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Clinical and Experimental Rheumatology Feb 2022Juvenile onset idiopathic inflammatory myopathy (IIM) has many similarities and distinct differences from adult-onset disease. This review will focus on recent... (Review)
Review
Juvenile onset idiopathic inflammatory myopathy (IIM) has many similarities and distinct differences from adult-onset disease. This review will focus on recent developments in understanding and treatment of juvenile dermatomyositis (JDM), the most common disease sub-type of IIM in childhood. JDM is a systemic immune mediated vasculopathy, increasingly recognised as a group of distinct phenotypes with variable presentation and outlook. This overview will describe long-term outlook and disease course including health-related quality of life and emerging treatments.
Topics: Dermatomyositis; Disease Progression; Humans; Myositis; Quality of Life; Vascular Diseases
PubMed: 35225221
DOI: 10.55563/clinexprheumatol/56ilob -
The New England Journal of Medicine Jun 2021
Topics: Aged; Dermatomyositis; Exanthema; Female; Humans; Skin
PubMed: 34161709
DOI: 10.1056/NEJMicm2033425 -
Current Pediatric Reviews 2021Juvenile dermatomyositis is the most common inflammatory myopathy in the pediatric age group and a major cause of mortality and morbidity in individuals with childhood... (Review)
Review
BACKGROUND
Juvenile dermatomyositis is the most common inflammatory myopathy in the pediatric age group and a major cause of mortality and morbidity in individuals with childhood rheumatic diseases. Mounting evidence suggests that early diagnosis and timely aggressive treatment are associated with better outcomes.
OBJECTIVE
The purpose of this article is to provide readers with an update on the evaluation, diagnosis, and the treatment of juvenile dermatomyositis.
METHODS
A PubMed search was performed in Clinical Queries using the key term "juvenile dermatomyositis" in the search engine. The search strategy included meta-analyses, randomized controlled trials, clinical trials, observational studies, and reviews. The search was restricted to English literature. The information retrieved from the above search was used in the compilation of the present article.
RESULTS
Juvenile dermatomyositis is a chronic autoimmune inflammatory condition characterized by systemic capillary vasculopathy that primarily affects the skin and muscles with possible involvement of other organs. In 2017, the European League Against Rheumatism (EULAR) and the American College of Rheumatology (ACR) developed diagnostic criteria for juvenile idiopathic inflammatory myopathies and juvenile dermatomyositis. In the absence of muscle biopsies which are infrequently performed in children, scores (in brackets) are assigned to four variables related to muscle weakness, three variables related to skin manifestations, one variable related to other clinical manifestations, and two variables related to laboratory measurements to discriminate idiopathic inflammatory myopathies from non-idiopathic inflammatory myopathies as follows: objective symmetric weakness, usually progressive, of the proximal upper extremities (0.7); objective symmetric weakness, usually progressive, of the proximal lower extremities (0.8); neck flexors relatively weaker than neck extensors (1.9); leg proximal muscles relatively weaker than distal muscles (0.9); heliotrope rash (3.1); Gottron papules (2.1); Gottron sign (3.3); dysphagia or esophageal dysmotility (0.7); the presence of anti-Jo-1 autoantibody (3.9); and elevated serum levels of muscle enzymes (1.3). In the absence of muscle biopsy, a definite diagnosis of idiopathic inflammatory myopathy can be made if the total score is ≥7.5. Patients whose age at onset of symptoms is less than 18 years and who meet the above criteria for idiopathic inflammatory myopathy and have a heliotrope rash, Gottron papules or Gottron sign are deemed to have juvenile dermatomyositis. The mainstay of therapy at the time of diagnosis is a high-dose corticosteroid (oral or intravenous) in combination with methotrexate.
CONCLUSION
For mild to moderate active muscle disease, early aggressive treatment with high-dose oral prednisone alone or in combination with methotrexate is the cornerstone of management. Pulse intravenous methylprednisolone is often preferred to oral prednisone in more severely affected patients, patients who respond poorly to oral prednisone, and those with gastrointestinal vasculopathy. Other steroid-sparing immunosuppressive agents such as cyclosporine and cyclophosphamide are reserved for patients with contraindications or intolerance to methotrexate and for refractory cases, as the use of these agents is associated with more adverse events. Various biological agents have been used in the treatment of juvenile dermatomyositis. Data on their efficacy are limited, and their use in the treatment of juvenile dermatomyositis is considered investigational.
