et al. proposed an alternative classification
system [4, 5]. Using this system a case could be classified as
systemic sclerosis if three of the following ‘ABCDCREST’
criteria were present: autoantibodies to centromere proteins,
ScL-70 (topoisomerase-1, fibrillin), bibasilar pulmonary fi-
brosis, contractures of the digital joints or prayer sign, dermal
thickening proximal to the wrists, calcinosis cutis, Raynaud’s
phenomenon, esophageal distal hypomotility or reflux esoph-
agitis, sclerodactyly or non-pitting digital edema, or t-
elangiectasia. They demonstrated improved sensitivity
(99 %) and specificity (100 %) compared with the 1980
criteria [5]. However, this system was not widely adopted
nor externally validated [5].
In order to improve classification of systemic sclerosis at an
earlier stage, and incorporate vascular and serologic advances,
LeRoy and Medsger proposed an alternate classification sys-
tem [6] (Table 1). Patients could be classified as limited

Table 1 Previous iterations of

SSc classification criteria Reference SSc classification criteria

VEDOSS very early diagnosis of
systemic sclerosis, SSc systemic
sclerosis
ARA criteria 1980 [2] 1 major criterion: proximal scleroderma defined as tightening, thickening, and
non-pitting induration proximal to the metacarpophalangeal or
metatrasophalangeal joints or 2 or more minor criteria: (1) sclerodactyly,
(2) digital pitting scars of the fingertips or loss of the substance of the distal
pad, (3) bilateral basilar pulmonary fibrosis
Nadashkevich et al. Any 3 of ABCDCREST: (1) autoantibodies to: centromere proteins, ScL-70
2004 [4, 5] (topoisomerase-1, fibrillin), (2) bibasilar pulmonary fibrosis, (3) contractures
of the digital joints or prayer sign, (4) dermal thickening proximal to the
wrists, (5) calcinosis cutis, (6) Raynaud’s phenomenon, (7) esophageal
distal hypomotility or reflux esophagitis, (8) sclerodactyly or non-pitting
digital edema, (9) telangiectasia
LeRoy and Medsger Limited SSc (lSSc) (1) Raynaud’s phenomenon
2001 [6] And (2) abnormal wide-field nailfold capillaroscopy or (3) SSc selective
autoantibodies; limited cutaneous (lcSSc); Criteria for lSSc and cutaneous
changes distal to the elbow, knees, and clavicles; Diffuse cutaneous (dcSSc);
Criteria for lSSc and cutaneous involvement of the arms, chest, abdomen,
back, or thighs
Avouac et al. 2011, (1) Raynaud’s phenomenon, (2) puffy fingers, (3) antinuclear antibodies, and (4)
VEDOSS criteria [7] capillaroscopy or (5) SSc-specific antibodies
Curr Rheumatol Rep (2015) 17: 32
systemic sclerosis (lSSc) if they had Raynaud’s phenomenon
and abnormal wide-field nailfold capillaroscopy or systemic
sclerosis selective autoantibodies. Patients could be classified
as limited cutaneous (lcSSc), if they fulfilled criteria for lSSc
and had cutaneous changes distal to the elbow, knees, and
clavicles. Patients could be classified as diffuse cutaneous
(dcSSc) if they fulfilled criteria for lSSc and had cutaneous
involvement of the arms, chest, abdomen, back, or thighs [6].
This classification system was not as widely used, possibly
because lSSc and lcSSc are easily confused [12]. Indeed,
when sub-setting systemic sclerosis, the criteria of LeRoy
et al. 1988 categorizing cases of systemic sclerosis as limited
versus diffuse is most frequently cited [13, 14]. Using this
system, individuals with no skin involvement or skin involve-
ment of the hands, face, feet, and forearms (acral) are classi-
fied as ‘limited.’ Individuals with skin involvement of the
torso and acral regions are classified as ‘diffuse’ [13]. It is
not commonly appreciated that this system does not classify
skin involvement of the proximal arms or thighs [15]. Twelve
other systemic sclerosis subset classification systems have
been proposed ranging between two and six subsets
[16–26]. These systems are infrequently utilized, and systems
with three or more subsets do not appear to have added pre-
dictive value with regards to prognosis [14].
Due to concerns that internal organ involvement may de-
