Materiały źródłowe

• #1 Dermatomyositis diagnosis & treatment for optimal prognosis

  https://managedermatomyositis.com/diagnosis-of-dermatomyositis/

  Early diagnosis and treatment of dermatomyositis is essential for optimal prognosis. Delaying treatment of dermatomyositis can lead to irreversible muscle damage and persistent muscle weakness. Diagnosis of dermatomyositis is challenging because of the heterogeneity between myositis subtypes, and overlap with symptoms from a number of other diseases. There is a lack of clear diagnostic criteria for idiopathic inflammatory myopathies. Additionally, multisystem involvement can complicate the diagnostic process, with wide variation in how the different subtypes manifest. Diagnosis of idiopathic inflammatory myopathies includes assessment of the clinical history, disease progression, and pattern of muscle involvement and/or cutaneous features. Except for some cases of dermatomyositis that have a typical presentation, diagnosis is usually not straightforward and requires a combination of tests. A number of diagnostic tests are available for patients where dermatomyositis is suspected, including serum muscle enzymes (e.g. creatine kinase), myositis antibodies, electrodiagnostic studies, muscle biopsy and muscle MRI. Muscle biopsy is the gold standard for diagnosis of dermatomyositis. Several criteria have been developed to diagnose dermatomyositis, including the Bohan and Peter Criteria; however, these have not been validated. Although the Bohan and Peter Criteria demonstrate a high degree of accuracy, they have limitations. For example, they do not include the use of myositis-specific autoantibodies to identify the specific idiopathic inflammatory myopathy subtype, which are important for diagnosis. More recently, EULAR/ACR classification criteria were developed for idiopathic inflammatory myopathies and these criteria have been partially validated and been shown to generally perform better than existing criteria. Heterogeneity between myositis subtypes, the non-specific nature of symptoms, and multisystem involvement make diagnosis of dermatomyositis challenging. One of the main challenges in diagnosing dermatomyositis is that the signs and symptoms of the condition can vary widely from person to person and may be similar to those of other autoimmune disorders or muscle diseases. This can make it difficult to accurately diagnose the condition based on symptoms alone. In addition, dermatomyositis can involve multiple systems in the body, such as the skin, muscles, and lungs, which can further complicate the diagnosis.

• #1 Diagnosing Dermatomyositis | NYU Langone Health

  https://nyulangone.org/conditions/dermatomyositis/diagnosis

  Specialists at NYU Langone have the experience and resources to diagnose dermatomyositis, a rare inflammatory condition characterized by a skin rash and chronic muscle weakness. […] Doctors can often identify the signs and symptoms of dermatomyositis during a medical history and physical exam. […] Early diagnosis of dermatomyositis is important because it allows doctors to start treatment before the condition progresses. […] To diagnose the condition, a doctor asks about your health, including when you first noticed symptoms, how frequently they occur, whether you have trouble swallowing or breathing, and whether you are able to perform everyday tasks, such as brushing your teeth or getting up from a chair. […] A technician takes a small sample of blood, then sends it to a laboratory to test it for substances associated with dermatomyositis that may indicate muscle damage or inflammation.

• #1 Dermatomyositis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK558917/

  Initial testing in suspected cases of dermatomyositis should include muscle enzymes, such as creatine kinase (CK), aldolase, lactate dehydrogenase (LDH), aspartate aminotransferase (AST) and alanine aminotransferase (ALT). […] Muscle biopsy is the most accurate test to confirm the diagnosis of dermatomyositis and to exclude other causes of muscle weakness or skin rash. However, choosing the right muscle for a biopsy is crucial to prevent a missing diagnosis. […] The recently formulated European League Against Rheumatism and the American College of Rheumatology (EULAR/ACR) criteria for idiopathic inflammatory myopathies is a highly sensitive and specific probability-based classification system which can be used to identify cases of dermatomyositis. Initial workup for patients with suspected dermatomyositis includes testing for muscle enzymes and myositis specific autoantibodies.

• #1 Dermatomyositis: Practical Guidance and Unmet Needs

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10924937/

  Once a patient meets EULAR/ACR criteria for IIM, presence of heliotrope rash, Gottron papules, or Gottrons sign accurately subclassifies patients with DM. Objective muscle involvement, defined as abnormal manual muscle testing or other objective strength testing, further categorizes CDM patients. […] Due to requirement of skin manifestations for DM diagnosis and easy accessibility of the skin, one of the most common and valuable procedures in a diagnostic workup is a lesional cutaneous punch biopsy. The classic histologic findings in DM skin biopsies include vacuolar interface dermatitis with keratinocyte dyskeratosis, increased reticular dermal mucin, superficial dermal vascular dilatation, and variably dense perivascular lymphohistiocytic inflammation. […] Patients suspected of having DM should be screened for MAAs, including ANA and anti-extractable nuclear antigen (ENA) antibodies such as anti-SSA (Ro).

• #1

  https://www.healio.com/clinical-guidance/dermatomyositis/diagnostic-testing-presentation-and-diagnosis

  Key diagnostic tests for the diagnosis of DM include: […] Muscle enzymes including creatine kinase and aldolase […] Myositis-specific antibodies (MSA) (antisynthase antibodies, anti-signal recognition particle, anti-Mi-2, antii-MDA5, anti-TIF-1 gamma, and NXP2) […] Myositis associated autoantibodies (MAA) l (Anti-U1 ribonucleoprotein, anti-Ku, anti-PM-Scl) […] Skin biopsy if classic rash is not evident […] Electromyography to evaluate for myopathic findings […] Muscle MRI T2 SITR to evaluate for muscle edema […] Muscle biopsy. […] A common diagnostic procedure is a lesional cutaneous punch biopsy (skin biopsy). Skin biopsy may be helpful if findings from physical examinations are subtle or atypical. […] Muscle biopsy can confirm the diagnosis of DM and to exclude other causes of muscle weakness.

