Clinical manifestations of dermatomyositis and polymyositis in adults

Official reprint from UpToDate® www.uptodate.com

©2018 UpToDate®

Clinical manifestations of dermatomyositis and polymyositis in adults

Authors: Marc L Miller, MD, Ruth Ann Vleugels, MD, MPH
Section Editors: Ira N Targoff, MD, Jeremy M Shefner, MD, PhD, Jeffrey Callen, MD, FACP, FAAD
Deputy Editors: Monica Ramirez Curtis, MD, MPH, Abena O Ofori, MD

Contributor Disclosures

All topics are updated as new evidence becomes available and our peer review process is complete.
Literature review current through: Apr 2018. | This topic last updated: Jan 30, 2017.

INTRODUCTION — Dermatomyositis (DM) and polymyositis (PM) are idiopathic inflammatory myopathies,
characterized by the shared features of proximal skeletal muscle weakness and by evidence of muscle
inflammation [1-5]. DM, unlike PM, is associated with a variety of characteristic skin manifestations. A form of
DM termed amyopathic DM (ADM, historically termed "dermatomyositis sine myositis") is a condition in
which patients have characteristic skin findings of DM without weakness or abnormal muscle enzymes.

The clinical and serologic features of DM and PM vary among affected individuals and populations,
depending upon immunogenetic and possibly other genetic factors [6,7]. The immune mechanisms and
anatomic focus of injury within the muscle tissue in PM and DM appear distinct. The other major type of
idiopathic inflammatory myopathy is inclusion body myositis.

The clinical and laboratory manifestations of DM and PM in adults will be reviewed here. The diagnosis,
electrophysiologic and pathologic findings on diagnostic testing, differential diagnosis, and treatment of
these diseases and of the related disorders that occur in children (known as juvenile DM and PM); the
pathogenesis of inflammatory myopathies in adults and of juvenile DM and PM; the risk for malignancy in
patients with DM and PM; and the clinical manifestations, diagnosis, and treatment of inclusion body
myositis are discussed separately.

● (See "Diagnosis and differential diagnosis of dermatomyositis and polymyositis in adults".)

● (See "Initial treatment of dermatomyositis and polymyositis in adults" and "Treatment of recurrent and
resistant dermatomyositis and polymyositis in adults" and "Juvenile dermatomyositis and polymyositis:
Treatment, complications, and prognosis".)

● (See "Initial management of cutaneous dermatomyositis in adults" and "Management of refractory

cutaneous dermatomyositis in adults".)

● (See "Pathogenesis of inflammatory myopathies" and "Juvenile dermatomyositis and polymyositis:

Epidemiology, pathogenesis, and clinical manifestations".)

● (See "Malignancy in dermatomyositis and polymyositis".)

● (See "Clinical manifestations and diagnosis of inclusion body myositis" and "Management of inclusion
body myositis".)

EPIDEMIOLOGY — The combined incidence of dermatomyositis (DM) and polymyositis (PM) has been
estimated at 2 per 100,000 annually in the general population [8]. There is a female to male predominance of
- Page 1 of 18 -
Clinical manifestations of dermatomyositis and polymyositis in adults
about two to one. The peak incidence in adults occurs between the ages of 40 and 50, but individuals of any
age may be affected [9,10]. Estimates of prevalence range from 5 to 22 per 100,000 [11,12]. The estimated
annual incidence of the amyopathic subset of DM in the residents of Olmstead County in Minnesota was 0.2
per 100,000 persons in a study in which the annual incidence of DM of all types was approximately 1 per
100,000 [13].

CLINICAL MANIFESTATIONS — Dermatomyositis (DM) and polymyositis (PM) are both multisystem
disorders with a wide variety of clinical manifestations [9,10]. Most patients exhibit proximal skeletal muscle
weakness. Several characteristic skin eruptions are typical of DM, and a subset of patients with typical
cutaneous manifestations exhibit amyopathic DM (ADM), lacking evidence of muscle disease.

Interstitial pulmonary disease, dysphagia, and polyarthritis are also common in DM and PM, along with
constitutional symptoms; Raynaud phenomenon is present in some patients. Features that overlap with other
systemic rheumatic diseases, such as systemic lupus erythematosus (SLE) and systemic sclerosis (SSc), may
also be present. The risk of malignancy may be increased, particularly in patients with DM. Each of the major
clinical features is described in the sections below.

Muscle weakness — Muscle weakness is the most common feature of DM and PM; over 90 percent of
patients with PM present with muscle weakness [9]. However, cutaneous manifestations often precede or
accompany weakness, which is found at presentation in only 50 to 60 percent of patients with DM [9,10].
Typically mild myalgias and muscle tenderness occur in 25 to 50 percent of cases. (See 'Skin findings'
below.)

The distribution of weakness is characteristically symmetric and proximal in both PM and DM. Affected
muscles typically include the deltoids and the hip flexors. Weakness of the neck flexors is also common.
Distal muscle weakness, if present, tends to be mild and usually does not cause significant functional
impairment. Rarely, patients present with focal myositis that usually but not always progresses to the typical
generalized form over time [14]. (See "Approach to the patient with muscle weakness".)

Patients usually report a history of the insidious or subacute development of the muscle weakness, with
gradual worsening over a period of several months before medical attention is sought. However, an acute
onset of weakness is occasionally reported. Patients may describe increasing difficulty climbing stairs,
getting up from a chair, carrying heavy groceries, or picking up their children due to the proximal muscle
involvement. They may notice joint pain and swelling, if present, and they occasionally mistakenly ascribe
weakness to the joint involvement. Pain is mild, if present, and stiffness is not a prominent complaint. (See
'Antisynthetase syndrome' below and 'Overlap syndromes' below.)

Muscle atrophy is generally not seen in early cases, even in patients with marked weakness, but it may occur
in severe, longstanding disease.

Skin findings — Several distinct cutaneous eruptions, which are generally evident at the time of clinical
presentation, occur in DM but not in PM [15,16]. Other skin changes may occur in patients with PM and in
patients with DM and are not specific to either disorder. Dermatologic manifestations may be prominent but
can be quite subtle in some patients.

Characteristic dermatomyositis findings — Gottron's papules and the heliotrope eruption are the
hallmark and likely pathognomonic features of DM. Gottron's sign, photodistributed erythema, poikiloderma,
nailfold changes, scalp involvement, and calcinosis cutis are also characteristic and useful in distinguishing
- Page 2 of 18 -
Clinical manifestations of dermatomyositis and polymyositis in adults
DM from PM.

● Gottron's papules – Gottron's papules are erythematous to violaceous papules that occur
symmetrically over the extensor (dorsal) aspects of the metacarpophalangeal (MCP) and interphalangeal
(IP) joints (picture 1A-C). In addition, these lesions may involve the skin between the MCP and IP joints,
particularly when the eruption is prominent. Gottron's papules often have associated scale and may
ulcerate. When scaling is present, the lesions may mimic psoriasis or lichen planus.

● Gottron's sign – Definitions used for Gottron's sign have varied in the literature. We define Gottron's
sign as the presence of erythematous to violaceous macules, patches, or papules on the extensor
surfaces of joints in sites other than the hands, particularly the elbows, knees, or ankles. By contrast,
some authors have used the term Gottron's papules to refer to papules in these areas, reserving
Gottron's sign for macular or patch-like lesions (picture 2) [17].

● Heliotrope eruption – The heliotrope eruption is an erythematous to violaceous eruption on the upper
eyelids, sometimes accompanied by eyelid edema, which, at times, may be quite marked (picture 3A-D).