velop before an individual fulfills the 1980 criteria [27], the
European League Against Rheumatism Scleroderma Trial and
Research (EUSTAR) group proposed criteria for the very ear-
ly diagnosis of SSc (VEDOSS) [7]. Using this system, an
individual with the ‘red flags’ of Raynaud’s phenomenon,
puffy fingers, and antinuclear antibodies could be classified
as early systemic sclerosis if abnormal nailfold capillaroscopy
or systemic sclerosis specific antibodies are present [7]
(Table 1).
Advances in Criteria Development
The importance of rigorous classification criteria development
was recognized early on. Valid criteria are critical to epidemi-
ologic and clinical studies as misclassification would result in
individuals without the disease being included in the disease
group, and individuals with the disease being excluded [8].
Misclassification could cause anxiety in the patient, lead in-
vestigators towards misdirected efforts, and lead the physician
to institute the incorrect treatment [11]. Standards for the de-
velopment of classification criteria have intensified with time.
Both the American College of Rheumatology and European
League Against Rheumatism have made recommendations
for classification criteria development based on the principles
of measurement science [28–31]. These recommendations call
for increased collaboration between those with disease-
specific content expertise and those with methodologic
Page 3 of 8 32
expertise (clinical epidemiologists). The recommendations
call for consideration of the purpose of the criteria (clinical
research, epidemiologic studies), methods of criteria genera-
tion (literature search, expert and/or patient opinion), evalua-
tion of psychometric properties (validity, reliability, feasibili-
ty), and careful selection of cases and appropriate controls
[31].
A review of all the classification criteria in the rheumatic
diseases, undertaken with the support of the American
College of Rheumatology Classification and Response
Criteria Subcommittee of the Committee on Quality
Measures, found that many criteria sets in rheumatology
had methodologic issues relating to the derivation sam-
ple case and control selection [8]. It was recommended
that criteria sets should be independently validated in
cohorts of patients and controls separate from the deri-
vation cohort [8]. Given that disease-specific knowledge
had been gained, and the rigors of classification criteria
development had changed, it was felt that many classi-
fication criteria sets needed to be updated [8].
New Classification Criteria for Systemic Sclerosis
With the support of the American College of Rheumatology
and European League Against Rheumatism, a joint interna-
tional, multiphase effort was undertaken to develop a new
classification criteria set [32••, 33••]. The main objective
was to develop a criteria set that classified a greater breadth
of the spectrum of systemic sclerosis, thereby allowing more
individuals the opportunity to participate in clinical trials
[32••, 33••]. In particular, the aim was to classify those with
early and/or limited disease. The aim was also to try to incor-
porate new knowledge gained over the recent decades regard-
ing systemic sclerosis, while using a state-of-the-art
methodologic approach. This approach involved a balanced
use of expert-based and data-driven methods, and implemen-
tation of bias reduction strategies. Most notably, considerable
attention was made to avoid bias introduced by circular rea-
soning. This bias was an important threat to the development
of other criteria sets in rheumatology [8, 28]. This is a bias that
can occur when the investigators who derive the classification
criteria, test the criteria in data from patients from their prac-
tice, who initially informed their concept of the disease [28].
The systemic sclerosis classification criteria steering commit-
tee made efforts to ensure that systemic sclerosis experts who
were asked to contribute knowledge were separate from the
centers that contributed data [32••, 33••].