• #1 Dermatomyositis Workup: Approach Considerations, Laboratory Studies, Imaging Studies

  https://emedicine.medscape.com/article/332783-workup

  Circulating autoantibodies characteristic of dermatomyositis have been identified and may be helpful in the classification of subtypes for prognosis, but they are not used for routine diagnosis. […] A positive antinuclear antibody (ANA) finding is common in patients with dermatomyositis, but is not necessary for diagnosis. […] MRI may be useful in assessing for the presence of an inflammatory myopathy in patients without weakness. It can assist in differentiating steroid myopathy from continued inflammation and may serve as a guide in selecting a muscle biopsy site. […] CT scanning of the chest, abdomen, and pelvis is useful in the evaluation of potential malignancy that might be associated with dermatomyositis. […] Muscle biopsy, either open or via needle, may enhance the clinician’s ability to diagnose dermatomyositis. The biopsy results may be useful in differentiating steroid myopathy from active inflammatory myopathy when patients have been on corticosteroid therapy but are still weak.

• #1 Dermatomyositis: Symptoms, Causes, and Treatments

  https://www.webmd.com/skin-problems-and-treatments/what-is-dermatomyositis

  Spontaneous pain sudden pain without a noticeable cause […] Signs that there is an inflammation in your body, such as fever […] Arthritis that doesnt destroy the joint […] Blood tests that show high levels of certain enzymes or autoantibodies that are specific to the disorder, or show that theres inflammation somewhere in your body […] EMG test that shows specific muscle changes […] Muscle biopsy findings that show muscle damage caused by inflammation

• #1 Diagnosing Dermatomyositis | NYU Langone Health

  https://nyulangone.org/conditions/dermatomyositis/diagnosis

  To confirm a diagnosis of dermatomyositis, our doctors may perform a punch biopsy, in which a dermatologist uses a device that resembles a pencil to puncture the skin and remove a small tissue sample. […] An electromyogram (EMG) can reveal whether dermatomyositis has caused muscles to become less responsive to nerve impulses, which normally trigger muscle movement. […] An MRI scan uses a magnetic field and radio waves to create computerized, three-dimensional images of structures inside the body. […] After a doctor has confirmed that you have dermatomyositis, he or she almost always recommends further testing to screen for medical conditions commonly associated with dermatomyositis. […] Chronic inflammation can cause changes in the body’s cells, putting adults with dermatomyositis at risk for cancer in the first three to five years after diagnosis.

• #1 Dermatomyositis: symptoms, diagnosis and treatment

  https://www.synlab-sd.com/en/blog/health-and-wellness-en/dermatomyositis-its-relationship-with-immunity-and-the-advancement-of-clinical-analysis/

  How Is Dermatomyositis Diagnosed? The diagnosis of dermatomyositis is based on the presence of clinical manifestations, complemented by laboratory investigations, including: […] Laboratory tests: Muscle enzyme levels, such as creatine phosphokinase (CK), lactate dehydrogenase (LDH), aldolase, and transaminases (AST and ALT), are usually elevated. The assessment of myositis-specific autoantibodies is also employed. […] Imaging tests: Ultrasound and magnetic resonance imaging (MRI) can help locate the best muscle group for biopsy. […] Biopsy: Muscle biopsy, skin biopsy, and electromyography (EMG) are generally performed in cases with atypical presentation or when the diagnosis is uncertain. […] The variability in dermatomyositis clinical presentation poses a significant diagnostic challenge. Due to the considerable heterogeneity among patients, the European League Against Rheumatism and the American College of Rheumatology established new classification criteria in 2017 for idiopathic inflammatory myopathies in both adults and children, including their major subgroups.

• #1 Polymyositis / Dermatomyositis Diagnosis and Treatment – Autoimmune Disorders for Medicine

  https://www.picmonic.com/pathways/medicine/courses/standard/pathology-196/autoimmune-disorders-39340/polymyositis-dermatomyositis-diagnosis-and-treatment_2640

  Electromyography, or EMG, is a diagnostic test that uses electrical energy to demonstrate muscular activity. Either a surface or needle electrode is placed on or within the muscle to trigger and measure electrical activity produced by a muscle; these measurements are then used to generate an electromyogram. Patients with DM or PM have weak muscle fibers, and therefore abnormal EMG findings, like spontaneous fibrillations and repetitive discharges. EMG serves to differentiate myopathies from neuropathic disorders like myasthenia gravis. […] For a definitive diagnosis of PM or DM, a muscle biopsy provides positive confirmation. Biopsy for PM shows CD8+ T-cell infiltration, along with necrotic muscle fibers. Muscle biopsy for DM shows CD4+ T-cell infiltration along with perifascicular atrophy, and a skin biopsy may also support the etiology of the cutaneous features.

• #1 Dermatomyositis: Practical Guidance and Unmet Needs

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10924937/

  MSAs are detected in 60% of DM patients, and incidence may approach 80-90% with appropriate assays. Importantly, presence of 1 MSA in the same individual is rare, increasing their usefulness as biomarkers for specific DM phenotypes. […] If clinical suspicion for DM is high, but clinical features and/or serologic data concerning myositis remain equivocal, EMG studies may be considered and show abnormalities supporting diagnosis in ~70-90% of DM cases. […] Although muscle biopsy has classically been a key diagnostic procedure in patients with suspected DM, its invasive nature coupled with the discovery of MSAs, predictive value of serologic muscle inflammation markers, pathognomonic cutaneous manifestations, and hallmark cutaneous histologic features have made it less commonly utilized.

• #1 Dermatomyositis – Diagnosis & treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/dermatomyositis/diagnosis-treatment/drc-20353192

  If your doctor suspects that you have dermatomyositis, he or she might suggest some of the following tests: […] A blood test will let your doctor know if you have elevated levels of muscle enzymes that can indicate muscle damage. A blood test can also detect autoantibodies associated with different symptoms of dermatomyositis, which can help in determining the best medication and treatment. […] A small piece of skin or muscle is removed for laboratory analysis. A skin sample can help confirm the diagnosis of dermatomyositis. A muscle biopsy might reveal inflammation in your muscles or other problems, such as damage or infection. If the skin biopsy confirms the diagnosis, a muscle biopsy might not be necessary. […] For dermatomyositis, questions to ask your doctor include: What tests might I need? Are special preparations required? […] Your doctor will likely ask you questions, such as: What, if anything, seems to improve your symptoms?

