Review

doi: 10.1111/joim.12524

Diagnosis and classification of idiopathic inflammatory

myopathies
I. E. Lundberg1, F. W. Miller2, A. Tjarnlund1 & M. Bottai3
From the 1Rheumatology Unit, Department of Medicine, Karolinska University Hospital, Solna, Karolinska Institutet, Stockholm, Sweden;
2
National Institute of Environmental Health Sciences, National Institutes of Health Clinical Research Center, Bethesda, MD, USA; and
3
Institute for Environmental Medicine, Karolinska Institutet, Stockholm, Sweden

Content List Read more articles from the symposium: The First International Conference on Myositis.

Abstract. Lundberg IE, Miller FW, Tj

arnlund A, Bottai M
(Karolinska Institutet, Stockholm, Sweden; National
Institutes of Health Clinical Research Center,
Bethesda, MD, USA). Diagnosis and classification
of idiopathic inflammatory myopathies (Review
Symposium). J Intern Med 2016; 280: 3951.
The idiopathic inflammatory myopathies (IIMs) are
a heterogeneous group of diseases, collectively
termed myositis, sharing symptoms of muscle
weakness, fatigue and inflammation. Other organs
are frequently involved, supporting the notion that
these are systemic inflammatory diseases. The
IIMs can be subgrouped into dermatomyositis,
polymyositis and inclusion body myositis. The
myositis-specific autoantibodies (MSAs) identify
other and often more distinct clinical phenotypes,
such as the antisynthetase syndrome with antisyn-
thetase autoantibodies and frequent interstitial
lung disease and anti-SRP and anti-HMGCR
autoantibodies that identify necrotizing myopathy.
The MSAs are important both to support myositis
diagnosis and to identify subgroups with different
patterns of extramuscular organ involvement such
as interstitial lung disease. Another cornerstone in
the diagnostic procedure is muscle biopsy to iden-
tify inflammation and to exclude noninflammatory
myopathies. Treatment effect and prognosis vary
by subgroup. To develop new and better therapies,
validated classification criteria that identify dis-
tinct subgroups of myositis are critical. The lack of
such criteria was the main rationale for the devel-
opment of new classification criteria for IIMs,
which are summarized in this review; the historical
background regarding previous diagnostic and
classification criteria is also reviewed. As the IIMs
are rare diseases with a prevalence of 10 in
100 000 individuals, an international collabora-
tion was essential, as was the interdisciplinary
effort including experts in adult and paediatric
rheumatology, neurology, dermatology and epi-
demiology. The new criteria have been developed
based on data from more than 1500 patients from
47 centres worldwide and are based on clinically
easily available variables.
Keywords: criteria, dermatomyositis, idiopathic
inflammatory myopathy, inclusion body myositis,
polymyositis.

The Master said, . . . If names are not Introduction

correct, language is not in accordance
The idiopathic inflammatory myopathies (IIMs),
with the truth of things. If language is not
collectively termed myositis, are a heterogeneous
in accordance with the truth of things,
group of disorders that are characterized clinically
affairs cannot be carried out to success . . .
by chronic muscle weakness and low muscle
Therefore a superior man considers it
endurance and by inflammatory cell infiltrates in
necessary that the names he uses be
muscle tissue. Based on differences in clinical and
spoken appropriately . . . What the supe-
histopathological features, they have for many
rior man requires, is just that in his words
years been subdivided into polymyositis (PM),
there may be nothing incorrect.
dermatomyositis (DM) and inclusion body myositis
Confucius, Chinese sage and philosopher,
(IBM) [1]. The frequent presence of inflammatory
551479 BC, from Analects, Book XIII

2016 The Association for the Publication of the Journal of Internal Medicine 39
 I. E. Lundberg et al.
cell infiltrates with a high proportion of T lympho-
cytes in muscle tissue together with the commonly
occurring autoantibodies suggests that these are
autoimmune disorders. IIMs are not only muscle
disorders as other organs are often affected, such
as the skin in DM and the lungs, heart, joints and
gastrointestinal tract in both PM and DM, suggest-
ing that these are systemic autoimmune disease.
Interestingly, the newly identified so-called myosi-
tis-specific autoantibodies (MSAs) are associated
with distinct clinical phenotypes as reviewed in
detail by Betteridge and McHigh in this issue of
Journal of Internal Medicine [2]. Recently, other
subsets of IIMs have been identified, including
clinically amyopathic DM and the so-called
immune-mediated necrotizing myopathy (IMNM)
which is characterized predominantly by muscle
fibre necrosis and specific autoantibodies (anti-SRP
or anti-HMGCR) [3, 4]. Based on recent observa-
tions, it is becoming more likely that different
subsets of IIMs may have different molecular path-
ways that lead to different clinical phenotypes and
serotypes, and for which, different therapies may be
effective. Classification of IIMs and their different
subsets is essential to provide a better understand-
ing of the underlying disease processes. Progress in
the development of new classification criteria will be
discussed in this review together with the historical
background of currently used diagnostic and
classification criteria for IIMs.
Diagnostic and classification criteria: what is the difference?
Classification criteria are developed for use in
clinical research in order to make it possible to
compare different studies of the same disease.
Diagnostic criteria are intended for clinical use to
aid the clinician in the diagnostic workup of
patients. Sometimes classification criteria are
incorrectly used as a diagnostic tool leading to a
lack of a diagnosis in some patients. Diagnostic
criteria should be based on clinical and laboratory
manifestations that are present in patients early in
the disease course, and preferentially at presenta-
tion to the clinician. Ideally, diagnostic criteria
should be developed from a prospectively followed
cohort, with registration of early symptoms and
signs, and whether or not patients have the diag-
nosis in question will become clear with time.