Topics: Antibodies, Antinuclear; Child; Dermatomyositis; Humans; Methotrexate; Myositis; Skin; United States
PubMed: 33902423
DOI: 10.2174/1573396317666210426105045 -
CMAJ : Canadian Medical Association... Mar 2019
Topics: Dermatomyositis; Humans; Male; Middle Aged
PubMed: 30910882
DOI: 10.1503/cmaj.180947 -
Current Rheumatology Reports Dec 2019Juvenile dermatomyositis is a heterogeneous disease with variable clinical outcomes. Here, we describe the recognised subtypes of idiopathic inflammatory myositis which... (Review)
Review
PURPOSE OF REVIEW
Juvenile dermatomyositis is a heterogeneous disease with variable clinical outcomes. Here, we describe the recognised subtypes of idiopathic inflammatory myositis which occur in children, with particular reference to disease-associated autoantibodies.
RECENT FINDINGS
Large cohort studies have demonstrated that myositis autoantibodies are common in juvenile dermatomyositis and can be found in the majority of patients. They identify homogenous clinical subgroups and inform prognosis, particularly the risks of developing interstitial lung disease. Descriptions of immune-mediated necrotising myositis in juvenile patients have highlighted a rare but important clinical subset typically associated with severe muscle disease and treatment resistance. It is increasingly apparent that autoantibodies can provide detailed information on prognosis and the likely disease associations in those with juvenile dermatomyositis. Further work is needed to establish how this knowledge should influence our approach to treatment.
Topics: Child; Dermatomyositis; Humans
PubMed: 31828535
DOI: 10.1007/s11926-019-0871-4 -
The American Journal of Medicine Aug 2022
Topics: Autoantibodies; Dermatomyositis; Humans; Paraneoplastic Syndromes
PubMed: 35219689
DOI: 10.1016/j.amjmed.2022.01.016 -
Modern Rheumatology Nov 2018Idiopathic inflammatory myopathies (IIMs) are heterogeneous disorders that affect the skeletal muscles. Polymyositis, dermatomyositis, and inclusion body myositis are... (Review)
Review
Idiopathic inflammatory myopathies (IIMs) are heterogeneous disorders that affect the skeletal muscles. Polymyositis, dermatomyositis, and inclusion body myositis are major IIM subsets. Immune-mediated necrotizing myopathy became recognized as a potentially new IIM subset. Since the new classification criteria published by the International Myositis Classification Criteria Project have higher sensitivity and specificity for IIM classification and subclassification than the previous criteria, they should help precise diagnosis. It should be noted that several tests available in current clinical practice, such as electromyography, magnetic resonance imaging, and other myositis-specific autoantibodies than anti-Jo-1 antibodies, were not included in the new criteria. As for treatment, glucocorticoids are used empirically as the first-line treatment despite their various adverse effects. Concomitant treatment with steroid-sparing immunosuppressive agents, including methotrexate, azathioprine, calcineurin inhibitors, mycophenolate mofetil, and cyclophosphamide, reduces successfully initial glucocorticoid doses for the remission induction, the relapse risk during glucocorticoid tapering, and adverse effects of glucocorticoids. Treatment with biologics, including rituximab and abatacept, seems promising in some IIM patients. Multi-target treatment with glucocorticoids and several steroid-sparing immunosuppressive agents is effective in refractory IIM patients. Considering proven steroid-sparing efficacy and tolerability of multi-target treatment in patients with other autoimmune diseases, it should be a good therapeutic option for IIMs.
Topics: Anti-Inflammatory Agents; Dermatomyositis; Humans; Polymyositis
PubMed: 29669460
DOI: 10.1080/14397595.2018.1467257 -
Revista Medica Del Instituto Mexicano... Jan 2023Dermatomyositis positive anti-melanoma differentiation-associated gene 5 (anti-MDA5 DM) is a rare disease that represents less than 2%. The prevalence of anti-MDA5 DM... (Review)
Review
Dermatomyositis positive anti-melanoma differentiation-associated gene 5 (anti-MDA5 DM) is a rare disease that represents less than 2%. The prevalence of anti-MDA5 DM ranges from 7 to 60%, with higher prevalence in Asian (11-60%) and women. The clinical picture may be variable and is accompanied by the typical features of dermatomyositis, such as periorbital heliotrope (blue-purple) rash with edema, erythematous rash on the face, or the anterior chest (in a V-sign), and back and shoulders (in a shawl sign), violaceous papules or plaques located on the dorsal part of the metacarpophalangeal or interphalangeal joints, which are pathognomonic by definition; yet, one of the most striking signs is the painful ulceration skin that is found in 82% of cases, which is deep and in punching holes or showing hyperkeratotic crusts. For diagnosis is necessary the typical DM rashes (Gottron's papules or Gottron's sign and heliotrope rash), along with either an "interface dermatitis" skin pathology or evidence of myositis or a MSA (myositis-specific autoantibodies). Immunoprecipitation is the gold standard method to detect MSA. Combinations of glucocorticoids and immunosuppressants are used for treatment; besides, it is necessary the detection of rapidly progressive interstitial disease (RP-ILD) with a high-resolution CT because of its high association with fatal prognosis.