• #1 Dermatomyositis Workup: Approach Considerations, Laboratory Studies, Imaging Studies

  https://emedicine.medscape.com/article/332783-workup

  Findings on muscle biopsy can be diagnostic. Muscle biopsy in patients with dermatomyositis reveals perivascular and interfascicular inflammatory infiltrates with adjoining groups of muscle fiber degeneration/regeneration. […] Although inflammation is the histologic hallmark of dermatomyositis, polymyositis, and inclusion-body myositis, dermatomyositis is the only 1 of the 3 that shows perifascicular atrophy. […] The Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI) is a clinical tool used to assess disease activity in cutaneous dermatomyositis.

• #1 Adult-onset dermatomyositis

  https://dermnetnz.org/topics/adult-onset-dermatomyositis

  Dermatomyositis is an idiopathic inflammatory myopathy characterised by skeletal muscle weakness and skin changes. […] The diagnosis of dermatomyositis is suggested by the clinical features, and confirmed on investigations. […] A skin biopsy of the rash shows an interface dermatitis similar to cutaneous lupus erythematosus, so histology alone cannot be used to distinguish the two conditions. […] Blood tests assess the myositis and autoantibody subsets. […] Further investigation of the muscle disease may require electromyography (EMG), muscle biopsy, and magnetic resonance imaging (MRI). […] Age-appropriate investigations for an underlying malignancy should be considered after careful history and general examination. […] Dermatomyositis may need to be distinguished from other idiopathic inflammatory myopathies, such as polymyositis or inclusion body myositis.

• #1 Dermatomyositis: Practical Guidance and Unmet Needs

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10924937/

  Dermatomyositis is a heterogeneous idiopathic inflammatory myopathy associated with various cutaneous manifestations and variable presence of myositis, interstitial lung disease, and other visceral organ involvement. An accurate diagnosis of dermatomyositis requires correlating clinical examination findings with serological and histological findings. Familiarity with pathognomonic and common cutaneous manifestations of dermatomyositis, which are highlighted here, can be especially helpful in making an accurate diagnosis. Additionally, evaluating patients for presence of myositis-specific autoantibodies can further support or refute a dermatomyositis diagnosis. […] Initial evaluation for suspected DM should begin with a thorough history and physical examination, including total body skin examination and muscle strength testing of extremities and neck flexors. Historically, DM was clinically classified by the Bohan and Peter criteria, which divided IIMs into five groups: polymyositis (PM), DM, DM/PM associated with cancer, childhood DM/PM, and PM/DM with associated collagen-vascular disease. They described five major criteria to define DM: (1) symmetric weakness of limb-girdle muscles and anterior neck flexors; (2) muscle biopsy evidence of myositis; (3) elevation in serum skeletal-muscle enzymes; (4) a triad of needle EMG findings; and (5) presence of dermatologic features including heliotrope rash, Gottrons sign, and erythematous dermatitis involving the face, neck, and upper torso.

• #1 Diagnosis and differential diagnosis of dermatomyositis and polymyositis in adults – UpToDate

  https://www.uptodate.com/contents/diagnosis-and-differential-diagnosis-of-dermatomyositis-and-polymyositis-in-adults

  Diagnosis and differential diagnosis of dermatomyositis and polymyositis in adults […] The diagnosis and differential diagnosis of DM and „PM” in adults will be reviewed here. […] Dermatomyositis (DM) and polymyositis (PM) are idiopathic inflammatory myopathies, characterized by the shared features of proximal skeletal muscle weakness and evidence of muscle inflammation. […] A form of DM, termed clinically amyopathic DM (CADM, also known as dermatomyositis sine myositis), is a condition in which patients have characteristic skin findings of DM without objective muscle weakness. […] There is growing understanding that the term PM is falling out of favor given the increased understanding that many cases that would previously have been classified as PM are now better classified as the antisynthetase syndrome (without a characteristic rash), overlap myositis (with another connective tissue disease), or immune-mediated necrotizing myopathy (IMNM). […] While it is now understood that muscle biopsy features are distinct in IMNM, previous studies often labeled these cases as PM, assuming the primary inflammation had been missed on the muscle biopsy sampling.

• #1 Amyopathic dermatomyositis: Causes, symptoms, and more

  https://www.medicalnewstoday.com/articles/amyopathic-dermatomyositis

  How do doctors diagnose amyopathic dermatomyositis? […] Information from the 2019 review states that the European League Against Rheumatism and American College of Rheumatology method for diagnosing ADM requires a doctor to check a person for Gottron papules, Gottron sign, and heliotrope rash. […] If a person has two out of three of these symptoms alongside no muscular symptoms, a doctor may diagnose ADM. They may also diagnose the condition if a person has one of the main criteria and two minor criteria, such as itching and calcinosis. […] During the diagnosis of ADM, a doctor may ask a person about their symptoms and medical history. After examining a persons skin, a doctor may perform certain blood tests. The presence of anti-melanoma differentiation-associated protein 5 (MDA5) antibodies in a persons blood may indicate that they have ADM. […] A doctor may also test the levels of muscle enzymes in a persons blood. Elevated levels of these enzymes may indicate that muscle fibers have been damaged. This would mean that a person had dermatomyositis rather than ADM.