Classification criteria on the other hand are based
on accumulated clinical manifestations and labo-
ratory tests that are or have been present in
patients who have developed a disease with high
certainty of diagnosis. This could be investigated in
40 2016 The Association for the Publication of the Journal of Internal Medicine
Journal of Internal Medicine, 2016, 280; 3951
Review Symposium: Diagnosis and classification of IIMs
a casecontrol study with retrospectively collected
data from cases with IIMs and from comparator
cases with disorders that mimic or have been con-
sidered to be IIM (non-IMM). As there is no gold
standard for IIM, the treating physician0 s diagnosis
of IIM could be used as the correct diagnosis. The
casecontrol design has been applied in the devel-
opment of new classification criteria, and the process
and preliminary results will be discussed below.
Diagnosis of adult IIMs
The diagnosis of IIMs is based on clinical symp-
toms such as subacute development of symmetri-
cal muscle weakness and muscle fatigue, most
prominent in proximal muscles, and signs such as
laboratory results supporting skeletal muscle
inflammation and muscle fibre degeneration and
repair (regeneration) (Table 1). The most easily
available test to demonstrate skeletal muscle
involvement is the measurement of serum levels
of muscle enzymes; the most frequently measured
enzyme is creatine phosphokinase (CK), but others
include lactate dehydrogenase (LD), aspartate
Table 1 Tools for diagnosis of idiopathic inflammatory
myopathies
Clinical history of muscle weakness or muscle fatigue
Clinical examination: muscle atrophy, weakness, skin
rash, joints, lungs, heart
History of previous medication
Family history of rheumatic or muscle disease
Muscle enzymes in serum (CK, LD, AST, ALT and
aldolase)
Muscle biopsy from affected skeletal muscle
MRI of affected muscle with T1 and T2 (STIR) images
Myositis-specific and myositis-associated
autoantibodies (see Table 2)
EMG
When myositis diagnosis has been confirmed, other
organ involvement should be searched for:
HRCT of lungs
Pulmonary function tests and diffusion capacity
ECG
Echocardiography
CK, creatine phosphokinase; LD, lactate dehydrogenase;
AST, aspartate transaminase; ALT, alanine transami-
nase; EMG, electromyogram; MRI, magnetic resonance
imaging; ECG, electrocardiography; HRCT, high-resolu-
tion computed tomography.
 I. E. Lundberg et al.
Table 2 Autoantibodies in idiopathic inflammatory
myopathies
Myositis-associated
Myositis-specific autoantibodies autoantibodies
Anti-aminoacyl-tRNA Anti-SSA/Ro
synthetases Anti-Ro52
Anti-Jo-1 Anti-Ro60
Anti-PL-7 Anti-La
Anti-PL-12 Anti-PM-Scl 75
Anti-EJ Anti-PM-Scl 100
Anti-OJ Anti-Ku
Anti-KS Anti-U1RNP
Anti-Zo Anti-cN-1A
Anti-YRS
Anti-Mi-2
Anti-SRP
Anti-TIF1-c
Anti-NXP-2
Anti-MDA5
Anti-SAE
Anti-HMGCR
Anti-FHL1
transaminase (AST), alanine transaminase (ALT)
and, less commonly, aldolase. Importantly, ele-
vated serum levels of muscle enzymes are not
specific for myositis as elevated levels could be
seen in many other myopathies and normal muscle
enzyme levels do not exclude myositis. Muscle
biopsy with histopathological evaluation of frozen
muscle tissue by an experienced muscle patholo-
gist is a very useful tool and is central to the
diagnostic workup of adult IIMs both to confirm
skeletal muscle inflammation and to exclude other
myopathies. A muscle biopsy is recommended by
experts as mandatory to classify IIM patients
without a typical DM skin rash. A muscle biopsy
is also important to subclassify patients (e.g. into
those with IBM or IMNM). Immunohistochemical
staining such as for major histocompatibility com-
plex class 1 (MHC-1), T cells and macrophages may
be helpful to confirm signs of inflammation. Of
note, a normal muscle biopsy does not exclude
IIMs as the inflammatory infiltrates can be
unevenly distributed. Magnetic resonance imaging
(MRI) with T1 and T2 (STIR images) of skeletal
muscle may be helpful to identify areas of muscle
inflammation by detection of skeletal muscle
oedema [5]. Sites of inflammation can be used to
target muscle biopsies [6]. Although the oedema in
skeletal muscles on MRI is not specific for myositis,
it is more commonly seen in myositis in compar-
ison with noninflammatory myopathies. MRI can
also detect muscle damage in the case of fat
replacement of muscle tissue or fibrosis.
Review Symposium: Diagnosis and classification of IIMs
Electromyography (EMG) is another tool to detect
myopathies, and some changes may also distin-
guish between necrosis and denervation, such as
the size, shape and recruitment pattern of the
motor unit potential, although there are no specific
EMG features for myositis.
Autoantibody testing has become an important
tool for diagnosis of IIMs and also to identify
subgroups of IIMs with different clinical pheno-
types and prognosis (Table 2). There are the so-
called myositis-associated antibodies such as
anti-Ro52, anti-Ro60, anti-La, anti-U1RNP, PM-
Scl and anti-Ku, which can be present in other
systemic autoimmune diseases including sys-
temic lupus erythematosus (SLE), Sj ogrens syn-
drome and systemic sclerosis (SSc). Although
these autoantibodies are not specific for myositis,
their presence may be helpful to distinguish an
inflammatory myopathy from a nonautoimmune
myopathy. There are also the so-called MSAs as
described in more detail by Betteridge and
McHugh [2]. These are almost exclusively present
in IIMs or in subgroups of the diseases. The most
common are anti-Jo-1 antibodies, which are pre-
sent in approximately 2025% of patients with
PM or DM. The anti-Jo-1 antibodies, targeting
histidyl-tRNA synthetase, are strongly associated
with distinct clinical manifestations: myositis,
interstitial lung disease (ILD), arthritis, fever,
Raynauds phenomenon and skin rash on the
hands termed mechanics hands. These features,
together known as the antisynthetase syndrome
(ASS), can also be present without clinical muscle
involvement. There are seven other antisynthetase
autoantibodies that can be seen in the ASS, some
of which are more frequently associated with ILD
and some with myositis (Table 2). These autoan-
tibodies all target antigens that are ubiquitously
expressed in the cytoplasm of all nucleated cells
with some variation in expression between differ-
ent organs, which may explain the link, for
example, between muscle and lung in the ASS
[7] A new MSA has been discovered that targets a
muscle-specific protein, four and a half limb
domain 1 (FHL1) [8]. This antibody was present
in approximately 25% of patients with PM, DM or
IBM with the main clinical features of severe
muscle atrophy and dysphagia but an absence of
lung or joint involvement, supporting the hypoth-
esis that different autoantibodies are associated
with distinct clinical phenotypes and that differ-
ent molecular pathways may predominate in
different subsets of myositis.
