PO203 Assessing disease activity and cardiac involvement in myositis
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Background
Methods of monitoring disease activity and detecting cardiac involvement in myositis are inadequate, contributing to poor outcomes. We investigated the potential for a panel of serum muscle damage markers to address these issues.Methods
Disease activity and cardiac involvement in adult myositis patients was assessed and serum total creatine kinase (CK), cardiac troponin-T (cTnT), cardiac troponin-I (cTnI) and CK-MB were measured. Convergent construct validity assessment using Spearman’s ranked correlation and logistic regression were used.Results
Of 46 patients, most had dermatomyositis (39%). The mean age was 52 years. 72% were female. Strongest correlations were observed between cTnT and manual muscle testing scores (rho −0.53, p<0.001), and between CK-MB and the Health Assessment Questionnaire (HAQ) (rho 0.44, p=0.002). Combining an abnormal CK and CK-MB identified a group with a significantly higher HAQ (OR 2.06, 95% CI 1.07–3.97, p=0.031), whereas this was not found in those with an abnormal CK alone. Serum muscle damage marker levels were not significantly different in those with cardiac involvement (n=8) although an abnormal cTnI had 95% specificity for cardiac involvement.Conclusion
When assessing disease activity in myositis, serum cTnT and CK-MB appear useful in addition to total CK, whereas cTnI may assist identification of cardiac involvement.Keywords:
Troponin complex
Creatine kinase
The authors describe the myositis-specific and myositis-associated antibodies (MSA-s) in idiopathic inflammatory myopathies and dermatomyositis, with particular emphasis on aspects of clinical relevancy. The frequency and clinical significance of MSA-s in 50 dermatomyositis patients at our Clinic are presented. The most common MSA, anti-TIF-1 antibodies were found in 14% of our dermatomyositis patients and they were associated with cancers, in accordance with previous reports. For patients with a high tumor risk, a comprehensive tumor research using all imaging devices should be performed.
Clinical Significance
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Troponin complex
Troponin C
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Background A small subset of patients with dermatomyositis develop the characteristic cutaneous manifestations without muscle involvement, the so-called dermatomyositis sine myositis or amyopathic dermatomyositis. Whether systemic treatment of skin disease can prevent the development of myositis remains controversial. Objective The purpose of this study was to evaluate the development of the disease in terms of the onset of muscle symptoms under systemic treatment with corticosteroids or antimalarials. Methods Five patients with dermatomyositis sine myositis were included in this study. All skin biopsy specimens had features consistent with dermatomyositis. Muscle enzymes, electromyograms and muscle biopsies did not reveal any abnormality. Results Corticosteroids were given in three patients and hydroxychloroquine in two. Both regimens proved to be effective in the treatment of skin symptoms. Four patients, three on corticosteroids and one on hydroxychloroquine, did not develop muscle disease 4–7 years after presentation. One patient on hydroxychloroquine showed laboratory evidence of myositis 6 years after initiation of treatment. Conclusion It seems that muscle disease may appear within long periods of time after the onset of the cutaneous manifestations. The term dermatomyositis sine myositis can be used as a provisional diagnosis. Our observations suggest that systemic treatment of skin disease with corticosteroids is successful and may alter the disease course. Hydroxychloroquine may be helpful in some cases.
Hydroxychloroquine
Muscle disease
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Idiopathic inflammatory dermatomyopathies have been historically defined by broad clinical and pathological criteria,whose spectrum of clinical illness includes clinically amyopathic dermatomyositis,classic dermatomyositis and polymyositis.Clinical manifestations of this entity are diverse with various degrees of muscular,cutaneous and pulmonary involvement.Myositis-specific autoantibodies and myositis-associated autoantibodies are frequently detected in sera of patients with idiopathic inflammatory dermatomyopathies,some of which are closely associated with the clinical characteristics and specific for the diagnosis of idiopathic inflammatory dermatomyopathies. Recently, some autoantibodies such as anti-clinically amyopathic dermatomyositis (CADM) 140 antibody and anti-small ubiquitin-like modifier activating enzyme (SAE) antibody have been detected in sera of patients with idiopathic inflammatory dermatomyopathies,which are not closely associated with the initiation of myositis and should not be classified as myositis-specific autoantibodies,but as an independent subset of antibodies specific for idiopathic inflammatory dermatomyopathies.