• #1 Orphanet: Juvenile dermatomyositis

  https://www.orpha.net/en/disease/detail/93672

  Diagnosis is based on the clinical signs and magnetic resonance imaging (MRI) of muscle. Muscle and skin involvements should be evaluated with specific standardized tools. Muscle biopsy and electromyographic testing may also be used, especially in case of atypical JDM. Muscle enzymes (creatine kinase) may be elevated. In JDM, 65% of the patients have myositis-specific antibodies with different prevalence compared to adult onset DM (20-25% with anti-TIF1-, 18-20% with anti-NXP2, 4-10% with anti-Mi2). Lung and cardiac involvement should be assessed at diagnosis. […] Differential diagnosis in JDM may include mitochondrial myopathies, infectious myopathies, other forms of inflammatory myopathies, particularly autoimmune necrotizing myopathy, as well as Duchenne muscular dystrophy or Becker muscular dystrophy, systemic lupus erythematosus, and juvenile idiopathic arthritis. Monogenic interferonopathies, such as proteasome-associated autoinflammatory syndrome and STING-associated vasculopathy with onset in infancy syndrome, may mimic JDM.

• #1 Dermatomyositis Workup: Approach Considerations, Laboratory Studies, Imaging Studies

  https://emedicine.medscape.com/article/332783-workup

  The workup for dermatomyositis may include selected laboratory tests and diagnostic imaging (eg, magnetic resonance imaging [MRI], chest radiography, ultrasonography, electromyography [EMG], or computed tomography [CT]), as well as muscle and skin biopsy and other tests as appropriate. […] In adult patients with dermatomyositis, assessment for malignancy should be performed upon initial diagnosis and repeated at least annually for 3 years. The risk of malignancy increases with age. The exact testing order should be based on the patient’s sex, age, and race; however, testing beyond age-appropriate screening is most often recommended. […] Muscle enzyme levels are often abnormal during the course of dermatomyositis, except in patients with amyopathic dermatomyositis (ADM). The most sensitive/specific enzyme abnormality is elevated creatine kinase (CK), but aldolase studies and other tests (eg, for aspartate aminotransferase [AST] or lactic dehydrogenase [LDH]) may also yield abnormal results.

• #1 Dermatomyositis | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/dermatomyositis?lang=us

  Dermatomyositis is an idiopathic inflammatory myopathy, presumably autoimmune in etiology, which carries an increased risk of malignancy. It is considered a distinct condition to anti-synthetase syndrome. […] There is a sixfold increased risk of malignancy in dermatomyositis (cf. twofold in polymyositis). Multiple risk factors for the development of malignancy have been identified: 60 years old, male, dysphagia, necrosis of the skin, cutaneous vasculitis, accelerated onset of disease, increased creatine kinase (CK) levels, increased ESR and C-reactive protein (CRP) levels, certain antibodies in adult-onset disease: anti-TIF1, anti-NXP2. […] Management of myositis is primarily with immunosuppression. Options include corticosteroids and steroid-sparing agents (e.g. azathioprine, mycophenolate, methotrexate, etc.).

• #1

  https://www.healio.com/clinical-guidance/dermatomyositis/diagnostic-testing-presentation-and-diagnosis

  T2-weighted muscle magnetic resonance imaging (MRI) can identify areas of muscle edema in patients with normal or only mildly elevated muscle enzymes. […] Patients should undergo age-appropriate cancer screening exams such as pelvic exams, breast exams, testicular exams, and rectal exams to screen for malignancy. […] Once a DM diagnosis is made, patients should be monitored for development of visceral organ involvement and malignancy, which contribute to the patients morbidity and mortality.

• #1 Timely dermatomyositis diagnosis, treatment remain elusive | MDedge

  https://mdedge.com/rheumatologynews/article/167148/lupus-connective-tissue-diseases/timely-dermatomyositis-diagnosis

  There was a median 1-year delay between the onset of symptoms and diagnosis of classic dermatomyositis, and a 17-month delay before diagnosis of amyopathic dermatomyositis, based on a review of 232 dermatomyositis patients seen at the University of Pennsylvania, Philadelphia. […] Just 103 (44.4%) patients were diagnosed with dermatomyositis (DM) right out of the gate. Among the other 129, 48 (37.2%) were diagnosed with lupus, 38 (29.5%) with undifferentiated connective tissue disease, 10 (7.8%) went undiagnosed, and 33 (25.5%) were diagnosed with rosacea, psoriasis, rheumatoid arthritis, fibromyalgia, lichen planus, and a number of other conditions. By the time the DM diagnosis was finally confirmed, almost every patient had Gottron’s papules or sign. […] Misdiagnosis of dermatomyositis (DM) is nothing new, but the study brings home just how common the problem is, even at a major academic medical institution.

• #1 Diagnosing and treating dermatomyositis: capillaroscopy’s role

  https://en.capillary.io/posts/dermatomyositis-diagnosis/

  Doctors use a number of diagnostic measures to determine whether a person has dermatomyositis. These are the typical diagnostic factors. […] Nailfold capillaroscopy is shown to be a potentially useful tool to assess disease activity in new-onset of dermatomyositis. Researchers found that the number of enlarged capillaries was positively correlated with the patients symptoms, observed by the clinician. […] Nailfold capillaroscopy is a good tool for diagnosing and monitoring autoimmune diseases like dermatomyositis, as long as the interpretations are consistent and accurate.

• #1 Dermatomyositis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK558917/

  Early recognition and prompt initiation of treatment can reduce the morbidity and mortality of patients. The first-line treatment for dermatomyositis is prednisolone. […] Concomitant treatment with glucocorticoids and immunosuppressants should be given to patients who are resistant to treatment with glucocorticoids alone or have extra muscular complications or severe weakness.

• #1 :: AD :: Annals of Dermatology

  https://anndermatol.org/DOIx.php?id=10.5021/ad.24.022

  In patients with an initial CDASI-A score 14 points, a 40% change in the CDASI-A score was found to indicate a meaningful change in QoL. […] The TIS was calculated based on the levels of improvement and relative weights of each CSM. […] Despite its usefulness to discriminate responses in active myositis, its applicability and sensitivity in skin-predominant DM is less straightforward. […] The DM Outcomes for Muscle and Skin (DMOMS) allows equal weighting of skin and muscle disease and incorporates many aspects of the TIS used in the intravenous immunoglobulin (IVIG) trial. […] The use of IVIG is not entirely new. […] The mechanism of action of IVIG in autoimmune diseases is still not clearly elucidated. […] This was the key trial for the US Food and Drug Administration (FDA) approval of IVIG for the treatment of DM.