The candidate criteria were nominated through two inde-
pendent Delphi exercises conducted in North America
through the Scleroderma Clinical Trials Consortium (SCTC)
and in Europe through EUSTAR [34•]. The SCTC systemic
sclerosis experts were asked to nominate items used in daily
32 Page 4 of 8
practice to diagnose SSc whereas the EUSTAR systemic scle-
rosis experts were asked to identify items suitable for diagno-
sis of early systemic sclerosis [34•, 35]. The subtle differences
in emphasis between the two groups allowed for a broader
array of items for nomination. One hundred six experts rated
168 items. Low-scoring items were excluded, leaving 102
items. The items were rated and reduced through a consensus
meeting of 16 international experts using nominal group tech-
nique. This reduced the candidate criteria to 23 items [34•].
Using applied Bayesian methods, the face, discriminant
and construct validity of these items were tested using
cases and controls sampled from the Toronto, Pittsburg,
Madrid, and Berlin Connective Tissue Disease,
Canadian Scleroderma Research Group, and 1,000
Faces of Lupus databases [36•]. The criteria were tested
in 750 SSc cases and 1,071 controls (systemic lupus
erythematosus, inflammatory myositis, Sjogren’s syn-
drome, Raynaud’s syndrome, mixed connective tissue
disease, and idiopathic pulmonary arterial hypertension).
Scleroderma skin changes (odds ratio (OR) 427, 95 %
Credible Interval (CrI) 257, 691), telangiectasia (OR 91,
95 % CrI 58, 155), RNA polymerase III antibody (OR
75, 95 % CrI 13, 313), and puffy fingers (OR 35, 95
% CrI 24, 49) were highly discriminatory between cases
and controls whereas reduced forced vital capacity (OR 0.9,
95 % CrI 0.6, 1.3) and reduced DLCO (OR 1.5, 95 % CrI 1.1,
2.0) did not discriminate well [36•].
Using multicriteria decision analysis (also known as con-
joint analysis or forced choice methods) with 1000Minds soft-
ware, the 23 candidate criteria were further reduced and
weighted, and a threshold for classification identified [37•].
Through this process, ‘skin thickening sparing the fingers’
was made an exclusionary criterion. If this is present, the
classification criteria should not be applied (see Fig. 1).
BSkin thickening of the fingers extending proximally to the
metacarpophalangeal joints^ was a sufficient criterion. If pres-
ent, the individual could be classified as systemic sclerosis.
Due to poor ability to discriminate cases from controls, limited
reliability in the clinical setting, or little added value in clas-
sification (low weight), digital pulp loss, dysphagia, esopha-
geal reflux, diffusing capacity, forced vital capacity, and anti-
nuclear antibody were removed. Acro-osteolysis, pitting
scars, and digital ulcers were grouped under the heading ‘fin-
ger tip lesions.’ Systemic sclerosis specific antibodies were
also grouped under one criterion [37•]. This methodologic
approach facilitated a system of classification that produced
a measure of probability that an individual with certain fea-
tures could be classified as systemic sclerosis. The draft clas-
sification system and weights were then tested against a range
of cases that reflected low to high probability of having sys-
temic sclerosis rated by experts. A total score of 9 or more was
identified as the threshold with the least misclassification
[32••, 33••] (Fig. 1).
Curr Rheumatol Rep (2015) 17: 32
Performance of the New Systemic Sclerosis Criteria
The operating characteristics of the criteria set was tested in
derivation and validation cohorts consisting of cases and con-
trols from 13 North American and 10 European centers. The
controls were purposefully selected to represent conditions
that could be confused with SSc and included cases of eosin-
ophilic fasciitis, scleromyxedema, dermatomyositis, poly-
myositis, mixed connective tissue disease, undifferentiated
connective tissue disease, generalized morphea, nephrogenic
sclerosing fibrosis, and graft versus host disease. Using the
derivation cohort, the criteria set was further refined. Finger