2016 The Association for the Publication of the Journal of Internal Medicine 41
Journal of Internal Medicine, 2016, 280; 3951
 I. E. Lundberg et al.
The gold standard method of assessment for MSAs
has been immunoprecipitation, but this technique
is not feasible for use in clinical practice. Several
different commercial tests have been developed
with varying specificity and sensitivity, and in the
clinical context, it is important to check the validity
of a commercial test to avoid over-interpretation of
such tests. A line blot assay with most of the MSAs
has been partly validated and can be used in
clinical practice [9]. Recently, anti-cytosolic 50 -
nucleotidase (CN)-1A antibody has been detected
in patients with IBM and rarely in the other
myositis subgroups [10, 11]. As this autoantibody
could be detected in other autoimmune disorders
such as Sjogrens syndrome and SLE, it should be
classified as a myositis-associated antibody. The
sensitivity and specificity of this antibody are
currently being investigated.
Once a diagnosis of IIM has been made, further
investigations are recommended to clarify extra-
muscular organ involvement; as such involve-
ment may affect management and prognosis. In
particular, the lungs and the gastrointestinal
tract are frequently involved. Therefore, investi-
gations of lung involvement with high-resolution
computed tomography (HRCT) and pulmonary
function tests are recommended even in patients
without clinical symptoms from the lungs. Like-
wise, radiography or video radiography of the
pharynx and oesophagus is recommended as part
of the diagnostic workup as swallowing problems
may lead to aspiration pneumonia and early
death. Heart involvement may also occur, and
ECG and echocardiogram are recommended to
screen for heart muscle involvement in cases with
IIM as heart involvement can be subclinical [12].
Adult patients with myositis, and especially those
with DM, have an increased risk of associated
malignant disease; this risk is less certain for
patients with PM and IBM. Therefore, screening
for malignancies in patients with DM is generally
recommended, particularly if patients have anti-
TIF-1 gamma antibodies and/or if they have a
poor response to conventional immunosuppres-
sive treatment. No specific malignancy is linked
to DM; thus, a general screening is recommended
based on sex and age, and with more careful
surveillance of elderly individuals.
History of the classification criteria for PM/DM
Over the past 45 years, investigators and clinicians
have struggled with how to best define and
42 2016 The Association for the Publication of the Journal of Internal Medicine
Journal of Internal Medicine, 2016, 280; 3951
Review Symposium: Diagnosis and classification of IIMs
separate the IIMs from other conditions with sim-
ilar clinical, laboratory and pathological features,
to result in a more homogeneous group of patients
for clinical and laboratory studies. Given the rarity
and heterogeneity of these disorders, however, the
different myositis classification criteria that have
been proposed by the specialists who evaluate and
study such patients have many limitations.
Because most criteria have been based on expert
opinion alone and because different studies have
used varying criteria, different populations with
possibly dissimilar risk factors and pathogeneses
have been studied. This has resulted in the inabil-
ity to compare epidemiological studies or trials of
different therapies or even of the same therapy.
Unfortunately, the lack of consistency amongst
classification criteria for IIMs may have resulted in
reduced interest by the pharmaceutical industry
and funding agencies in supporting myositis clin-
ical trials.
The major publications focusing on PM/DM clas-
sification criteria over the past 45 years are sum-
marized in Table 3. Some studies included IBM
criteria to allow for an overall classification of IIMs
(for a comprehensive review of the IBM criteria, see
article by Hilton-Jones and Brady in this issue of
Journal of Internal Medicine [13]). One of the first
major attempts to classify PM/DM was reported by
Medsger et al. [14]. Their criteria were based on
personal experience and emphasized muscle
weakness, muscle biopsy showing inflammation,
EMG abnormalities consistent with myopathy,
elevated blood activity of the enzymes associated
with myositis and clinical responses to corticos-
teroids. DeVere and Bradley [15] published their
classification criteria in a similar series of cases
defined by weakness, muscle pain and tenderness,
characteristic myopathic EMG changes, an inflam-
matory muscle biopsy and elevated serum CK
activity.
The seminal publications by Bohan and Peter [16],
advanced the field by providing the most compre-
hensive evaluation criteria for PM and DM, which
led to the classification criteria that have been used
most extensively during the last four decades
(Table 4). Key aspects of their criteria that distin-
guished them from prior attempts were as follows:
the requirement first to exclude all other forms of
myopathy; an approach to estimate the certainty of
diagnosis of both PM and DM by defining possible,
probable and definite disease criteria; the initial
inclusion of characteristic rashes to distinguish
 I. E. Lundberg et al. Review Symposium: Diagnosis and classification of IIMs

Table 3 Summary of the features of major proposed criteria for idiopathic inflammatory myopathies

Criteria proponents MW MPT MBx EMG ENZ CUT DEF MSA SCF References
Medsger et al. X X X X X [14]
DeVere & Bradley X X X X X [15]
Bohan & Peter X X X X X X [16]
Dalakas X X X X X X X [1]
Tanimoto et al. X X X X X X X X X [19]
Targoff et al. X X X X X X X [20]
Dalakas & Hohlfeld Xa X X X X Xb [23]
Van der Meulen et al. X X X X X [22]
Hoogendijk et al.c X Xd X X X X [24]
Oddis et al. X X X X X X X [25]

CUT, cutaneous features (V sign, shawl sign, Gottrons papules, heliotrope rash); DEF, disease definition (i.e. definite,
probable, possible); EMG, electromyography; ENZ, elevated muscle enzymes (i.e. creatine kinase, aldolase); MBx,
inflammation observed in muscle biopsy; MPT, muscle pain and tenderness; MSA, myositis-specific autoantibody (e.g.