Key words:
Dermatomyositis; Autoantibodies; Myositis
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Inclusion body myositis
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Dermatomyositis is a systemic autoimmune disease which belongs to the group of idiopathic inflammatory myopathies. The disease is rare with an incidence of 0.1-1/100,000 and a prevalence of 1-6/100,000. Women are affected twice as often as men. In some patients the disease presents with dermatologic changes weeks to months before the myopathy arises. It was observed that in some patients the myositis develops much later and sometimes not at all. Therefore, the term of dermatomyositis sine myositis used in earlier literature has been changed to amyopathic dermatomyositis. The most important question is whether the patient needs systemic therapy with its possible side effects yet possibly preventing the appearance of myositis or only local therapy for the skin manifestations is necessary. The goal of this article is to summarize the latest findings in amyopathic dermatomyositis.
Muscle disease
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Abstract Background A small subset of patients with dermatomyositis develop the characteristic cutaneous manifestations without muscle involvement, the so‐called dermatomyositis sine myositis or amyopathic dermatomyositis. Whether systemic treatment of skin disease can prevent the development of myositis remains controversial. Objective The purpose of this study was to evaluate the development of the disease in terms of the onset of muscle symptoms under systemic treatment with corticosteroids or antimalarials. Methods Five patients with dermatomyositis sine myositis were included in this study. All skin biopsy specimens had features consistent with dermatomyositis. Muscle enzymes, electromyograms and muscle biopsies did not reveal any abnormality. Results Corticosteroids were given in three patients and hydroxychloroquine in two. Both regimens proved to be effective in the treatment of skin symptoms. Four patients, three on corticosteroids and one on hydroxychloroquine, did not develop muscle disease 4–7 years after presentation. One patient on hydroxychloroquine showed laboratory evidence of myositis 6 years after initiation of treatment. Conclusion It seems that muscle disease may appear within long periods of time after the onset of the cutaneous manifestations. The term dermatomyositis sine myositis can be used as a provisional diagnosis. Our observations suggest that systemic treatment of skin disease with corticosteroids is successful and may alter the disease course. Hydroxychloroquine may be helpful in some cases.
Hydroxychloroquine
Muscle disease
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Juvenile Dermatomyositis
Inflammatory myopathy
Inclusion body myositis
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Juvenile Dermatomyositis
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The idiopathic inflammatory myopathies, collectively named myositis, can be subclassified into polymyositis, dermatomyositis and inclusion body myositis. Patients with polymyositis and dermatomyositis usually respond to immunosuppressive treatment, at least with partial improvement, whereas patients with inclusion body myositis often are treatment resistant. These differences in treatment response suggest different pathogenic mechanisms and that treatment strategies should be different in these different subsets of myositis. Only a few controlled trials have been performed in patients with myositis, thus treatment recommendations are mainly based on case series and clinical experience. This lecture will include update on (1) Pharmacological and non-pharmacological treatment (exercise) for polymyositis and dermatomyositis; (2) The role of biologics in myositis (2) Outcome measures for polymyositis and dermatomyositis, and (3) Treatment recommendations for inclusion body myositis. For polymyositis and dermatomyositis there is scientific data to support that treatment should be based on a combination of immunosuppressive treatment and physical exercise. The basis of treatment is still glucocorticoids often with high starting doses e.g. 0.75- 1 mg/kg/day needed. Improvement of muscle performance is often slow and high doses of glucocorticoids may be needed for 1 month of more. As side effects of glucocorticoids are common and some patients may not respond favorably to glucocorticoid treatment alone, most experts suggest another immunosuppressive agent as glucocorticoid saving and to improve the effects of treatment. The most often used drugs are methotrexate and azathioprine. So far the experience of biologics in treatment of polymyositis and dermatomyositis is limited but the results of a placebo-controlled trial using rituximab will be discussed. Physical exercise in combination with immunosuppressive drugs has clear beneficial effects in established disease on muscle performance and health related quality of life and should be recommended. Furthermore, physical exercise seems to be safe also in early phases of disease. The most important outcome measure in polymyositis and dermatomyositis is muscle performance, strength and endurance, and the influence of disease activity or damage respectively on these measures. In addition, both polymyositis and dermatomyositis, are systemic inflammatory diseases and other organs are frequently involved and therefore also need to be measured. The variability of disease manifestations of polymyositis and dermatomyositis is captured using the International Myositis Assessment and Clinical Studies Group (IMACS) core set of disease activity. Inclusion body myositis (IBM) is a slowly progressive disorder, where the importance of the immune system in the pathophysiology is unclear. Some patients experience a partial improvement with glucocorticoids in the initial phases of disease whereas other patients do not respond at al. therefore IBM is an important differential diagnosis to refractory polymyositis. Characteristics of IBM will be discussed as well as results of some pharmacological interventions.
Disclosure of Interest
None DeclaredInclusion body myositis
Antisynthetase syndrome
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