flexion contractures, calcinosis, tendon or bursal friction rubs,
renal crisis, and esophageal dilation were removed, as they did
not provide added value in sensitivity or specificity.
In the derivation cohort, the new classification criteria had
excellent sensitivity (0.95, 95 % confidence interval (CI) 0.90,
0.98) and specificity (0.93, 95 %CI 0.86, 0.97). This remained
true in the validation cohort with a sensitivity of 0.91 (95 %CI
0.87, 0.94) and specificity 0.92 (95 %CI 0.86, 0.96). The new
criteria had demonstrable added value above the 1980 criteria,
and the LeRoy and Medsger criteria. Other groups have inde-
pendently, externally, validated the criteria, and the excellent
performance characteristics hold true [38, 39] (Table 2). The
improvement in classification criteria sensitivity has been
partly attributed to the inclusion of Raynaud’s phenomenon
and puffy fingers [38]. The sensitivity of the new criteria have
marked improvement in limited cutaneous disease (particular-
ly individuals without skin involvement proximal to the
metacarpophalangeal joints) and those with early disease less
than or equal to 3 years [38] (Table 2).
Areas of Controversy
Since its publication, the new classification criteria have had
some criticism. One area of controversy relates to the applica-
tion of the classification criteria to individuals with mixed
connective tissue disease [15]. The 1980 criteria investigators
made the decision to exclude mixed connective tissue disease
patients from the derivation processes as they felt that it was
‘virtually impossible’ to distinguish from SSc [15]. Both the
criteria testing [36•], derivation, and validation cohorts [32••]
of the new SSc criteria included mixed-connective-tissue-
disease patients. Since many mixed connective tissue disease
patients are SSc-predominant in their clinical features, it was
decided that these criteria could be applied to mixed connec-
tive tissue disease patients when considering enrollment into
systemic sclerosis clinical trials [32••]. In a Norwegian cohort
of 178 mixed connective tissue disease patients, 10 % fulfilled
the new criteria [39]. It has been noted that this cohort study
did not collect data on telangiectasia or nailfold capillary
abnormalities, therefore 10 % may be an underestimate [9].
Curr Rheumatol Rep (2015) 17: 32
Fig. 1 ACR EULAR
classification criteria for systemic
sclerosis
A second area of controversy relates to the difference be-
tween classification criteria and diagnostic criteria [40]. Most
of the rheumatic diseases do not have a singular diagnostic
test. Physician judgment, taking into account clinical and in-
vestigational subtleties, is required to make a diagnosis.
Physician expertise is gained through years of specialized ed-
ucation and training [41]. This is complemented by years of
clinical experience. Although it has been recognized that clas-
sification criteria inform that concept of the disease [5], the
American College of Rheumatology makes a clear distinction
between classification and diagnostic criteria [40]. Diagnostic
criteria are intended to reflect aspects of the disease that char-
acterize most cases that encompass the spectrum of the
Page 5 of 8 32
disease. Classification criteria are designed to identify a
well-characterized group of patients within the spectrum of
the disease. The disease prevalence, prevalence of similar dis-
eases, and geography can affect the degree of overlap [40].
The performance of criteria depends on both the pre-test prob-
ability of the disease (prevalence of the disease and similar
conditions), and the sensitivity and specificity of the criteria
[40]. Racial and ethnic variation within a geographical area
may also affect this. Importantly, accurate diagnosis may have
impact on physician billing and patient medical coverage [40].
Diagnostic criteria that are highly specific will leave some
cases undiagnosed consequently resulting in denial of treat-
ment coverage if insurance companies and government
32 Page 6 of 8 Curr Rheumatol Rep (2015) 17: 32