Jo-1 for [1] and [19] but all available for [20]); MW, proximal muscle weakness; SCF, subclassification (i.e.
dermatomyositis, inclusion body myositis, polymyositis and cancer-associated, unspecified, isolated, overlap and
amyopathic forms of myositis).
a
Myopathic muscle weakness based on a number of exclusions and inclusions: affecting proximal more than distal
muscles; sparing eye and facial muscles; characterized by a subacute onset (weeks to months); rapid progression in
patients who have no family history of neuromuscular disease; no exposure to myotoxic drugs or toxins; no signs of
biochemical muscle disease; pattern distinct from inclusion body myositis.
b
Amyopathic dermatomyositis is a separate category.
c
Detailed inclusion and exclusion criteria.
d
Detailed pathological features.
Modified from [29].

DM; more detailed descriptions of each of the other
criteria; and definitions for five subgroups of PM/
DM, including juvenile, overlap and cancer-
associated forms of myositis. Despite the major
advances provided by these criteria and their
continued use to the present day, several limita-
tions have created difficulties in their interpreta-
tion, which is likely to have resulted in different
populations being examined in the various studies
that used these criteria. First, the Bohan and Peter
criteria resulted from a case series of 153 patients,
and data were developed at a single institution
based on clinical observations [17]. Secondly, they
did not provide clear instructions for ruling out all
other forms of myopathy, and IBM and many other
myopathies had not yet been identified. Thirdly, the
number of features needed to fulfil certain criteria
was not fully specified, and many of the features
proposed are observer dependent and nonspecific.
Importantly, the characteristic rashes of DM
were not specified, which resulted in extensive
controversy regarding which of the many rashes
that occurs in DM could be used for classification.
Finally, the sensitivity and specificity of these
criteria for differentiating DM/PM from a wide
variety of confounding dermatological or neuro-
muscular conditions were not determined.
However, a subsequent analysis suggested that
they had a sensitivity of 93% and a specificity
of 93% for distinguishing PM/DM from SSc and
SLE [18].
Based on personal observations, Dalakas proposed
a modification of the Bohan and Peter criteria for
PM/DM but also added criteria for IBM and iden-
tified important muscle biopsy features that could
distinguish between these disorders [1]. He also
suggested that definite disease required all the
criteria to be present and that probable disease
included all of the criteria except the muscle biopsy
features.
The next important publication relating to IIM
classification criteria was a multispecialty effort
in Japan [19]. A retrospective study by question-
naire was conducted in dermatology, neurology
and rheumatology departments throughout the
country. Many disease features that had been
proposed in prior criteria sets were assessed, but
new variables were also included that had not yet

2016 The Association for the Publication of the Journal of Internal Medicine 43
Journal of Internal Medicine, 2016, 280; 3951
 I. E. Lundberg et al.
Table 4 Bohan and Peter criteria for polymyositis and
dermatomyositis
First rule out all other forms of myopathy
1 Symmetrical weakness, usually progressive, of the
limb-girdle muscles
2 Muscle biopsy evidence of myositis
Necrosis of type I and type II muscle fibres,
phagocytosis, degeneration and regeneration of
myofibres with variation in myofibre size,
endomysial, perimysial, perivascular or interstitial
mononuclear cells
3 Elevation of serum levels of muscle-associated
enzymes
CK, aldolase, LD, transaminases (ALT/SGPT and
AST/SGOT)
4 Electromyographic triad of myopathy
(a) Short, small, low-amplitude polyphasic motor unit
potentials
(b) Fibrillation potentials, even at rest
(c) Bizarre high-frequency repetitive discharges
5 Characteristic rashes of dermatomyositis
Definite PM defined as all first four elements, probable PM
as three of first four and possible PM as two of first four;
definite DM defined as rash plus three other elements,
probable DM as rash plus two others and possible DM as
rash plus one other
ALT/SGPT, alanine transaminase/serum glutamic pyru-
vate transaminase; AST/SGOT, aspartate transaminase/
serum glutamic oxaloacetic transaminase; CK, creatine
kinase; LD, lactate dehydrogenase.
Modified from [16].
been assessed but were thought by the respon-
dents to be helpful. Consequently, the following
nine features were proposed as criteria for PM/DM:
(i) heliotrope rash, Gottrons sign or linear extensor
erythema; (ii) proximal weakness of the upper or
lower extremity and trunk; (iii) elevation of CK or
aldolase levels; (iv) muscle pain on grasping or
spontaneous muscle pain; (v) the EMG triad of
myopathy; (vi) presence of anti-Jo-1 autoantibod-
ies; (vii) nondestructive arthritis or arthralgias;
(viii) signs of systemic inflammation (fever >37 C
at the axilla, elevated C-reactive protein or ery-
throcyte sedimentation rate); and (ix) muscle
biopsy evidence of myositis (inflammatory infiltrate
with degeneration or necrosis of muscle, active
phagocytosis, central nuclei or active regenera-
tion). With these criteria, definite PM was defined
as any four of the nine features without rash, with
a sensitivity of 99% and a specificity against all
other diseases of 95%. Definite DM was defined as
rash plus four other features, with a sensitivity of
94% and specificity against SLE and SSc skin
44 2016 The Association for the Publication of the Journal of Internal Medicine
Journal of Internal Medicine, 2016, 280; 3951
Review Symposium: Diagnosis and classification of IIMs
lesions of 90%. Although these criteria had the
advantage of having been developed by a national
multidisciplinary team, a limitation was that the
evaluators had varying degrees of experience and
training in evaluating myositis, and so the consis-
tency of the evaluation and completeness of the
questionnaires were unclear. Additionally, there
were limited numbers and types of alternative
disorders for comparison, and IBM, cancer-asso-
ciated myositis and juvenile myositis were not part
of the evaluation process. Finally, this study was
based on questionnaires and retrospective chart
reviews, PM was diagnosed only by rheumatolo-
gists and neurologists, and DM was diagnosed only
by dermatologists and rheumatologists, and
apparently Gottrons papules were not assessed
at all.