• #1 Dermatomyositis – Symptoms, diagnosis and treatment | BMJ Best Practice US

  https://bestpractice.bmj.com/topics/en-us/595

  Dermatomyositis (DM) is an idiopathic autoimmune inflammatory myopathy characterized by distinctive skin manifestations. Diagnosis is based on the presence of a symmetric proximal myopathy, elevated muscle enzymes, myopathic changes on electromyography, a characteristic muscle biopsy, and a typical skin rash (e.g., periorbital dusky violaceous erythema, or macular violaceous erythema such as in V, shawl, and Gottron signs). […] Some patients present with typical cutaneous manifestations but have delayed-onset, subclinical, or absent muscle disease. […] Key diagnostic factors include Gottron papules, heliotrope rash with or without periorbital edema, macular violaceous erythema, periungual erythema, nail-fold capillary dilation, cuticular overgrowth, „mechanic’s” hands, and proximal muscle weakness. […] Diagnostic tests include serum creatine kinase (CK), serum aldolase, muscle biopsy, electromyogram (EMG), skin biopsy, antinuclear antibody (ANA), and myositis-specific antibodies (MSAs) and myositis-associated antibodies (MAAs).

• #1 Dermatomyositis | AAFP

  https://www.aafp.org/pubs/afp/issues/2001/1101/p1565.html

  Dermatomyositis is an idiopathic inflammatory myopathy with characteristic skin manifestations. […] Early recognition and treatment are important ways to decrease the morbidity of systemic complications. […] Based on the classification system, diagnostic criteria were developed to further assist in the confidence of the diagnosis. […] Recommended tests that should be performed during the initial evaluation are listed in Table 6. […] Common laboratory manifestations of dermatomyositis are listed in Table 7. […] A complete history should be obtained and a physical examination performed, including a thorough review of systems, with an emphasis on myositis-related presentations and evidence of skin changes. […] Although an increased risk of malignancy has not been associated with juvenile dermatomyositis, it has been demonstrated in adults with dermatomyositis.

• #2 Dermatomyositis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK558917/

  Dermatomyositis is a rare condition that causes muscle inflammation. It presents with symmetric proximal muscle weakness, skin rash, and extramacular manifestations, such as esophageal dysfunction and interstitial lung disease. Dermatomyositis is strongly associated with malignancy, especially in adults. This activity outlines the evaluation and management of dermatomyositis and highlights the role of the interprofessional team in improving care for patients with this condition. […] Dermatomyositis is a rare acquired immune-mediated muscle disease characterized by muscle weakness and skin rash. It is classified as one of the idiopathic inflammatory myopathies (IIM). […] A comprehensive history and physical exam should be conducted in suspected cases of dermatomyositis, keeping in mind the following objectives: Identify the typical muscular and cutaneous signs and symptoms of dermatomyositis.

• #2 Dermatomyositis Workup: Approach Considerations, Laboratory Studies, Imaging Studies

  https://emedicine.medscape.com/article/332783-workup

  The workup for dermatomyositis may include selected laboratory tests and diagnostic imaging (eg, magnetic resonance imaging [MRI], chest radiography, ultrasonography, electromyography [EMG], or computed tomography [CT]), as well as muscle and skin biopsy and other tests as appropriate. […] In adult patients with dermatomyositis, assessment for malignancy should be performed upon initial diagnosis and repeated at least annually for 3 years. The risk of malignancy increases with age. The exact testing order should be based on the patient’s sex, age, and race; however, testing beyond age-appropriate screening is most often recommended. […] Muscle enzyme levels are often abnormal during the course of dermatomyositis, except in patients with amyopathic dermatomyositis (ADM). The most sensitive/specific enzyme abnormality is elevated creatine kinase (CK), but aldolase studies and other tests (eg, for aspartate aminotransferase [AST] or lactic dehydrogenase [LDH]) may also yield abnormal results.

• #2 Dermatomyositis: Practical Guidance and Unmet Needs

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10924937/

  MSAs are detected in 60% of DM patients, and incidence may approach 80-90% with appropriate assays. Importantly, presence of 1 MSA in the same individual is rare, increasing their usefulness as biomarkers for specific DM phenotypes. […] If clinical suspicion for DM is high, but clinical features and/or serologic data concerning myositis remain equivocal, EMG studies may be considered and show abnormalities supporting diagnosis in ~70-90% of DM cases. […] Although muscle biopsy has classically been a key diagnostic procedure in patients with suspected DM, its invasive nature coupled with the discovery of MSAs, predictive value of serologic muscle inflammation markers, pathognomonic cutaneous manifestations, and hallmark cutaneous histologic features have made it less commonly utilized.

• #2 Dermatomyositis – Wikipedia

  https://en.wikipedia.org/wiki/Dermatomyositis

  The diagnosis of dermatomyositis is based on five criteria, which are also used to differentially diagnose with respect to polymyositis: […] Diagnosis is typically based on some combination of symptoms, blood tests, electromyography, and muscle biopsies. […] The fifth criterion is what differentiates dermatomyositis from polymyositis; the diagnosis is considered definite for dermatomyositis if three of items 1 through 4 are present in addition to 5, probable with any two in addition to 5, and possible if just one is present in addition to 5. […] Dermatomyositis is associated with autoantibodies, especially antinuclear antibodies (ANA). […] Around 80% of people with DM test positive for ANA and around 30% of people have myositis-specific autoantibodies which include antibodies to aminoacyl-tRNA synthetases (anti-synthetase antibodies), including antibodies against histidinetRNA ligase (Anti-Jo1); antibodies to signal recognition particle (SRP); and anti-Mi-2 antibodies.