● Facial erythema – Patients may have midfacial erythema that can mimic the malar erythema seen in
SLE (picture 3A, 3E). In contrast to those with SLE, patients with DM will often have involvement of the
nasolabial fold, which can be helpful in distinguishing these two photosensitive midfacial eruptions.

● Photodistributed poikiloderma (including the shawl and V signs) – Poikiloderma refers to skin that
demonstrates both hyperpigmentation and hypopigmentation, as well as telangiectasias and epidermal
atrophy. In DM, patients may demonstrate poikiloderma in any photo-exposed site; however, classic
areas of involvement are the upper back (shawl sign) (picture 4A-B) and the V of the neck and upper
chest. The poikiloderma in DM often presents with a violaceous hue. Early in the course of cutaneous
disease, these areas may demonstrate only erythema rather than well-developed poikiloderma (picture
5). The erythema may be macular (nonpalpable) or papular. In rare patients, these lesions become
thickened and resemble papular mucinosis. The cutaneous eruption of DM is often associated with
significant pruritus, which may assist in distinguishing its photo-exacerbated eruption from that of lupus
erythematosus (LE).

● Holster sign – Patients with DM may also have poikiloderma on the lateral aspects of the thighs,
referred to as the "Holster sign" (picture 6A-B). It is unclear why this cutaneous manifestation occurs on
this classically photo-protected site.

● Generalized erythroderma – In rare patients, erythroderma may occur, which involves extensive
cutaneous surface area, including areas that are less exposed to ultraviolet light.

● Periungual abnormalities – The capillary nail beds in DM may be erythematous and may show vascular
changes similar to those observed in other systemic rheumatic diseases (eg, scleroderma and SLE).
Abnormal capillary nail bed loops may be evident, with alternating areas of dilatation and dropout and
with periungual erythema (picture 7A-B). In addition, cuticular overgrowth, sometimes termed "ragged
cuticles," is characteristic and may be associated with hemorrhagic infarcts within the hypertrophic area
(picture 7A). The degree of cuticular involvement is thought to reflect ongoing cutaneous disease activity,
representing active vasculopathy [18].

● Psoriasiform changes in scalp – Changes in the scalp resembling seborrheic dermatitis or psoriasis

- Page 3 of 18 -
Clinical manifestations of dermatomyositis and polymyositis in adults

occur in a high percentage of patients with DM (picture 8). The scalp involvement in DM is diffuse, often
associated with poikilodermatous changes and with prominent scaling. Scalp involvement may result in
severe burning, pruritus, and/or sleep disturbance. In addition, severe pruritus may occur in patients
without visible disease.

● Calcinosis cutis – The deposition of calcium within the skin, a finding known as calcinosis cutis, occurs
commonly in juvenile DM. It is infrequent in adult DM. In children, calcinosis has been associated with a
delay in treatment with glucocorticoids and/or immunosuppressive therapy. Calcinosis cutis, which is
known to be very challenging to treat, may be seen in a variety of conditions, including SSc, particularly
limited cutaneous SSc; SLE (rarely); and overlap connective tissue disorders. It may be more common in
patients with DM with the anti-p140/anti-MJ autoantibody [19,20]. (See 'Other myositis-specific
autoantibodies' below.)

Skin findings in antisynthetase syndrome — Patients with either DM or PM (classically those with the
antisynthetase syndrome) may have "mechanic's hands," which present with hyperkeratotic, fissured skin on
the palmar and lateral aspects of the fingers. Occasionally, these changes result in irregular, dirty-appearing
horizontal lines that resemble those of a manual laborer (picture 9) [21]. (See 'Antisynthetase syndrome'
below.)

Skin findings in MDA-5-associated dermatomyositis — Patients with melanoma differentiation-

associated gene 5 (MDA-5) antibodies are now known to have a characteristic phenotype with classic
findings that include cutaneous ulceration involving the Gottron's papules, elbows, digital pulp, and nailfolds;
erythematous, painful palmar macules and papules; alopecia; oral ulcers; arthritis; and amyopathic disease
[22]. In addition, patients with MDA-5 antibodies are at a higher risk for interstitial lung disease (ILD),
including a rapidly progressive presentation with high mortality. Recognizing the cutaneous features are thus
imperative in order to allow the clinician to closely monitor the patient's pulmonary status, particularly in the
absence of widely available autoantibody testing.

Rare cutaneous findings — Rarely reported cutaneous findings in patients with DM have included
ichthyosis, panniculitis, cutaneous vasculitis, lichen planus, porcelain white atrophic scars, vesicle and bullae
formation, follicular hyperkeratosis, malakoplakia, papular mucinosis, and flagellate erythema [15,16,23-25].
Diffuse non-pitting edema is another rare manifestation of DM, and may be a marker of more aggressive
disease [26].

Lung disease — ILD is an important complication in at least 10 percent of cases of DM and PM. In DM, it
can be observed in patients with either classic or amyopathic disease. In addition, respiratory insufficiency
may result from diaphragmatic and chest wall muscle weakness. The occurrence of ILD may be associated
with rapidly progressive pulmonary failure and death. ILD in the inflammatory myopathies often occurs in the
context of antisynthetase antibodies and the antisynthetase syndrome. These issues are discussed in detail
separately. (See "Interstitial lung disease in dermatomyositis and polymyositis: Clinical manifestations and
diagnosis" and 'Myositis-specific autoantibodies' below and 'Antisynthetase syndrome' below.)

Malignancy — An increased rate of malignancy has been described, with a greater risk in patients with DM.
The spectrum of malignancies generally parallels the distribution in the general population with a few
possible exceptions. This issue is discussed separately. (See "Malignancy in dermatomyositis and
polymyositis".)

Esophageal disease — Weakness of the striated muscle of the upper one-third of the esophagus (and/or the
- Page 4 of 18 -
Clinical manifestations of dermatomyositis and polymyositis in adults

oropharyngeal muscles) contributes to dysphagia, nasal regurgitation, and/or aspiration [27]. Esophageal
involvement is more common in older patients and may underlie the increased incidence of bacterial
pneumonia [28].

Cardiac disease — Cardiac involvement with histologic evidence of myocarditis is well-described in DM and
PM, and subclinical manifestations are frequent, including conduction abnormalities and arrhythmia detected
by electrocardiographic studies [29,30]. Symptomatic cardiac disease, such as congestive heart failure, is
less common [31]. However, patients with DM and PM are also at increased risk for myocardial infarction
[32,33]. A large, retrospective, population-based study found a nearly three- and fourfold increased risk of MI
among 350 and 424 patients with incident DM and PM, respectively, as compared with those without an
inflammatory myopathy, after controlling for relevant risk factors such as age, sex, glucocorticoids, and
nonsteroidal antiinflammatory drugs (NSAIDs) [33].

The use of serum creatine kinase (CK), the CK-MB/total CK ratio, and cardiac troponin T in evaluating
patients for cardiac involvement is problematic in patients with inflammatory myositis, and additional testing
may be required in patients in whom a myocardial infarction is suspected clinically [34-37]. (See 'Cardiac
enzymes and troponins' below.)

Antisynthetase syndrome — Up to 30 percent of patients with DM or PM have a constellation of clinical

findings termed the "antisynthetase syndrome" [38-40]. These findings include relatively acute disease onset,
constitutional symptoms (eg, fever and weight loss), myositis, the Raynaud phenomenon, mechanic's hands,
arthritis that is generally nonerosive, and ILD [41]. Affected patients have antibodies to aminoacyl-transfer
ribonucleic acid (tRNA) synthetase enzymes; the presence of one of these antibodies is highly specific for
DM, PM, or ILD [42-44]. (See 'Myositis-specific autoantibodies' below and "Interstitial lung disease in
dermatomyositis and polymyositis: Clinical manifestations and diagnosis" and 'Antisynthetase antibodies'
below.)