Targoff and colleagues [20] took a different
approach and first considered whether a patient
met defined criteria for IIMs and, if so, then
considered whether the patient met subclassifica-
tion criteria for the IIM phenotypes of PM, DM or
IBM. Their intentions were to modify the Bohan
and Peter criteria by adding specificity where
needed and to include, for the first time, a role for
all the known MSAs. They reiterated that all non-
IIMs should be carefully excluded using state-of-
the-art methods, realizing that these will change
over time as technologies and understanding of the
disorders evolve. The six primary classification
criteria for IIMs that they proposed included the
five previously identified by Bohan and Peter (but
with DM for the first time specifically defined by
heliotrope rash, Gottrons papules or Gottrons
sign; see Fig. 1) plus any of the MSAs then
commercially available with a validated assay
[e.g. autoantibodies against aminoacyl-tRNA syn-
thetases, chromodomain helicase DNA-binding
protein 3 (anti-Mi-2 autoantibodies) or the signal
recognition particle (anti-SRP autoantibodies)].
Using this approach, they defined definite, proba-
ble and possible IIMs as any four, three and two of
the six criteria, respectively. Regarding the sub-
classification criteria, DM was defined by the
presence of any one of the above-mentioned rashes
and PM by the absence of these rashes. IBM was
defined by also meeting the criteria of Griggs et al.
[21], and juvenile myositis was defined by age at
onset <18 years.
In a retrospective evaluation, van der Meulen
et al. [22] combined prior criteria with a critical
pathology focus and concluded that only a small
 I. E. Lundberg et al.
(a)
(b)
(c)
Fig. 1 Pathognomonic rashes of dermatomyositis: Got-
trons papules, red or violet papules occurring over the
knuckles, the interphalangeal joints and other extensor
surfaces (a); Gottrons sign, red or violet macules occurring
over the knuckles and sometimes the interphalangeal
joints in patients with dermatomyositis (b); and heliotrope
rash, red or violet eruption over the upper eyelids and
sometimes extending around the eye (c). Reproduced from
[30] with permission.
number of patients suspected of having PM actu-
ally met their criteria, which included subacute
onset (<1 year); symmetrical, proximal more than
Review Symposium: Diagnosis and classification of IIMs
distal muscle weakness, or muscle soreness; CK
levels more than twice the upper limit of normal;
and mononuclear cells (MNCs) surrounding and
ideally invading non-necrotic endomysial myofi-
bres. Definite DM required the typical skin rash
or perifascicular atrophy on muscle biopsy. Of
interest, they found that 23% of the patients
studied did not meet these criteria, and so they
created a new category of unspecified myositis.
These subjects had a perimysial or perivascular
MNC infiltrate but without endomysial MNCs
surrounding and invading non-necrotic fibres or
perifascicular atrophy or rash. Possible myositis,
defined as CK levels more than twice the upper
limit of normal and necrotizing myopathy with few
or no MNCs on biopsy, was identified in 18% of
their patients. They concluded that PM is over-
diagnosed and that other categories of myositis
should be established. A limitation of their criteria
is that a new, large category of unspecified
myositis was established for which there was no
information on how to assess or treat patients in
this subgroup.
In 2003, Dalakas and Hohlfeld [23] updated the
Bohan and Peter criteria by adding more patholog-
ical focus and details, as well as including criteria
for amyopathic DM. Their muscle biopsy criteria
were as follows: for definite PM, primary inflam-
mation with the CD8/MHC-1 complex and no
vacuoles; for probable PM, ubiquitous MHC-1
expression but no CD8+ cell infiltrates or vacuoles;
for definite DM, perifascicular, perimysial or
perivascular infiltrates, perifascicular atrophy
and rash; for probable DM, perifascicular, per-
imysial or perivascular infiltrates, perifascicular
atrophy but no rash; and for amyopathic DM, a
rash but biopsy findings nonspecific or diagnostic
for DM, and no weakness present.
As part of the European Neuromuscular Centre
(ENMC) and Muscle Study Group (MSG) work-
shops, a group of myologists proposed criteria for
IIMs, excluding IBM, based on expert opinion [24].
These criteria elements are summarized in Table 5,
and how to apply them to each category of myositis
is shown in Table 6 [24]. These criteria are unique
in that they include a detailed list of inclusion and
exclusion elements to be applied to clinical, labo-
ratory (including MRI and MSAs) and pathological
features.
A comparison of performance of some of the main
criteria used to classify IIMs including the ENMC
2016 The Association for the Publication of the Journal of Internal Medicine 45
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 I. E. Lundberg et al.