• #2 Dermatomyositis Workup: Approach Considerations, Laboratory Studies, Imaging Studies

  https://emedicine.medscape.com/article/332783-workup

  Circulating autoantibodies characteristic of dermatomyositis have been identified and may be helpful in the classification of subtypes for prognosis, but they are not used for routine diagnosis. […] A positive antinuclear antibody (ANA) finding is common in patients with dermatomyositis, but is not necessary for diagnosis. […] MRI may be useful in assessing for the presence of an inflammatory myopathy in patients without weakness. It can assist in differentiating steroid myopathy from continued inflammation and may serve as a guide in selecting a muscle biopsy site. […] CT scanning of the chest, abdomen, and pelvis is useful in the evaluation of potential malignancy that might be associated with dermatomyositis. […] Muscle biopsy, either open or via needle, may enhance the clinician’s ability to diagnose dermatomyositis. The biopsy results may be useful in differentiating steroid myopathy from active inflammatory myopathy when patients have been on corticosteroid therapy but are still weak.

• #2

  https://www.healio.com/clinical-guidance/dermatomyositis/diagnostic-testing-presentation-and-diagnosis

  Muscle biopsy findings consistent with DM include: […] Perivascular and perimysial inflammatory infiltrate that consists of B cells, CD4+ T helper cells, macrophages, and plasmacytoid dendritic cells. […] Atrophy of muscle fibers (perifascicular atrophy), especially around fascicles, with degenerating and regenerating muscle fibers. […] Microangiopathy caused by Injury to intramuscular blood vessels on capillaries […] Complement deposition on endomysial capillaries […] Decreased capillary density. […] Muscle enzymatic markers are released into the serum when skeletal muscle is damaged, such as creatine phosphokinase (CK), aspartate aminotransferase (AST), alanine aminotransferase (ALT), lactate dehydrogenase (LDH), and aldolase. […] If clinical features and/or serologic data concerning myositis remain ambiguous and there is a suspicion of DM, electromyography (EMG) may be considered. EMG may provide information supporting diagnosis in ~70-90% of cases of DM.

• #2 Dermatomyositis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK558917/

  Initial testing in suspected cases of dermatomyositis should include muscle enzymes, such as creatine kinase (CK), aldolase, lactate dehydrogenase (LDH), aspartate aminotransferase (AST) and alanine aminotransferase (ALT). […] Muscle biopsy is the most accurate test to confirm the diagnosis of dermatomyositis and to exclude other causes of muscle weakness or skin rash. However, choosing the right muscle for a biopsy is crucial to prevent a missing diagnosis. […] The recently formulated European League Against Rheumatism and the American College of Rheumatology (EULAR/ACR) criteria for idiopathic inflammatory myopathies is a highly sensitive and specific probability-based classification system which can be used to identify cases of dermatomyositis. Initial workup for patients with suspected dermatomyositis includes testing for muscle enzymes and myositis specific autoantibodies.

• #2 Dermatomyositis: Practical Guidance and Unmet Needs | ITT

  https://www.dovepress.com/dermatomyositis-practical-guidance-and-unmet-needs-peer-reviewed-fulltext-article-ITT

  Initial evaluation for suspected DM should begin with a thorough history and physical examination, including total body skin examination and muscle strength testing of extremities and neck flexors. […] According to the Bohan and Peter criteria, a DM diagnosis is considered definite if 3 criteria and characteristic rash are present, probable if 2 criteria and rash are present, and possible if 1 criterion and rash are present. […] Once a patient meets EULAR/ACR criteria for IIM, presence of heliotrope rash, Gottron papules, or Gottrons sign accurately subclassifies patients with DM. […] Due to requirement of skin manifestations for DM diagnosis and easy accessibility of the skin, one of the most common and valuable procedures in a diagnostic workup is a lesional cutaneous punch biopsy.

• #2 Advances in Juvenile Dermatomyositis: Pathophysiology, Diagnosis, Treatment and Interstitial Lung Diseases—A Narrative Review

  https://www.mdpi.com/2227-9067/11/9/1046

  Tanimoto’s criteria have been used for the diagnosis of both adult and juvenile PM/DM in Japan, but include EMG and only anti-ARS antibodies as MSAs, which are rarely detected in JDM. […] The modified criteria also included MSAs such as anti-MDA5, anti-NXP2, and anti-TIF1-γ antibodies in addition to anti-ARS autoantibodies. […] In 2017, the European Alliance of Associations for Rheumatology and American College of Rheumatology published classification criteria for IIM, which is aimed for research use in both adults and children. […] A study of a European cohort confirmed high sensitivity and specificity and suggested that the inclusion of ILD improves sensitivity. […] Upon the diagnosis of IIM, physicians need to know that serum levels of muscle-derived enzymes may be within the normal range in CAJDM and tend toward normal in patients untreated for a long period. […] Muscle biopsy is not mandatory in (J)DM with typical skin disease, but should be performed for the diagnosis of JDM sine dermatitis, JPM, JIMNM, or JCTM/overlap myositis.

• #2 Dermatomyositis – Wikipedia

  https://en.wikipedia.org/wiki/Dermatomyositis

  Magnetic resonance imaging may be useful to guide muscle biopsy and to investigate involvement of internal organs; X-ray may be used to investigate joint involvement and calcifications. […] A given case of dermatomyositis may be classified as amyopathic dermatomyositis if only skin is affected and no muscle weakness for longer than 6 months is seen according to one 2016 review, or two years according to another.

• #2 Adult-onset dermatomyositis

  https://dermnetnz.org/topics/adult-onset-dermatomyositis

  Dermatomyositis is an idiopathic inflammatory myopathy characterised by skeletal muscle weakness and skin changes. […] The diagnosis of dermatomyositis is suggested by the clinical features, and confirmed on investigations. […] A skin biopsy of the rash shows an interface dermatitis similar to cutaneous lupus erythematosus, so histology alone cannot be used to distinguish the two conditions. […] Blood tests assess the myositis and autoantibody subsets. […] Further investigation of the muscle disease may require electromyography (EMG), muscle biopsy, and magnetic resonance imaging (MRI). […] Age-appropriate investigations for an underlying malignancy should be considered after careful history and general examination. […] Dermatomyositis may need to be distinguished from other idiopathic inflammatory myopathies, such as polymyositis or inclusion body myositis.