This syndrome can be further characterized as follows:

● Not all patients with antisynthetase antibodies or even those classified as having the antisynthetase
syndrome have all manifestations of this syndrome. The syndrome is generally considered present in
patients with an antisynthetase antibody plus two of the following features, which are elements of the
syndrome: ILD, inflammatory myopathy, and inflammatory polyarthritis.

● This group of clinical findings or this general clinical picture is not specific for antisynthetase antibodies.
Patients with other types of autoantibodies (eg, anti-PM-Scl or anti-U1 ribonucleoprotein [RNP]
antibodies) can also present with these types of features. However, patients with antisynthetase
antibodies generally have more prominent or severe myositis and ILD, and they usually lack some of the
other clinical features seen in patients with these other autoantibodies.

● Some patients with antisynthetase antibodies have relatively little or no myositis, while ILD or other
features are more prominent. The absence of myositis is seen more often with some antisynthetase
antibodies than with others.

Amyopathic dermatomyositis — A distinct group of patients with "clinically amyopathic dermatomyositis"

(CADM), historically called "dermatomyositis sine myositis," have classic cutaneous findings of DM without
clinical evidence of muscle weakness [45-48]. There are two subsets within this group. One subset, referred
to as "hypomyopathic dermatomyositis" (HDM), has subclinical evidence of myositis upon investigation by
- Page 5 of 18 -
Clinical manifestations of dermatomyositis and polymyositis in adults

laboratory, electromyography, muscle biopsy, or imaging despite a lack of clinical muscle weakness; the
other subset, classified as "amyopathic dermatomyositis" (ADM), includes patients without clinical weakness
and without either laboratory or muscle study abnormalities.

ADM is considered "provisional" after six months and "confirmed" after two years [49,50]. A definite
diagnosis of ADM is further supported by such patients having neither received systemic immunosuppressive
therapy for more than two consecutive months within the first six months of skin disease onset nor taken
drugs known to induce DM-like skin changes [50].

CADM comprises 10 to 30 percent of DM cases [13,51]. Patients with CADM also appear to be at increased
risk for internal malignancy, although this risk may be less than for patients with DM [13,51,52]. In addition,
some evidence suggests that the presence of anti-MDA-5 antibodies, previously referred to as anti-CADM-
140 autoantibodies, suggests that the patient is likely to have amyopathic disease and characteristic
cutaneous findings [22,53]. (See 'Skin findings in MDA-5-associated dermatomyositis' above and 'Other
myositis-specific autoantibodies' below.)

Overlap syndromes — DM and PM may overlap with features of other connective tissue diseases,
particularly scleroderma, SLE, and mixed connective tissue disease, as well as (less often) rheumatoid
arthritis and Sjögren's syndrome. The myopathy associated with the other connective tissue diseases varies
from clinically insignificant (with minimal muscle enzyme elevations and minimal inflammatory changes on
muscle biopsy) to typically severe DM or PM in which myopathy dominates the clinical picture [9,10,54]. (See
"Overview of the clinical manifestations of systemic sclerosis (scleroderma) in adults" and "Overview of the
clinical manifestations of systemic lupus erythematosus in adults" and "Clinical manifestations of mixed
connective tissue disease".)

LABORATORY FINDINGS — Several laboratory findings are characteristic of dermatomyositis (DM) and
polymyositis (PM). These include:

● Elevated levels of muscle enzymes [9,55]

● Autoantibodies, including antinuclear antibodies, in up to 80 percent of patients with DM and PM

[9,10,55,56]; myositis-specific autoantibodies, in at least 30 to 40 percent of patients [38,57-59]; and
myositis-associated autoantibodies, especially in patients with overlap syndromes

● Elevated levels of serum and urine myoglobin [60,61]

The erythrocyte sedimentation rate (ESR) is often normal or is only mildly elevated, even in patients with
active muscle disease [5].

Muscle enzymes — Creatine kinase (CK), lactate dehydrogenase (LD), aldolase, aspartate aminotransferase
(AST), and alanine aminotransferase (ALT) are all muscle enzymes that may be elevated in patients with
inflammatory myopathy and other muscle disorders. (See "Muscle enzymes in the evaluation of
neuromuscular diseases".)

At some point in the course of the disease, almost all patients with DM and PM, except those with
amyopathic DM (ADM), have an elevation in at least one muscle enzyme; most have elevations in all
enzymes. In a review of 153 patients with DM or PM, normal results were found for CK in 5 percent, for
aldolase in 4 percent, for LD in 9 percent, and for the aminotransferases in 15 to 17 percent [9]. However,
these data may underestimate the frequency of normal CK concentrations because the Bohan and Peter
- Page 6 of 18 -
Clinical manifestations of dermatomyositis and polymyositis in adults

criteria employed in that study included muscle enzyme elevation as a disease criterion. Additionally, the
study was performed before many autoantibody tests and magnetic resonance imaging (MRI) studies were
available to facilitate diagnosis. (See "Diagnosis and differential diagnosis of dermatomyositis and
polymyositis in adults", section on 'Classification criteria'.)

The level of serum CK can vary widely. In untreated patients with active muscle disease, it is usually more
than 10-fold the upper limit of normal (ie, at least 2000 to 3000 international units/L). In severe cases, the
serum CK concentration may be elevated more than 50-fold or even 100-fold (ie, up to 10,000 to 20,000
international units/L), and higher levels are sometimes seen.

A correlation between the severity of the weakness and the height of elevation in serum muscle enzymes may
be seen, although the degree of muscle dysfunction may be much greater than the enzyme levels would
suggest [10]. In some cases, there may be elevations in serum muscle enzymes without discernible muscle
weakness. This is particularly observed in patients with early disease.

The occurrence of muscle weakness with relatively normal enzyme levels is more likely to occur in DM than
PM. Other patients with no clinical muscle involvement, as in ADM, will also have normal enzyme levels.
Persistently low serum muscle enzyme levels in the setting of obvious muscle weakness may also occur in
patients with advanced disease and significant loss of muscle mass.

Cardiac enzymes and troponins — Patients with myositis may have an elevated serum CK-MB fraction
in the absence of myocarditis, which is usually attributed to increased expression in regenerating skeletal
muscle affected by the inflammatory disease or, less often, to involvement of the myocardium by the
myositis. Myocardial infarction may be suspected in these patients, who may require additional testing. (See
"Troponin testing: Clinical use" and "Clinical manifestations and diagnosis of myocarditis in adults", section
on 'Cardiac biomarkers'.)

Measurement of cardiac troponin I may be helpful in patients in whom it may be difficult clinically to
determine whether elevations in CK or other muscle enzymes are due to cardiac rather than skeletal muscle
disease. Increased levels of cardiac troponin I appear relatively specific for myocardial injury, unlike
elevations of total CK, CK-MB, and other muscle enzymes or of cardiac troponin T, all of which may be seen
both in skeletal muscle inflammatory myopathy and in cardiac disease [5,35-37,55]. (See 'Cardiac disease'
above and "Troponin testing: Clinical use".)