Topics: Humans; Female; Dermatomyositis; Myositis; Prognosis; Exanthema; Autoantibodies
PubMed: 36542793
DOI: No ID Found -
Der Hautarzt; Zeitschrift Fur... Aug 2015Dermatomyositis is a rare idiopathic inflammatory myopathy that affects adults and children, mostly female. Hallmarks of the disease are myositis with necrosis,... (Review)
Review
Dermatomyositis is a rare idiopathic inflammatory myopathy that affects adults and children, mostly female. Hallmarks of the disease are myositis with necrosis, regeneration and perifascicular atrophy accompanied by a typical skin rash with heliotrope erythema, Gottron's sign, Gottron's papules and nail fold changes with splinter hemorrhage. Typical skin symptoms may appear 6 months up to 2 years before muscle involvement (amyopathic dermatomyositis). New myositis-specific antibodies may allow clinicoserologic correlations within a heterogeneous clinical spectrum. Autoantibody profiles, subtype of myositis, overlap with other collagen vascular disorders and/or malignancy (paraneoplastic dermatomyositis) as well as age of the patients all have a considerable impact on course and prognosis. Infections, drugs and tumors may trigger activation of T and B cells, plasmacytoid dendritic cells, overproduction of type I interferons and complement-mediated endothelial cell damage resulting in vasculopathy. UV radiation may also trigger dermatomyositis. Oral corticosteroids (1.5-2.0 mg/kg body weight/day) are the mainstay of treatment until improvement of muscle symptoms and/or normalization of muscle enzymes with subsequent slow tapering. Corticosteroids may be given as monotherapy or combined with steroid-sparing immunosuppressive agents' i.e. azathioprine, methotrexate, mycophenolate mofetil or high-dose intravenous immunoglobulins. Prognosis has improved considerably since use of high-dose corticosteroids, from 50 to 90% response rate. New therapies with biologicals (anti-CD20-, anti-TNFalpha-, anti-interferon antibodies) and Janus kinase inhibitors are currently being evaluated.
Topics: Adrenal Cortex Hormones; Antibodies, Monoclonal; Dermatomyositis; Diagnosis, Differential; Humans; Immunosuppressive Agents; Molecular Targeted Therapy
PubMed: 26219226
DOI: 10.1007/s00105-015-3659-0 -
Lancet (London, England) Jan 2000Dermatomyositis is one of the idiopathic inflammatory myopathies with characteristic cutaneous manifestations including the heliotrope rash, Gottron's papules, cuticular... (Review)
Review
Dermatomyositis is one of the idiopathic inflammatory myopathies with characteristic cutaneous manifestations including the heliotrope rash, Gottron's papules, cuticular changes including periungual telangiectasia, a photodistributed erythema or poikiloderma, and a scaly alopecia. Dermatomyositis has been linked to cancer, particularly ovarian cancer. Cancer-associated disease is more commonly found in older patients, and when present, is associated with a poor prognosis. A childhood form of the disease exists and is frequently complicated by the development of calcinosis. Dermatomyositis is a systemic disorder and whereas the skin and muscles are the most commonly affected organs, patients may have arthralgias, arthritis, oesophageal disease, or cardiopulmonary dysfunction. Recently described serological abnormalities, known as myositis-specific antibodies, add credence to the notion that this disorder is distinct from all other collagen-vascular diseases, and may lead to important discoveries about the pathogenesis of the inflammatory myopathies, which are not currently of practical use in the clinic or office. Management of the patient with myositis usually includes systemic corticosteroids with or without an immunosuppressive agent. Cutaneous disease is more difficult to manage, but antimalarials, methotrexate, and intravenous immunoglobulin are effective in small, often open-label, studies.
Topics: Adrenal Cortex Hormones; Adult; Child; Dermatomyositis; Humans; Neoplasms; Polymyositis; Prognosis
PubMed: 10615903
DOI: 10.1016/S0140-6736(99)05157-0