Table 5 Elements of the classification criteria for idiopathic
inflammatory myopathies (except IBM) approved by the
Myositis Study Group and the 119th European
Neuromuscular Centre workshop
1 Clinical criteria
Inclusion criteria
(a) Onset usually over 18 years (postpuberty), onset
may be in childhood in DM and nonspecific
myositis
(b) Subacute or insidious onset
(c) Pattern of weakness: symmetrical
Proximal > distal, neck flexor > neck extensor
(d) Rash typical of DM: heliotrope (purple) periorbital
oedema; violaceous papules (Gottrons papules) or
macules (Gottrons sign), scaly if chronic, at
metacarpophalangeal and interphalangeal joints
and other bony prominences; erythema of chest
and neck (V sign) and upper back (shawl sign)
Exclusion criteria
(a) Clinical features of IBM (see Griggs et al. [21]:
asymmetrical weakness, wrist/finger flexors same
or worse than deltoids; knee extensors and/or
ankle dorsiflexors same or worse than hip flexors)
(b) Ocular weakness, isolated dysarthria, neck
extensor > neck flexor weakness
(c) Toxic myopathy (e.g. recent exposure to myotoxic
drugs), active endocrinopathy (hyper- or
hypothyroid, hyperparathyroid), amyloidosis,
family history of muscular dystrophy or proximal
motor neuropathies (e.g. SMA)
2 Elevated serum creatine kinase level
3 Other laboratory criteria
(a) Electromyography
Inclusion criteria
(I) Increased insertional and spontaneous activity in
the form of fibrillation potentials, positive sharp
waves or complex repetitive discharges
(II) Morphometric analysis reveals the presence of
short duration, small amplitude, polyphasic
MUAPs
Exclusion criteria
(I) Myotonic discharges that would suggest proximal
myotonic dystrophy or other channelopathy
(II) Morphometric analysis reveals predominantly
long-duration, large-amplitude MUAPs
(III) Decreased recruitment pattern of MUAPs
(b) MRI: diffuse or patchy increased signal (oedema)
within muscle tissue on STIR images
(c) Myositis-specific antibodies detected in serum
4 Muscle biopsy inclusion and exclusion criteria
(a) Endomysial inflammatory cell infiltrate (T cells)
surrounding and invading non-necrotic muscle
fibres
(b) Endomysial CD8+ T cells surrounding but not
definitely invading non-necrotic muscle fibres, or
ubiquitous MHC-1 expression
(c) Perifascicular atrophy
(d) MAC depositions on small blood vessels, reduced
capillary density, tubuloreticular inclusions in
endothelial cells on EM or MHC-1 expression of
perifascicular fibres
(e) Perivascular, perimysial inflammatory cell
infiltrate
46 2016 The Association for the Publication of the Journal of Internal Medicine
Journal of Internal Medicine, 2016, 280; 3951
Review Symposium: Diagnosis and classification of IIMs
Table 5 (Continued )
(f) Scattered endomysial CD8+ T-cell infiltrate that
does not clearly surround or invade muscle fibres
(g) Many necrotic muscle fibres as the predominant
abnormal histological feature. Inflammatory cells
are sparse or only slightly perivascular; perimysial
infiltrate is not evident. MAC deposition on small
blood vessels or pipestem capillaries on EM may be
seen, but tubuloreticular inclusions in endothelial
cells are uncommon or not evident.
(h) Rimmed vacuoles, ragged red fibres, cytochrome C
oxidase-negative fibres that would suggest IBM
(i) MAC deposition on the sarcolemma of non-necrotic
fibres and other indications of muscular
dystrophies with immunopathology
DM, dermatomyositis; EM, electromyography; IBM, inclu-
sion body myositis; MAC, membrane attack complex;
MHC, major histocompatibility complex; MRI, magnetic
resonance imaging; MUAP, motor unit action potential;
SMA, spinal muscular atrophy; STIR, short-tau inversion
recovery.
Modified from [24].
criteria was performed in a small single-centre
cohort [25]. The study confirmed the limitations of
existing criteria and emphasized the need to
develop new and improved classification criteria
for IIMs.
Oddis et al. [26], as part of the efforts of the
International Myositis Assessment and Clinical
Study Group (IMACS) to develop multidisciplinary
consensus for conducting and reporting myositis
trials, also suggested modifications to the Bohan
and Peter criteria that defined Gottrons papules or
heliotrope rash as the required rashes for DM and
suggested that a muscle biopsy consistent with PM
was also a necessary criterion for PM.
The IMCCP criteria: initiation, methodology and results
The international interest group IMACS was estab-
lished in 1999 to develop outcome measures for
use in clinical trials and to facilitate collaborative
myositis research [27]. Through this collaborative
network, it was apparent that there was a strong
need to develop new classification criteria where
novel technologies such as immunohistochemical
staining of muscle biopsies, MRI of skeletal mus-
cles and new MSAs would be taken into account
and could be used for clinical and molecular
studies of myositis. Thus, a group of experts in
myositis and epidemiology met in 2004 to initiate a
project to define new classification criteria for
 I. E. Lundberg et al.
myositis with high sensitivity and specificity, start-
ing what resulted in a long-term project that is now
coming to the end.
It was clear that the myositis criteria project
needed to be multidisciplinary and international,
with the aim, if possible, of developing common
criteria for paediatric and adult myositis patients.
This project, the International Myositis Classifica-
tion Criteria Project (IMCCP), included an interna-
tional steering group representing adult and
paediatric rheumatology, neurology, dermatology
and epidemiology, with representatives from the
USA and Europe. Endorsement was sought from
major scientific organizations including the Amer-
ican College of Rheumatology (ACR), European
League Against Rheumatism (EULAR), the Muscle
Study Group, IMACS, Childhood Arthritis and
Rheumatology Research Alliance (CARRA), Paedi-
atric Rheumatology European Society (PReS) net-
work for juvenile DM (JDM) and the Paediatric
Rheumatology International Trials Organisation
(PRINTO). The major aims of the project were to
develop new classification criteria that should
distinguish patients with IIMs from those with
non-IIM and, furthermore, that the criteria should
identify subgroups of IIMs. It was decided that the
process should be data-driven and include both
paediatric and adult cases and comparator cases
with non-IIM. Initially, variables to be collected
were identified based on previously published
criteria for IIMs including IBM and additional
variables were added based on expert opinion with
contributions from myologists within the large
IMACS network. The decision on which variables
to be included was based on discussions with
project participants using nominal group tech-
nique and was finally made by the IMCCP steering
group. All variables to be included in the project
were then operationally defined. Inclusion criteria
for cases and comparators were defined as: (i)
diagnosis for at least 6 months prior to study
inclusion; (ii) physician certainty of diagnosis
either known IIMs or, as comparators, known non-
IIM cases where myositis was considered in the
initial differential diagnosis; and (iii) patients with
the most complete data were prioritized to acquire
the most complete data in a consistent manner. A
maximum of 40 cases with an equal number of
comparators was collected from each centre; for
paediatric cases, a minimum of five cases with
comparators was required per centre. As there is
no gold standard for diagnosis of IIMs, it was
Review Symposium: Diagnosis and classification of IIMs
decided that the diagnosis of the expert clinician
who enrolled the patients should be used.