Autoantibodies — Antinuclear antibodies detected by standard immunofluorescence methods may be

present in up to 80 percent of patients with DM or PM [38,56,62]. Testing for specific autoantibodies may
demonstrate either of the following:

● Myositis-specific autoantibodies, which are detected primarily in patients with inflammatory myositis and
which may offer information regarding prognosis and potential patterns of organ involvement

● Myositis-associated autoantibodies, which are found with other autoimmune rheumatic diseases that
may be associated with myositis

Myositis-specific autoantibodies — Several categories of autoantibodies found primarily in patients with

myositis and directed against cytoplasmic proteins, ribonucleoproteins, and certain nuclear antigens are
termed myositis-specific autoantibodies [38,42,44,57,63]. These autoantibodies occur in approximately 30
percent of patients with DM and PM.
- Page 7 of 18 -
Clinical manifestations of dermatomyositis and polymyositis in adults

Particular myositis-specific autoantibodies or classes of myositis–specific autoantibodies are associated with

particular clinical syndromes within the myositis spectrum and may be associated with certain
histopathologic findings [44,64-68]. It is not known whether such antibodies have a direct role in disease
pathogenesis, and this question remains an area of strong interest to investigators. (See "Diagnosis and
differential diagnosis of dermatomyositis and polymyositis in adults", section on 'MSA and histopathology'
and "Pathogenesis of inflammatory myopathies".)

There are several categories of myositis-specific autoantibodies, including:

● Antibodies to aminoacyl-transfer (t)RNA synthetases (antisynthetase antibodies), including anti-Jo-1

● Antibodies to signal recognition particle (SRP)

● Antibodies to Mi-2, a nuclear helicase

Other types of myositis-specific autoantibodies that do not fall into one of these categories have also been
described, as discussed below [68].

Antisynthetase antibodies — Anti-Jo-1 antibodies are the most common myositis-specific

autoantibody and the most frequently observed antisynthetase antibody. Anti-Jo-1 is seen in about 20
percent of patients with idiopathic inflammatory myopathy. Anti-Jo-1 antibodies are directed against histidyl–
tRNA synthetase, one of a group of enzymes that catalyze the attachment of specific amino acids to their
cognate tRNAs during the process of protein synthesis. These antibodies are strongly associated with several
clinical findings, including interstitial lung disease (ILD), the Raynaud phenomenon, arthritis, and mechanic's
hands (picture 9) [38,63]. (See 'Antisynthetase syndrome' above and 'Skin findings in antisynthetase
syndrome' above.)

In one study at an academic medical center, inflammatory myositis, often with the antisynthetase syndrome,
was present in 94 percent of 81 patients with anti-Jo-1 antibodies in whom testing had been performed for
suspected autoimmune disease [69]. DM or PM was present in 88 percent, and an undifferentiated
connective tissue disease or overlap syndrome was present in 12 percent of patients, several of whom had
systemic sclerosis (SSc). Other findings in the patients positive for Jo-1 included ILD (69 percent), arthritis (57
percent), the Raynaud phenomenon (53 percent), mechanic's hands (17 percent), and sclerodactyly (12
percent).

Anti-Jo-1 and other antisynthetase antibodies, such as anti-PL-12, have been observed in some patients with
ILD who lack evidence of myositis [44]. Other antisynthetase antibodies include antibodies to the OJ, EJ, PL-
7, PL-12, KS, Zo, and Ha antigens [43,70,71]. In the aggregate, these are found in 1 to 5 percent of idiopathic
inflammatory myopathy patients. Patients with these other antisynthetase antibodies may have clinical
manifestations similar to those with anti-Jo-1-antibodies, but differences have sometimes been noted,
particularly in the frequency of myositis [44].

Anti-SRP antibodies — The signal recognition particle (SRP) is involved in the translocation of newly
synthesized proteins into the endoplasmic reticulum. Anti-SRP antibodies have been found in about 5
percent of patients with inflammatory myopathy and were described almost exclusively in patients diagnosed
with PM [64,72]. Unlike other patients with PM, however, the muscle biopsies in these patients typically show
muscle fiber necrosis and endomysial fibrosis but show little or no inflammatory cell infiltrate [64,73]. In one
study of patients with such disease, termed necrotizing myopathy, anti-SRP antibodies were present in 6 of
- Page 8 of 18 -
Clinical manifestations of dermatomyositis and polymyositis in adults

38 patients (16 percent) [74].

Anti-SRP antibodies are associated with severe myopathy and aggressive disease that may be difficult to
control, even with high-dose glucocorticoids and immunosuppressive agents. These antibodies are not
entirely specific for necrotizing myopathy or polymyositis, however, as they have also been described in two
patients each with limb girdle muscular dystrophy and SSc, in a patient with the antisynthetase syndrome
without myopathy, and in several patients with DM [5,73,75]. (See "Initial treatment of dermatomyositis and
polymyositis in adults".)

Anti-Mi-2 antibodies — Anti-Mi-2 antibodies are directed against a helicase involved in transcriptional
activation [76]. Among patients with DM, anti-Mi-2 antibodies are present in about 7 percent of Caucasians
and in about 30 percent of those from Central America [6]. They are associated with the relatively acute onset
of DM, are traditionally associated with a classic shawl or V sign, and may respond well to therapy [38]. (See
'Characteristic dermatomyositis findings' above.)

Other myositis-specific autoantibodies — A number of other myositis-specific autoantibodies have

been described in different populations of patients with myositis. The clinical usefulness of these
autoantibodies in patients suspected of having DM or PM is not well-established:

● Antibodies directed against hPMS-1, a deoxyribonucleic acid (DNA) mismatch repair enzyme, are
myositis-specific autoantibodies reported to occur in 7.5 percent of patients with myositis [77].

● Antibodies that have high specificity for ADM were first reported in Japanese patients [78-80]. These
antibodies recognize a protein involved in innate immune responses, called clinically ADM (CADM)-140,
a 140kD polypeptide. This protein is the RNA helicase encoded by the melanoma differentiation-
associated gene 5 (MDA-5), and is now most often referred to as the anti-MDA-5 antibody [79,80]. In
reports from two different groups in Japan, the presence of anti-MDA-5 antibodies was also strongly
associated with the development of rapidly progressive ILD [78-80]. Similarly, in another cohort of 64
Chinese patients with DM or PM, anti-MDA-5 antibodies were strongly associated with rapidly
progressive ILD. These patients, however, when compared with Japanese cohorts in a meta-analysis,
demonstrated a significantly lower frequency of CADM [81]. (See 'Amyopathic dermatomyositis' above.)

Anti-MDA-5 has also been described in a small cohort of patients in the United States with DM but little
or no myositis, with increased risk of ILD, and with vasculopathy affecting the skin [22]. In addition to the
typical cutaneous signs of dermatomyositis, anti-MDA-5 patients characteristically develop cutaneous
ulceration involving Gottron's papules and Gottron's sign, digital pulp, and nailfolds, as well as
erythematous, painful palmar papules and macules, alopecia, and oral ulcers. These patients also
frequently have arthritis and amyopathic disease. Anti-MDA-5 antibodies are strongly associated with
ILD with a rapidly progressive course and poor overall survival related to pulmonary complications [82].
In a large case-control study investigating the rates of mortality among DM patients with various
myositis-associated antibodies, those with anti-MDA-5 antibodies displayed the lowest survival rates,
even when compared with individuals with malignancy-associated DM [83]. Further study in other
populations will help to clarify the diagnostic and prognostic utility of this antibody. Evidence suggests
that patients with MDA-5 antibodies should be monitored closely for lung involvement. (See 'Skin
findings in MDA-5-associated dermatomyositis' above.)