• #2 Diagnosing Dermatomyositis | NYU Langone Health

  https://nyulangone.org/conditions/dermatomyositis/diagnosis

  To confirm a diagnosis of dermatomyositis, our doctors may perform a punch biopsy, in which a dermatologist uses a device that resembles a pencil to puncture the skin and remove a small tissue sample. […] An electromyogram (EMG) can reveal whether dermatomyositis has caused muscles to become less responsive to nerve impulses, which normally trigger muscle movement. […] An MRI scan uses a magnetic field and radio waves to create computerized, three-dimensional images of structures inside the body. […] After a doctor has confirmed that you have dermatomyositis, he or she almost always recommends further testing to screen for medical conditions commonly associated with dermatomyositis. […] Chronic inflammation can cause changes in the body’s cells, putting adults with dermatomyositis at risk for cancer in the first three to five years after diagnosis.

• #2 Dermatomyositis (and polymyositis)

  https://www.pcds.org.uk/clinical-guidance/dermatomyositis

  Characteristic cutaneous features of dermatomyositis include a purple-red heliotrope facial rash, affecting the eyelids, upper cheeks, forehead and temples, frequently associated with oedema of the eyelids and periorbital skin. […] The rash can involve the nasolabial folds, which can help to distinguish DM from cutaneous LE, which spares this site. […] The most commonly affected organs are the cervix, lungs, pancreas, breasts, ovaries and gastrointestinal tract. […] Malignancy is only considered in adult dermatomyositis. […] The risk is lower for amyopathic dermatomyositis. […] Clinical features associated with an increased risk include age 45 at diagnosis, male gender, dysphagia, cutaneous necrosis, cutaneous vasculitis, and rapid onset of skin/muscle symptoms. […] Patients with TIF1 or NXP-2 antibodies (both myositis specific antibodies) have a high-risk of malignancy requiring thorough investigations including radiology.

• #2 Dermatomyositis | AAFP

  https://www.aafp.org/pubs/afp/issues/2001/1101/p1565.html

  Therefore, careful investigation for malignancy should be initiated at the time dermatomyositis is diagnosed. […] The goal is to improve function and prevent disability. […] The treatment regimen must be instituted early and requires a team approach between the physical therapist, dermatologist and family physician. […] Prednisone remains the initial oral pharmacologic agent, given in a single daily dose of 0.5 to 1.5 mg per kg until the serum creatine kinase (CK) level is normalized and then slowly tapered over the following 12 months. […] If no improvement in objective muscle strength occurs after three months of prednisone therapy, other immunosuppressive therapy should be considered. […] Poor prognostic indicators include recalcitrant disease, delay in diagnosis, older age, malignancy, fever, asthenia-anorexia, pulmonary interstitial fibrosis, dysphagia and leukocytosis.

• #2 Improving Dermatomyositis Diagnosis in Black Adults – Dermatomyositis and Other Inflammatory Myopathies

  https://www.medpagetoday.com/resource-centers/dermatomyositis-other-inflammatory-myopathies/improving-dermatomyositis-diagnosis-black-adults/4710

  Dermatomyositis (DM) is an autoimmune disease that causes inflammation, affecting the skin and muscles in varying ways; it can also lead to systemic manifestations, such as interstitial lung disease (ILD), cardiomyopathy/myocarditis, and several cancers. […] The muscular signs and symptoms are symmetric proximal muscle weakness and soreness. […] In this series, 93% of the patients had proximal muscle weakness, as evidenced by muscle biopsy and increased muscle enzymes, in addition to findings on magnetic resonance imaging and electromyography. […] Their goal was to aid in reducing the incidence of diagnostic delays and misdiagnoses. […] The authors suggest that these changes may be there but may be more subtle and, therefore, easier to miss, leading to diagnosis delay or misdiagnosis.

• #2 Timely dermatomyositis diagnosis, treatment remain elusive | MDedge

  https://mdedge.com/rheumatologynews/article/167148/lupus-connective-tissue-diseases/timely-dermatomyositis-diagnosis

  One of the take homes is that clinicians should never forget about the possibility of DM even when patients are diagnosed with other autoimmune diseases, and remain vigilant for erythema on the lateral thighs or nasolabial fold, Gottron’s papules, and other diagnostic giveaways, the researchers said. […] There’s also just not enough awareness that dermatomyositis can present without the classic muscle symptoms and findings, i.e. clinically amyopathic DM. While 49 of 120 patients with classic dermatomyositis (40.8%) were misdiagnosed or undiagnosed in the study, the number rose to 80 of 112 (71.4%) among amyopathic patients.

• #2 :: AD :: Annals of Dermatology

  https://anndermatol.org/DOIx.php?id=10.5021/ad.24.022

  Dermatomyositis (DM) is a rare autoimmune connective tissue disease with characteristic skin manifestations and possible muscle involvement. […] Correct diagnosis and evaluation of treatment effects is crucial not only for an optimal patient management but also for better clinical trials to develop new medications. […] This concept caused most of older clinical trials or epidemiologic studies to group DM and PM as one disease without clear distinction, complicating analyses. […] The longstanding inappropriate exclusion of CADM from the IIM classification system caused misclassification in 55.6% of patients, with a median time of delay to proper diagnosis of about 15 months and a substandard screening for DM-associated findings, such as interstitial lung disease (ILD) or cancer. […] The CDASI detects improvements in skin disease that are significant to patients, and a higher CDASI activity score correlated well with poorer quality of life (QoL).