An electronic database was constructed that
included the basic demographic characteristics of
patients and a total of 93 variables. As a result of the
contribution of myositis experts from 47 centres
worldwide (23 European, 17 North American, one
South American and six Asian sites), data were
collected from 976 patients with IIM and 624
comparators (adults and children). Of note, 20% of
the cases and comparators were of Asian origin. The
patients represented the major current clinical
subgroups of IIMs: JDM, PM, DM, IBM, amyopathic
DM, hypomyopathic DM, IMNM and juvenile PM.
The comparator group consisted of patients with a
broad spectrum of conditions that can mimic IIMs
from rheumatology, paediatric rheumatology, neu-
rology and dermatology clinics.
The analytical phase was initiated after a long
period to collect data from the cases and com-
parators. Because of the large amount of available
data, Professor M Bottai (Karolinska Institutet,
Stockholm, Sweden), a biostatistician with expe-
rience from similar work, was recruited to the
IMCCP project. As a first step, we wanted to
identify variables that distinguish IIMs from non-
IIMs with a high sensitivity and specificity. Three
classification techniques were explored: (i) a tra-
ditional sum-of-items model, in which patients
were classified as cases if they had a specified
number of items from a set of items similar to, for
example, the Bohan and Peter criteria; (ii) a
probability score model; and (iii) a classification
tree. The ensuing candidate criteria were exam-
ined with respect to statistical performance and
clinical relevance. Sixteen variables, which had
the best capacity to discriminate IIMs from non-
IIMs using a probability model, were identified
(Table 7) [28]. Furthermore, the 16 variables were
found to have different importance and were
therefore given a different weight or score. In
addition, the investigators wanted to investigate
the possibility of developing a set of criteria
without requiring muscle biopsies, particularly in
the context of children with a typical DM skin
rash. Therefore, two versions of the criteria were
tested, one with and one without muscle biopsies.
The best statistical performance with a balance
between sensitivity and specificity was found with
a 55% probability cut-off for both models. The
probability model with the different weights of the
2016 The Association for the Publication of the Journal of Internal Medicine 47
Journal of Internal Medicine, 2016, 280; 3951
 I. E. Lundberg et al.
variables offers flexibility in the number of vari-
ables that need to be tested. A probability of 55%
is recommended for research studies such as
register-based or natural history studies where
the sensitivity is important, whereas in clinical
trials a high specificity is recommended, that is a
high probability score. Thus, the experts in the
steering committee recommended a high proba-
bility of 90% for use in clinical trials. This is
comparable to defining different levels of sensitiv-
ity and specificity, using 55% probability for a
probable diagnosis and 90% probability for a
definite diagnosis of IIMs. Of note, the steering
committee recommended that muscle biopsy vari-
ables should be included for patients without skin
rash typical of DM. The new criteria were com-
pared to previously developed diagnostic or
classification criteria and were superior with
regard to sensitivity and specificity to all except
the Targoff criteria [20] which performed equally
well. However, the Targoff criteria require the
testing of more variables, for example both muscle
biopsy and EMG are required, so they are less
flexible. Moreover, the Targoff criteria variables
are not well defined and do not include all subsets
of IIMs.
After having classified a patient as having an IIM, a
classification tree was developed to identify the
subclass of IIM. Through the subclassification tree,
JDM, adult DM, clinically amyopathic DM, IBM
and PM could be identified. However, there were
too few patients in the cohort to identify the newly
defined subgroup of IMNM; thus, these patients
were included within the PM subgroup. Likewise,
there were too few cases to identify hypomyopathic
DM, and there were not enough non-JDM IIM cases
to identify variables for other subgroups of juvenile
myositis. By extrapolation from the adult IIMs in
the classification tree, a subgroup of juvenile
myositis other than JDM was suggested. The new
criteria with a probability score and subclassifica-
tion tree have been internally validated using a
bootstrap method and tested for sensitivity in
external cohorts with myositis cases with very good
results. Further validation will be needed in exter-
nal cohorts.
The new classification criteria are currently under
review by the ACR and EULAR criteria subcommit-
tees, as endorsement is being sought from these
organizations; thereafter, reports of these criteria
will be submitted for publication.
48 2016 The Association for the Publication of the Journal of Internal Medicine
Journal of Internal Medicine, 2016, 280; 3951
Review Symposium: Diagnosis and classification of IIMs
A webcalculator has been designed to support the
investigators in calculating the probability score. It
is available at http://www.imm.ki.se/biostatis-
tics/calculators/iim and maintained by the Unit
of Biostatistics, Karolinska Institutet. Using the
webcalculator, variables can be entered and the
calculator will generate a probability score for the
classification of myositis that will become more
accurate as further patient information is added.
The webcalculator will also provide a subgroup of
myositis if sufficient information is available, and
will be available as an app for smartphones. The
uploaded data can be stored in Excel sheets.
Summary
The new myositis classification criteria are data-
driven, based on patients and comparators from
many centres worldwide representing different
ethnicities. Thus, the patients should be repre-
sentative of various clinical phenotypes of myosi-
tis. The variables in the final version of the criteria
are easily available, clinically relevant and col-
lected in the routine diagnostic workup of patients
in most centres. Importantly, these are classifica-
tion criteria that were developed based on infor-
mation from patients with established disease,
and thus, they are not recommended for diagno-
sis. The probability model that has been devel-
oped is flexible, meaning that all variables do not
need to be tested to reach a high probability.