● Anti-p140, also termed anti-MJ, is an antibody against a 140-kD protein that is distinct from the anti-

- Page 9 of 18 -
Clinical manifestations of dermatomyositis and polymyositis in adults
CADM-140 antibodies. This antibody has been studied primarily in patients with juvenile DM, in whom it
is associated with calcinosis [19]. This association has also been described in adults with DM [20]. The
antibody is directed against nuclear matrix protein 2 (NXP2), which has a role in transcriptional
regulation. It has also been found in some adults with DM and ILD [71].

● Antibodies to a 155-kD protein were found in sera of 51 of 244 patients with myositis (21 percent); most
of the positive patients had a form of DM. Antibodies were present in only 1 of 108 patients with other
connective tissue diseases and in no normal controls [84]. Caucasian patients with this autoantibody had
a unique human leukocyte antigen (HLA) risk factor, HLA-DQA1*0301, and had an increased frequency
of the V sign rash. Patients with this autoantibody were clinically distinct from those with autoantibodies
to aminoacyl-transfer RNA synthetases.

This myositis-specific autoantibody, known as the anti-p155/140 antibody, has reactivity with the 155-kD
nuclear protein transcriptional intermediary factor (TIF)-1gamma and often also with the 140-kD TIF-
1alpha [71,84,85]. Reaction with TIF-1beta may also occur [85,86]. It has also been described in
Japanese patients [87]. The anti-155/140 antibody was detected in 7 of 52 patients (13 percent) with DM
but in none of the disease controls with PM, systemic lupus erythematosus (SLE), SSc, or idiopathic
interstitial pneumonia. Clinical associations of the anti-155/140 antibody include strong associations with
adult and juvenile DM, rather than PM, and with cancer-associated myositis. Flagellate erythema and the
conventional cutaneous DM findings of Gottron's papules and a heliotrope eruption may be present
[87,88]. The anti-p155/140 antibody is associated with an increased risk for cancer in patients with DM,
with a sensitivity approaching 70 percent and specificity approaching 90 percent [89]. (See "Malignancy
in dermatomyositis and polymyositis".)

● Myositis-specific autoantibodies that target the small ubiquitin-like modifier activating enzyme (SAE)
were found in 8 percent of 266 patients with adult DM but were not found in 250 patients with other
connective tissue diseases or in 50 healthy controls [90]. Most of the patients with anti-SAE antibodies
presented with skin disease, including heliotrope eruptions and Gottron's lesions, before progressing to
myositis with systemic manifestations, including dysphagia. The autoantibody was strongly associated
with the HLA-DQB1*03, HLA-DRB1*04, and HLA-DQA1*03 haplotypes.

Autoantibodies and overlap syndromes — The detection of anti-Ro, anti-La, anti-Sm, or anti-
ribonucleoprotein (RNP) antibodies in a patient with myositis suggests a diagnosis of myositis associated or
overlapping with another systemic rheumatic disease [56]. Anti-Ro52 antibodies are common in patients with
antisynthetase antibodies, and anti-Ro60 and anti-La may be seen in a smaller number of such patients and
in those with other myositis-specific antibodies. (See "The anti-Ro/SSA and anti-La/SSB antigen-antibody
systems" and "Antibodies to double-stranded (ds)DNA, Sm, and U1 RNP".)

The presence of anti-Ro52 without anti-Ro60 is more common in myositis than in other conditions and is
more common in patients with the antisynthetase syndrome than in others with myositis. In general, anti-Ro,
anti-La, and anti-U1 RNP can be seen in some patients who also have myositis-specific autoantibodies, but
myositis-specific autoantibodies tend to be mutually exclusive with each other. High titers of anti-RNP
antibodies are associated with mixed connective tissue disease, the overlap syndrome of myositis with
features of scleroderma and SLE [91]. (See "The anti-Ro/SSA and anti-La/SSB antigen-antibody systems",
section on 'Anti-Ro60 versus anti-Ro52 antibodies' and "Definition and diagnosis of mixed connective tissue
disease".)

- Page 10 of 18 -
Clinical manifestations of dermatomyositis and polymyositis in adults
Anti-PM-Scl and anti-Ku antibodies have been identified in patients with overlapping features of myositis and

scleroderma [58,65]. Many patients with these antibodies, however, do not have myositis. (See "Diagnosis
and differential diagnosis of systemic sclerosis (scleroderma) in adults", section on 'Laboratory testing'.)

The precise diagnosis of an underlying connective tissue disease is critical to patient management because
of the prognostic and treatment implications of specific diagnoses. (See "Antibodies to double-stranded
(ds)DNA, Sm, and U1 RNP" and "The anti-Ro/SSA and anti-La/SSB antigen-antibody systems".)

HISTOPATHOLOGY — Dermatomyositis (DM) and polymyositis (PM) can be distinguished from each other
and from other forms of myopathy by their histopathologic findings. In patients with DM, characteristic
findings may also be seen on skin biopsy, although these findings are very similar on light microscopy to
changes that can be seen in systemic lupus erythematosus (SLE). The histologic features of DM and PM are
described in detail separately. (See "Diagnosis and differential diagnosis of dermatomyositis and polymyositis
in adults", section on 'Muscle histopathology' and "Diagnosis and differential diagnosis of dermatomyositis
and polymyositis in adults", section on 'Skin histopathology'.)

ELECTROMYOGRAPHY — Characteristic electrophysiologic abnormalities on electromyography (EMG) are

often seen in inflammatory myopathy. However, such changes are not specific for the diagnoses of
dermatomyositis (DM) or polymyositis (PM), and the EMG is normal in approximately 10 percent of patients.
Similar findings may occur in various infectious, toxic, or metabolic myopathies. The EMG abnormalities seen
in DM and PM are described in detail separately. (See "Diagnosis and differential diagnosis of
dermatomyositis and polymyositis in adults", section on 'EMG findings'.)

MAGNETIC RESONANCE IMAGING — Magnetic resonance imaging (MRI) of skeletal muscles is a

noninvasive sensitive but nonspecific modality for detecting areas of muscle inflammation and edema with
active myositis, fibrosis, and calcification. (image 1) [92]. Findings on MRI in dermatomyositis (DM) and
polymyositis (PM) are described separately. (See "Diagnosis and differential diagnosis of dermatomyositis
and polymyositis in adults", section on 'MRI'.)

SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from selected
countries and regions around the world are provided separately. (See "Society guideline links:
Dermatomyositis and polymyositis".)

INFORMATION FOR PATIENTS — UpToDate offers two types of patient education materials, "The Basics"
and "Beyond the Basics." The Basics patient education pieces are written in plain language, at the 5th to 6th
grade reading level, and they answer the four or five key questions a patient might have about a given
condition. These articles are best for patients who want a general overview and who prefer short, easy-to-
read materials. Beyond the Basics patient education pieces are longer, more sophisticated, and more
detailed. These articles are written at the 10th to 12th grade reading level and are best for patients who want
in-depth information and are comfortable with some medical jargon.

Here are the patient education articles that are relevant to this topic. We encourage you to print or e-mail
these topics to your patients. (You can also locate patient education articles on a variety of subjects by
searching on "patient info" and the keyword(s) of interest.)