• #2 Dermatomyositis diagnosis & treatment for optimal prognosis

  https://managedermatomyositis.com/diagnosis-of-dermatomyositis/

  Early diagnosis and treatment of dermatomyositis is essential for optimal prognosis. Delaying treatment of dermatomyositis can lead to irreversible muscle damage and persistent muscle weakness. Diagnosis of dermatomyositis is challenging because of the heterogeneity between myositis subtypes, and overlap with symptoms from a number of other diseases. There is a lack of clear diagnostic criteria for idiopathic inflammatory myopathies. Additionally, multisystem involvement can complicate the diagnostic process, with wide variation in how the different subtypes manifest. Diagnosis of idiopathic inflammatory myopathies includes assessment of the clinical history, disease progression, and pattern of muscle involvement and/or cutaneous features. Except for some cases of dermatomyositis that have a typical presentation, diagnosis is usually not straightforward and requires a combination of tests. A number of diagnostic tests are available for patients where dermatomyositis is suspected, including serum muscle enzymes (e.g. creatine kinase), myositis antibodies, electrodiagnostic studies, muscle biopsy and muscle MRI. Muscle biopsy is the gold standard for diagnosis of dermatomyositis. Several criteria have been developed to diagnose dermatomyositis, including the Bohan and Peter Criteria; however, these have not been validated. Although the Bohan and Peter Criteria demonstrate a high degree of accuracy, they have limitations. For example, they do not include the use of myositis-specific autoantibodies to identify the specific idiopathic inflammatory myopathy subtype, which are important for diagnosis. More recently, EULAR/ACR classification criteria were developed for idiopathic inflammatory myopathies and these criteria have been partially validated and been shown to generally perform better than existing criteria. Heterogeneity between myositis subtypes, the non-specific nature of symptoms, and multisystem involvement make diagnosis of dermatomyositis challenging. One of the main challenges in diagnosing dermatomyositis is that the signs and symptoms of the condition can vary widely from person to person and may be similar to those of other autoimmune disorders or muscle diseases. This can make it difficult to accurately diagnose the condition based on symptoms alone. In addition, dermatomyositis can involve multiple systems in the body, such as the skin, muscles, and lungs, which can further complicate the diagnosis.

• #3 Dermatomyositis: Symptoms, Causes, and Treatments

  https://www.webmd.com/skin-problems-and-treatments/what-is-dermatomyositis

  Magnetic resonance imaging (MRI). Your doctor will use this to see where your muscles are inflamed. […] Skin or muscle biopsy. By removing a small part of your skin or muscle and looking at it under a microscope, your doctor can see if you have dermatomyositis. Biopsies can also rule out other diseases, like lupus. […] Dermatomyositis diagnostic criteria […] In order for your doctor to make an official dermatomyositis diagnosis, you have to meet certain criteria. Except for amyopathic dermatomyositis, which is diagnosed differently, you must have at least one of these skin symptoms for diagnosis: […] A red or purple rash with swelling on your upper eyelid, called a heliotrope rash […] Dilated capillaries (tiny blood vessels) at the skin folds around your fingernails, appearing as small red dots, called periungual telangiectasias

• #3 Diagnosis – Dermatomyositis – Diseases | Muscular Dystrophy Association

  https://www.mda.org/disease/dermatomyositis/diagnosis

  Diagnosing dermatomyositis may require a combination of testing modalities. People with the disease may exhibit: […] Characteristic pattern of muscle weakness […] Skin abnormalities (e.g., heliotrope eruption, Gottron sign, etc.) […] Elevated creatine kinase (CK) and other muscle enzymes, such as aldolase, LDH, or AST, ALT. While AST and ALT are liver enzymes, they are also enzymes that can be secreted from the muscle. […] Specific antibodies indicative of myositis or other autoimmune diseases […] Myositis-specific autoantibodies: […] Anti-synthetase antibodies such as Anti-Jo1, Anti-PL-12, Anti-Pl7, etc, […] Anti-Mi-2, Anti- NXP2, Anti-TIF-1 gamma, Anti-MDA5, Anti-SAE, (these are commonly seen in dermatomyositis) […] Assessment of electrical activity in muscles using EMG Distinctive pattern of electrical activity in affected muscles, as assessed by electromyogram […] Signs of muscle inflammation […] Perifasicular atrophy, perivascular inflammation indicative of dermatomyositis (e.g., blood vessel inflammation)

• #3 Dermatomyositis | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/dermatomyositis?lang=us

  MRI T2-weighted sequences are useful to guide muscle biopsy: areas of edema related to the active inflammatory process, non-specific end-stage fatty atrophic muscle should be avoided. Further imaging in the form of a contrast-enhanced CT of the chest, abdomen and pelvis may be undertaken to exclude an associated primary malignancy.

• #3 Improving Dermatomyositis Diagnosis in Black Adults – Dermatomyositis and Other Inflammatory Myopathies

  https://www.medpagetoday.com/resource-centers/dermatomyositis-other-inflammatory-myopathies/improving-dermatomyositis-diagnosis-black-adults/4710

  Dr Nambudiri told MedPage Today that “These findings help raise awareness for clinicians regarding key cutaneous diagnostic features of dermatomyositis in Black adults. We know there are disparities in dermatologic diagnosis and recognition of a variety of cutaneous conditions, and we hope such findings are able to help others recognize subtle morphologic changes and patterns to enhance timely diagnosis for our patients.”

• #4 Dermatomyositis: Symptoms, Causes, and Treatments

  https://www.webmd.com/skin-problems-and-treatments/what-is-dermatomyositis

  Red or purple hardened areas of skin or areas where the skin seems to be wearing away on the top parts of your finger joints or over your elbows or knees, called Gottrons sign or papules […] Red rashes, sometimes raised, that look like theyre forming a shawl pattern over your shoulders, arms, and upper back. Its also like a V-shape over the front of your neck and chest, reaching down to over the outside of your hip. This is called the shawl sign, V sign, or holster sign. […] A skin biopsy that shows lessening density or thickness in your capillaries and damage in the small blood vessels […] In addition to having something in the list above, you also must have at least four of these: […] Muscle weakness in both your shoulders, upper arms, hips, and thighs, and your trunk […] Muscle pain when you try to grasp something

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