However, in patients without typical DM skin
rash, a muscle biopsy is considered necessary to
both confirm signs of inflammation and exclude
other differential diagnoses. Furthermore, these
criteria apply to cases with a suspicion of myositis
when no better explanation for the symptoms
exists. Surprisingly, only one of the MSAs was
included in the new classification criteria. This
can be explained by the long process of collection
of patient information, as most of the new MSAs
were not available as routine tests when data were
initially collected. Thus, revision of the criteria
within a few years is recommended, by which time
more consistent serological information should be
available. EMG and MRI of muscles were not
included in the new criteria, reflecting the fact
that they are not often used in clinical practice
amongst the 47 centres that contributed with
cases for this study; interestingly, we could clas-
sify patients correctly with a high sensitivity and
specificity based on the variables in the newly
proposed classification criteria for myositis.
 I. E. Lundberg et al.
Table 6 Scoring of the classification criteria elements for
idiopathic inflammatory myopathies (except IBM) to define
different myositis phenotypes as approved by the Myositis
Study Group and the European Neuromuscular Centre
workshop
Definite polymyositis
1 All clinical criteria with the exception of rash
2 Elevated serum CK
3 Muscle biopsy criteria include a and exclude c, d, h, ia
Probable polymyositis
1 All clinical criteria with the exception of rash
2 Elevated serum CK
3 Other laboratory criteria (1 of 3)
4 Muscle biopsy criteria include b and exclude c, d, g, h, i
Definite dermatomyositis
1 All clinical criteria
2 Muscle biopsy criteria include c
Probable dermatomyositis
1 All clinical criteria
2 Muscle biopsy criteria include d or e, or elevated serum
CK, or other laboratory criteria (1 of 3)
Amyopathic dermatomyositis
1 Rash typical of DM: heliotrope, periorbital oedema,
Gottrons papules or sign, V sign, shawl sign, holster
sign
2 Skin biopsy demonstrates a reduced capillary density,
deposition of MAC on small blood vessels along the
dermalepidermal junction and variable keratinocyte
decoration for MAC
3 No objective weakness
4 Normal serum CK
5 Normal EMG
6 Muscle biopsy, if done, does not reveal features com-
patible with definite or probable DM
Possible dermatomyositis sine dermatitis
1 All clinical criteria with the exception of rash
2 Elevated serum CK
3 Other laboratory criteria (1 of 3)
4 Muscle biopsy criteria include c or d
Nonspecific myositis
1 All clinical criteria with the exception of rash
2 Elevated serum CK
3 Other laboratory criteria (1 of 3)
4 Muscle biopsy criteria include e or f and exclude all
others
Immune-mediated necrotizing myopathy
1 All clinical criteria with the exception of rash
2 Elevated serum CK
3 Other laboratory criteria (1 of 3)
4 Muscle biopsy criteria include g and exclude all others
a
Letters refer to those elements in Table 3.
CK, creatine kinase; DM, dermatomyositis; EMG, elec-
tromyography; IBM, inclusion body myositis; MAC, mem-
brane attack complex.
Modified from [24].
Review Symposium: Diagnosis and classification of IIMs
Table 7 Variables used in the development of new
classification criteria for idiopathic inflammatory
myopathies
Variable
Age of onset of first symptom assumed to be related to
the disease 18 years and <40 years
Age of onset of first symptom assumed to be related to
the disease 40 years
Objective symmetrical weakness, usually progressive, of
the proximal upper extremities
Objective symmetrical weakness, usually progressive, of
the proximal lower extremities
Neck flexors are relatively weaker than neck extensors
In the legs, proximal muscles are relatively weaker than
distal muscles
Heliotrope rash
Gottron0 s papules
Gottrons sign
Dysphagia or oesophageal dysmotility
Anti-Jo-1 (anti-histidyl-tRNA synthetase) autoantibody
present
Elevated serum levelsa of creatine kinase or lactate
dehydrogenase or aspartate aminotransferase or
alanine aminotransferase
Muscle biopsy features presence of:
Endomysial infiltration of mononuclear cells
surrounding, but not invading, myofibres
Perimysial and/or perivascular infiltration of
mononuclear cells
Perifascicular atrophy
Rimmed vacuoles
a
Serum levels above the upper limit of normal.
From [28].
In summary, many sets of diagnostic and classifi-
cation criteria for different forms of IIMs have been
proposed over five decades, each with specific
advantages and disadvantages. Most have been
based on clinical impressions rather than data
analyses, and none has been fully tested for
sensitivity and specificity against all the appropri-
ate disease confounders in adequately powered
studies [28]. Because of these limitations as well as
recent disagreements amongst rheumatologists,
neurologists, dermatologists and others regarding
the appropriate use of varying criteria for
2016 The Association for the Publication of the Journal of Internal Medicine 49
Journal of Internal Medicine, 2016, 280; 3951
 I. E. Lundberg et al.
classification of IIMs that would create difficulties
in comparing studies and clinical trials in the
future, many myologists have concluded that large,
multicentre and multispecialty studies, including
comparisons of IIMs with many confounding con-
ditions, are needed to define and validate new IIM
classification criteria. The result of this multidisci-
plinary effort is a new set of classification criteria
that have been partly validated with good results.
Further validation in external cohorts with com-
parator cases is needed.
Conflicts of interest statement
No conflicts of interest to declare.
Acknowledgements
This research was supported in part by the Intra-
mural Research Program of the NIH, National
Institute of Environmental Health Sciences. We
would like to thank Lisa Maroski for editorial
assistance, and Drs. Michael Ward and Andrew
Mammen for critical comments on the manuscript.
We also want to thank all our collaborators who
have contributed to the IMCCP.
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