● Basics topics (see "Patient education: Dermatomyositis (The Basics)" and "Patient education:
Polymyositis (The Basics)")

- Page 11 of 18 -
Clinical manifestations of dermatomyositis and polymyositis in adults
● Beyond the Basics topics (see "Patient education: Polymyositis, dermatomyositis, and other forms of

idiopathic inflammatory myopathy (Beyond the Basics)")

SUMMARY

● Dermatomyositis (DM) and polymyositis (PM) are both multisystem disorders with a wide variety of
clinical manifestations. Most patients exhibit proximal skeletal muscle weakness and evidence of muscle
inflammation, but the immune mechanisms and the anatomic focus of injury within the muscle tissue in
DM and PM appear distinct. The muscle weakness usually develops in an insidious or subacute fashion,
with gradual worsening over a period of several months. Muscle atrophy may be present in severe,
longstanding disease. Oropharyngeal and upper esophageal muscle involvement may lead to dysphagia,
nasal regurgitation, or aspiration. Respiratory failure may result from weakness of the diaphragm and
chest wall muscles. (See 'Clinical manifestations' above and 'Muscle weakness' above and 'Lung
disease' above and 'Esophageal disease' above.)

● Several characteristic skin eruptions are typical of DM, including Gottron's papules and the heliotrope
eruption (picture 1A, 3A); these changes are considered pathognomonic for DM. Photodistributed
erythema and poikiloderma, as well as nailfold changes, are also characteristic and useful in
distinguishing DM from PM. A form of DM, termed amyopathic DM (ADM, historically known as
"dermatomyositis sine myositis"), is a condition in which patients have characteristic skin findings of DM
without weakness, abnormal muscle enzymes, or other abnormal muscle studies. (See 'Clinical
manifestations' above and 'Skin findings' above and 'Amyopathic dermatomyositis' above.)

● Interstitial lung disease (ILD) may occur with DM (including ADM), PM, and overlap myositis. Dysphagia
and polyarthritis are also common in DM and PM, along with constitutional symptoms; the Raynaud
phenomenon is present in some patients. Features that overlap with other systemic rheumatic diseases,
such as systemic lupus erythematosus (SLE) and systemic sclerosis (SSc), may also be present. The risk
of malignancy may be increased, particularly in patients with DM. (See 'Clinical manifestations' above
and 'Lung disease' above and 'Esophageal disease' above and 'Antisynthetase syndrome' above and
'Overlap syndromes' above and 'Malignancy' above.)

● Elevations in serum creatine kinase (CK), lactate dehydrogenase, aldolase, and aminotransferases occur
in most patients. Muscle enzyme levels are useful in making the diagnosis and in following disease
activity. Although CK-MB elevation is not unusual, a common source is skeletal muscle, and
symptomatic cardiac muscle involvement is uncommon. (See 'Muscle enzymes' above and 'Cardiac
disease' above.)

● Autoantibodies are found in a majority of patients. Among the myositis-specific autoantibodies,

antibodies against aminoacyl transfer ribonucleic acid (tRNA) synthetases, particularly anti-histidyl-tRNA
synthetase antibodies (anti-Jo-1), have been associated with ILD, the Raynaud phenomenon, arthritis,
and mechanic's hands, known collectively as the antisynthetase syndrome. Additional myositis-specific
autoantibodies include other antisynthetase antibodies, anti-signal recognition particle antibodies, anti-
Mi-2 antibodies, and others. (See 'Autoantibodies' above and 'Antisynthetase syndrome' above.)

- Page 12 of 18 -
Clinical manifestations of dermatomyositis and polymyositis in adults
REFERENCES

1. Dalakas MC, Hohlfeld R. Polymyositis and dermatomyositis. Lancet 2003; 362:971.

2. Plotz PH, Dalakas M, Leff RL, et al. Current concepts in the idiopathic inflammatory myopathies:
polymyositis, dermatomyositis, and related disorders. Ann Intern Med 1989; 111:143.
3. Bohan A, Peter JB. Polymyositis and dermatomyositis (first of two parts). N Engl J Med 1975;
292:344.
4. Bohan A, Peter JB. Polymyositis and dermatomyositis (second of two parts). N Engl J Med 1975;
292:403.
5. Amato AA, Barohn RJ. Evaluation and treatment of inflammatory myopathies. J Neurol Neurosurg
Psychiatry 2009; 80:1060.
6. Shamim EA, Rider LG, Pandey JP, et al. Differences in idiopathic inflammatory myopathy
phenotypes and genotypes between Mesoamerican Mestizos and North American Caucasians:
ethnogeographic influences in the genetics and clinical expression of myositis. Arthritis Rheum
2002; 46:1885.
7. O'Hanlon TP, Carrick DM, Targoff IN, et al. Immunogenetic risk and protective factors for the
idiopathic inflammatory myopathies: distinct HLA-A, -B, -Cw, -DRB1, and -DQA1 allelic profiles
distinguish European American patients with different myositis autoantibodies. Medicine
(Baltimore) 2006; 85:111.
8. Jacobson DL, Gange SJ, Rose NR, Graham NM. Epidemiology and estimated population burden
of selected autoimmune diseases in the United States. Clin Immunol Immunopathol 1997; 84:223.
9. Bohan A, Peter JB, Bowman RL, Pearson CM. Computer-assisted analysis of 153 patients with
polymyositis and dermatomyositis. Medicine (Baltimore) 1977; 56:255.
10. Tymms KE, Webb J. Dermatopolymyositis and other connective tissue diseases: a review of 105
cases. J Rheumatol 1985; 12:1140.
11. Cooper GS, Stroehla BC. The epidemiology of autoimmune diseases. Autoimmun Rev 2003;
2:119.
12. Bernatsky S, Joseph L, Pineau CA, et al. Estimating the prevalence of polymyositis and
dermatomyositis from administrative data: age, sex and regional differences. Ann Rheum Dis
2009; 68:1192.
13. Bendewald MJ, Wetter DA, Li X, Davis MD. Incidence of dermatomyositis and clinically
amyopathic dermatomyositis: a population-based study in Olmsted County, Minnesota. Arch
Dermatol 2010; 146:26.
14. Sekiguchi K, Kanda F, Oishi K, et al. HLA typing in focal myositis. J Neurol Sci 2004; 227:21.
15. Callen JP, Wortmann RL. Dermatomyositis. Clin Dermatol 2006; 24:363.
16. Callen JP. Cutaneous manifestations of dermatomyositis and their management. Curr Rheumatol
Rep 2010; 12:192.
17. Dugan EM, Huber AM, Miller FW, et al. Photoessay of the cutaneous manifestations of the
idiopathic inflammatory myopathies. Dermatol Online J 2009; 15:1.
18. Smith RL, Sundberg J, Shamiyah E, et al. Skin involvement in juvenile dermatomyositis is
associated with loss of end row nailfold capillary loops. J Rheumatol 2004; 31:1644.
19. Gunawardena H, Wedderburn LR, Chinoy H, et al. Autoantibodies to a 140-kd protein in juvenile
- Page 13 of 18 -
Clinical manifestations of dermatomyositis and polymyositis in adults
dermatomyositis are associated with calcinosis. Arthritis Rheum 2009; 60:1807.
20. Ceribelli A, Fredi M, Taraborelli M, et al. Anti-MJ/NXP-2 autoantibody specificity in a cohort of
adult Italian patients with polymyositis/dermatomyositis. Arthritis Res Ther 2012; 14:R97.
21. Stahl NI, Klippel JH, Decker JL. A cutaneous lesion associated with myositis. Ann Intern Med
1979; 91:577.