Table 2 Performance of ACR EULAR SSc classification criteria

Cohort ACR EULAR criteria ACR EULAR criteria 1980 criteria 1980 Criteria
Sensitivity (95 % CI) Specificity (95 % CI) Sensitivity (95 % CI) Specificity (95 % CI)

Derivation cohort [32••] 95 % (90 %, 98 %) 93 % (86 %, 97 %) 80 % (72 %, 87 %) 77 % (68 %, 84 %)

Validation cohort [32••] 91 % (87 %, 94 %) 92 % (86 %, 96 %) 75 % (70 %, 80 %) 72 % (64 %, 79 %)
≤3 years disease duration [32••] 91 % (86 %, 96 %) 90 % (70 %, 99 %) 75 % (70 %, 80 %) 72 % (63 %, 79 %)
Canadian Scleroderma Research Group [38] 98 % NA 88 % NA
Limited cutaneous SSc [38] 99 % NA 86 % NA
≤3 years disease duration [38] 99 % NA 85 % NA
Norwegian cohort [39] 96 % (93 %, 97 %) 90 % (85 %, 94 %) 75 % NA

ACR American College of Rheumatology, EULAR European League Against Rheumatism, NA not available

agencies use the diagnostic criteria as a standard for reim-
bursement [40]. Conversely, individuals misdiagnosed with
a disease may have barriers in getting health insurance or life
insurance [40]. For these reasons, the diagnosis of systemic
sclerosis continues to remain in the realm of physician judg-
ment and not classification criteria.
A third area of controversy is the applicability of the new
systemic sclerosis classification criteria to juvenile systemic
sclerosis. Neither pediatric rheumatologists nor pediatric rheu-
matology case or control subjects were included in the devel-
opment or validation of the new criteria. Yet, studies do in-
clude both juvenile and adult onset systemic sclerosis patients
[42]. There is a need to test the validity of the new classifica-
tion criteria in juvenile systemic sclerosis. An additional issue
to be considered will include the timing of classification in
these cases. At present, the threshold of 16 years of age is
arbitrary and not supported by a biologic rationale [43].
A fourth controversy relates to the complexity of the sys-
temic sclerosis criteria set. There has been a trend in the rheu-
matic disease classification criteria towards additive point sys-
tems (e.g., rheumatoid arthritis, gout) [44]. Critics dislike the
cognitive mathematics required from a numeric additive sys-
tem and are concerned that there are still too many criteria.
Attempts to further reduce the number of criteria and simplify
the weighting (analogous to major and minor criteria) resulted
in a loss of sensitivity and specificity [32••]. The American
College of Rheumatology European League Against
Rheumatism systemic sclerosis classification criteria reflect a
tradeoff between a comprehensive system versus a useful one
that is easy to use [9]. A freely accessible, online tool has been
developed to assist in calculation of the score (http://
rheuminfo.com/physician-tools/ssc-calculator).
systemic sclerosis are a major advance in the field for a num-
ber of reasons. First, it reflects application of an innovative,
rigorous, criteria development strategy that is less susceptible
to bias, making this a methodologic standard on which subse-
quent criteria development will be based. These criteria reflect
a major, successful, international collaboration of 44 investi-
gators, and data on >1150 systemic sclerosis cases and >1300
controls [32••, 34•, 36•].
The new criteria build upon previous criteria by incorpo-
rating important elements (proximal scleroderma,
sclerodactyly, digital pits, pulmonary fibrosis, Raynaud’s phe-
nomenon, and scleroderma specific autoantibodies). The new
criteria add emphasis to the vasculopathic manifestations (pul-
monary arterial hypertension, telangiectasia, abnormal
nailfold capillaries). It emphasizes early manifestations, in
particular puffy fingers. Indeed, the VEDOSS group has dem-
onstrated that the inclusion of puffy fingers as a criterion im-
proves the ability to classify cases of early disease [45].
Together, these enhancements have resulted in a shift in the
conceptual framework of the disease for the next generation.
The new systemic sclerosis classification criteria have im-
proved sensitivity and specificity, most notably among those
with early, mild, or limited disease. The new criteria have
improved ability to classify cases of systemic sclerosis sine
scleroderma [38]. Patients with subtle features or mild disease
should be monitored carefully. We now have the opportunity
to learn whether these patients will evolve into more severe
disease [11]. The ability to classify a case at an earlier stage
may confer the opportunity to intervene and prevent disease
progression [11].

Conclusion

What Has Been Gained? In summary, the heterogeneous clinical phenotype, autoanti-
body profile, and prognosis of systemic sclerosis results in the
The new American College of Rheumatology European need for classification criteria to identify more homogenous
League Against Rheumatism classification criteria for groups of subjects for participation in research studies.
Curr Rheumatol Rep (2015) 17: 32
Although previous classification criteria have been proposed
and the 1980 classification criteria served the community well,
significant subgroups of SSc cases were not correctly classi-
fied. Developed using a rigorous methodologic approach, the
new American College of Rheumatology European League
Against Rheumatism classification criteria for systemic scle-
rosis build upon previous criteria, add emphasis to the
vasculopathic manifestations, and include the early manifes-
tation of puffy fingers. The criteria set have shifted the con-
ceptual framework of the disease for the next generation. The
new criteria have improved sensitivity and specifically, partic-
ularly among cases with early disease, mild disease, or limited
disease. Undoubtedly, this will lead to a paradigm shift in the
conduct of clinical trials in systemic sclerosis.
Acknowledgments Sindhu Johnson has been awarded a Canadian In-
stitutes of Health Research Clinician Scientist Award and is supported by
the Freda Fejer Fund and the Norton-Evans Fund for Scleroderma
Research.
Compliance with Ethics Guidelines
Conflict of Interest Sindhu Johnson declares grants from American
College of Rheumatology and from the European League Against
Rheumatism, during the writing of this paper. She declares that she is
Co-chair of the American College of Rheumatology Classification and
Response Criteria subcommittee.
Human and Animal Rights and Informed Consent This article does
not contain any studies with human or animal subjects performed by any
of the authors.
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