22. Fiorentino D, Chung L, Zwerner J, et al. The mucocutaneous and systemic phenotype of
dermatomyositis patients with antibodies to MDA5 (CADM-140): a retrospective study. J Am Acad
Dermatol 2011; 65:25.
23. Yamamoto T, Nishioka K. Flagellate erythema. Int J Dermatol 2006; 45:627.
24. Iorizzo LJ 3rd, Jorizzo JL. The treatment and prognosis of dermatomyositis: an updated review. J
Am Acad Dermatol 2008; 59:99.
25. Jara M, Amérigo J, Duce S, Borbujo J. Dermatomyositis and flagellate erythema. Clin Exp
Dermatol 1996; 21:440.
26. Milisenda JC, Doti PI, Prieto-González S, Grau JM. Dermatomyositis presenting with severe
subcutaneous edema: five additional cases and review of the literature. Semin Arthritis Rheum
2014; 44:228.
27. de Merieux P, Verity MA, Clements PJ, Paulus HE. Esophageal abnormalities and dysphagia in
polymyositis and dermatomyositis. Arthritis Rheum 1983; 26:961.
28. Marie I, Hatron PY, Levesque H, et al. Influence of age on characteristics of polymyositis and
dermatomyositis in adults. Medicine (Baltimore) 1999; 78:139.
29. Denbow CE, Lie JT, Tancredi RG, Bunch TW. Cardiac involvement in polymyositis: a
clinicopathologic study of 20 autopsied patients. Arthritis Rheum 1979; 22:1088.
30. Lundberg IE. The heart in dermatomyositis and polymyositis. Rheumatology (Oxford) 2006; 45
Suppl 4:iv18.
31. Danieli MG, Gelardi C, Guerra F, et al. Cardiac involvement in polymyositis and dermatomyositis.
Autoimmun Rev 2016; 15:462.
32. Tisseverasinghe A, Bernatsky S, Pineau CA. Arterial events in persons with dermatomyositis and
polymyositis. J Rheumatol 2009; 36:1943.
33. Rai SK, Choi HK, Sayre EC, Aviña-Zubieta JA. Risk of myocardial infarction and ischaemic stroke
in adults with polymyositis and dermatomyositis: a general population-based study.
Rheumatology (Oxford) 2016; 55:461.
34. Larca LJ, Coppola JT, Honig S. Creatine kinase MB isoenzyme in dermatomyositis: a noncardiac
source. Ann Intern Med 1981; 94:341.
35. Badsha H, Gunes B, Grossman J, Brahn E. Troponin I Assessment of Cardiac Involvement in
Patients With Connective Tissue Disease and an Elevated Creatine Kinase MB Isoform Report of
Four Cases and Review of the Literature. J Clin Rheumatol 1997; 3:131.
36. Kiely PD, Bruckner FE, Nisbet JA, Daghir A. Serum skeletal troponin I in inflammatory muscle
disease: relation to creatine kinase, CKMB and cardiac troponin I. Ann Rheum Dis 2000; 59:750.
37. Aggarwal R, Lebiedz-Odrobina D, Sinha A, et al. Serum cardiac troponin T, but not troponin I, is
elevated in idiopathic inflammatory myopathies. J Rheumatol 2009; 36:2711.
38. Love LA, Leff RL, Fraser DD, et al. A new approach to the classification of idiopathic inflammatory

- Page 14 of 18 -
Clinical manifestations of dermatomyositis and polymyositis in adults
myopathy: myositis-specific autoantibodies define useful homogeneous patient groups. Medicine
(Baltimore) 1991; 70:360.
39. Mimori T, Imura Y, Nakashima R, Yoshifuji H. Autoantibodies in idiopathic inflammatory myopathy:
an update on clinical and pathophysiological significance. Curr Opin Rheumatol 2007; 19:523.
40. Targoff IN. Autoantibodies and their significance in myositis. Curr Rheumatol Rep 2008; 10:333.
41. Katzap E, Barilla-LaBarca ML, Marder G. Antisynthetase syndrome. Curr Rheumatol Rep 2011;
13:175.
42. Targoff IN. Myositis specific autoantibodies. Curr Rheumatol Rep 2006; 8:196.
43. Betteridge Z, Gunawardena H, North J, et al. Anti-synthetase syndrome: a new autoantibody to
phenylalanyl transfer RNA synthetase (anti-Zo) associated with polymyositis and interstitial
pneumonia. Rheumatology (Oxford) 2007; 46:1005.
44. Mammen AL. Dermatomyositis and polymyositis: Clinical presentation, autoantibodies, and
pathogenesis. Ann N Y Acad Sci 2010; 1184:134.
45. Gerami P, Schope JM, McDonald L, et al. A systematic review of adult-onset clinically amyopathic
dermatomyositis (dermatomyositis siné myositis): a missing link within the spectrum of the
idiopathic inflammatory myopathies. J Am Acad Dermatol 2006; 54:597.
46. Euwer RL, Sontheimer RD. Amyopathic dermatomyositis (dermatomyositis siné myositis).
Presentation of six new cases and review of the literature. J Am Acad Dermatol 1991; 24:959.
47. Stonecipher MR, Jorizzo JL, White WL, et al. Cutaneous changes of dermatomyositis in patients
with normal muscle enzymes: dermatomyositis sine myositis? J Am Acad Dermatol 1993; 28:951.
48. Sontheimer RD. Clinically amyopathic dermatomyositis: what can we now tell our patients? Arch
Dermatol 2010; 146:76.
49. Sontheimer RD. Cutaneous features of classic dermatomyositis and amyopathic dermatomyositis.
Curr Opin Rheumatol 1999; 11:475.
50. Sontheimer RD. Would a new name hasten the acceptance of amyopathic dermatomyositis
(dermatomyositis siné myositis) as a distinctive subset within the idiopathic inflammatory
dermatomyopathies spectrum of clinical illness? J Am Acad Dermatol 2002; 46:626.
51. Galimberti F, Li Y, Fernandez AP. Clinically amyopathic dermatomyositis: clinical features,
response to medications and malignancy-associated risk factors in a specific tertiary-care-centre
cohort. Br J Dermatol 2016; 174:158.
52. Dawkins MA, Jorizzo JL, Walker FO, et al. Dermatomyositis: a dermatology-based case series. J
Am Acad Dermatol 1998; 38:397.
53. Cao H, Pan M, Kang Y, et al. Clinical manifestations of dermatomyositis and clinically amyopathic
dermatomyositis patients with positive expression of anti-melanoma differentiation-associated
gene 5 antibody. Arthritis Care Res (Hoboken) 2012; 64:1602.
54. Ringel RA, Brick JE, Brick JF, et al. Muscle involvement in the scleroderma syndromes. Arch Intern
Med 1990; 150:2550.
55. Hochberg MC, Feldman D, Stevens MB. Adult onset polymyositis/dermatomyositis: an analysis of
clinical and laboratory features and survival in 76 patients with a review of the literature. Semin
Arthritis Rheum 1986; 15:168.
56. Reichlin M, Arnett FC Jr. Multiplicity of antibodies in myositis sera. Arthritis Rheum 1984; 27:1150.
57. Brouwer R, Hengstman GJ, Vree Egberts W, et al. Autoantibody profiles in the sera of European
- Page 15 of 18 -
Clinical manifestations of dermatomyositis and polymyositis in adults
patients with myositis. Ann Rheum Dis 2001; 60:116.
58. Koenig M, Fritzler MJ, Targoff IN, et al. Heterogeneity of autoantibodies in 100 patients with
autoimmune myositis: insights into clinical features and outcomes. Arthritis Res Ther 2007; 9:R78.
59. Chinoy H, Salway F, Fertig N, et al. In adult onset myositis, the presence of interstitial lung disease
and myositis specific/associated antibodies are governed by HLA class II haplotype, rather than
by myositis subtype. Arthritis Res Ther 2006; 8:R13.

60. Kroll M, Otis J, Kagen L. Serum enzyme, myoglobin and muscle strength relationships in
polymyositis and dermatomyositis. J Rheumatol 1986; 13:349.
61. Bombardieri S, Clerico A, Riente L, et al. Circadian variations of serum myoglobin levels in normal
subjects and patients with polymyositis. Arthritis Rheum 1982; 25:1419.
62. Drake LA, Dinehart SM, Farmer ER, et al. Guidelines of care for dermatomyositis. American
Academy of Dermatology. J Am Acad Dermatol 1996; 34:824.
63. Plotz PH, Rider LG, Targoff IN, et al. NIH conference. Myositis: immunologic contributions to
understanding cause, pathogenesis, and therapy. Ann Intern Med 1995; 122:715.
64. Miller T, Al-Lozi MT, Lopate G, Pestronk A. Myopathy with antibodies to the signal recognition
particle: clinical and pathological features. J Neurol Neurosurg Psychiatry 2002; 73:420.
65. Jablonska S, Blaszyk M. Scleromyositis (scleroderma/polimyositis overlap) is an entity. J Eur Acad
Dermatol Venereol 2004; 18:265.
66. Greenberg SA, Sanoudou D, Haslett JN, et al. Molecular profiles of inflammatory myopathies.
Neurology 2002; 59:1170.
67. Mozaffar T, Pestronk A. Myopathy with anti-Jo-1 antibodies: pathology in perimysium and
neighbouring muscle fibres. J Neurol Neurosurg Psychiatry 2000; 68:472.
68. Gunawardena H, Betteridge ZE, McHugh NJ. Myositis-specific autoantibodies: their clinical and
pathogenic significance in disease expression. Rheumatology (Oxford) 2009; 48:607.
69. Stone KB, Oddis CV, Fertig N, et al. Anti-Jo-1 antibody levels correlate with disease activity in
idiopathic inflammatory myopathy. Arthritis Rheum 2007; 56:3125.
70. Kalluri M, Sahn SA, Oddis CV, et al. Clinical profile of anti-PL-12 autoantibody. Cohort study and
review of the literature. Chest 2009; 135:1550.
71. Betteridge ZE, Gunawardena H, McHugh NJ. Novel autoantibodies and clinical phenotypes in
adult and juvenile myositis. Arthritis Res Ther 2011; 13:209.
72. Targoff IN, Johnson AE, Miller FW. Antibody to signal recognition particle in polymyositis. Arthritis
Rheum 1990; 33:1361.
73. Hengstman GJ, ter Laak HJ, Vree Egberts WT, et al. Anti-signal recognition particle
autoantibodies: marker of a necrotising myopathy. Ann Rheum Dis 2006; 65:1635.
74. Christopher-Stine L, Casciola-Rosen LA, Hong G, et al. A novel autoantibody recognizing 200-kd
and 100-kd proteins is associated with an immune-mediated necrotizing myopathy. Arthritis
Rheum 2010; 62:2757.
75. Kao AH, Lacomis D, Lucas M, et al. Anti-signal recognition particle autoantibody in patients with
and patients without idiopathic inflammatory myopathy. Arthritis Rheum 2004; 50:209.
76. Seelig HP, Moosbrugger I, Ehrfeld H, et al. The major dermatomyositis-specific Mi-2 autoantigen
is a presumed helicase involved in transcriptional activation. Arthritis Rheum 1995; 38:1389.

- Page 16 of 18 -
Clinical manifestations of dermatomyositis and polymyositis in adults
77. Casciola-Rosen LA, Pluta AF, Plotz PH, et al. The DNA mismatch repair enzyme PMS1 is a
myositis-specific autoantigen. Arthritis Rheum 2001; 44:389.
78. Sato S, Hirakata M, Kuwana M, et al. Autoantibodies to a 140-kd polypeptide, CADM-140, in
Japanese patients with clinically amyopathic dermatomyositis. Arthritis Rheum 2005; 52:1571.
79. Sato S, Hoshino K, Satoh T, et al. RNA helicase encoded by melanoma differentiation-associated
gene 5 is a major autoantigen in patients with clinically amyopathic dermatomyositis: Association
with rapidly progressive interstitial lung disease. Arthritis Rheum 2009; 60:2193.
80. Nakashima R, Imura Y, Kobayashi S, et al. The RIG-I-like receptor IFIH1/MDA5 is a
dermatomyositis-specific autoantigen identified by the anti-CADM-140 antibody. Rheumatology
(Oxford) 2010; 49:433.
81. Chen Z, Cao M, Plana MN, et al. Utility of anti-melanoma differentiation-associated gene 5
antibody measurement in identifying patients with dermatomyositis and a high risk for developing
rapidly progressive interstitial lung disease: a review of the literature and a meta-analysis. Arthritis
Care Res (Hoboken) 2013; 65:1316.
82. Moghadam-Kia S, Oddis CV, Sato S, et al. Anti-Melanoma Differentiation-Associated Gene 5 Is
Associated With Rapidly Progressive Lung Disease and Poor Survival in US Patients With
Amyopathic and Myopathic Dermatomyositis. Arthritis Care Res (Hoboken) 2016; 68:689.
83. Hamaguchi Y, Kuwana M, Hoshino K, et al. Clinical correlations with dermatomyositis-specific
autoantibodies in adult Japanese patients with dermatomyositis: a multicenter cross-sectional
study. Arch Dermatol 2011; 147:391.
84. Targoff IN, Mamyrova G, Trieu EP, et al. A novel autoantibody to a 155-kd protein is associated
with dermatomyositis. Arthritis Rheum 2006; 54:3682.
85. Fujimoto M, Hamaguchi Y, Kaji K, et al. Myositis-specific anti-155/140 autoantibodies target
transcription intermediary factor 1 family proteins. Arthritis Rheum 2012; 64:513.
86. Satoh M, Chan JY, Ross SJ, et al. Autoantibodies to transcription intermediary factor TIF1β
associated with dermatomyositis. Arthritis Res Ther 2012; 14:R79.
87. Kaji K, Fujimoto M, Hasegawa M, et al. Identification of a novel autoantibody reactive with 155
and 140 kDa nuclear proteins in patients with dermatomyositis: an association with malignancy.
Rheumatology (Oxford) 2007; 46:25.
88. Chinoy H, Fertig N, Oddis CV, et al. The diagnostic utility of myositis autoantibody testing for
predicting the risk of cancer-associated myositis. Ann Rheum Dis 2007; 66:1345.
89. Gold HL, Smith GP. Clinical significance of p155 antibody in dermatomyositis. Osteoarthritis
Dermatol 2013; 1:1.
90. Betteridge ZE, Gunawardena H, Chinoy H, et al. Clinical and human leucocyte antigen class II
haplotype associations of autoantibodies to small ubiquitin-like modifier enzyme, a
dermatomyositis-specific autoantigen target, in UK Caucasian adult-onset myositis. Ann Rheum
Dis 2009; 68:1621.
91. Nimelstein SH, Brody S, McShane D, Holman HR. Mixed connective tissue disease: a subsequent
evaluation of the original 25 patients. Medicine (Baltimore) 1980; 59:239.
92. May DA, Disler DG, Jones EA, et al. Abnormal signal intensity in skeletal muscle at MR imaging:
patterns, pearls, and pitfalls. Radiographics 2000; 20 Spec No:S295.

Topic 5159 Version 17.0

- Page 17 of 18 -
Clinical manifestations of dermatomyositis and polymyositis in adults

© 2018 UpToDate, Inc. All rights reserved.

- Page 18 of 18 -