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Rheumatology AND Dermatomyositis [keywords]
- Activated IL-22 pathway occurs in the muscle tissues of patients with polymyositis or dermatomyositis and is correlated with disease activity. [JOURNAL ARTICLE]
- Rheumatology (Oxford) 2014 Mar 5.
Objective. The aim of this study was to assess the expression of IL-22, IL-22 receptor 1 (IL-22R1), IL-22 binding protein (IL-22BP) and p-STAT3 in muscle tissue from patients with PM and DM.Methods. Levels of IL-22, IL-22R1, IL-22BP and STAT3 mRNA were quantified by RT-PCR. The expression of IL-22, IL-22R1, IL-22BP and p-STAT3 was also analysed using immunohistochemistry.Results. Significant modulation of the IL-22 pathway was observed in inflammatory myopathic tissues. In particular, a significant overexpression of IL-22 at the protein but not the mRNA level was observed in PM/DM tissues and was correlated with myositis activity. IL-22R1 aberrant expression was also observed among infiltrating mononuclear cells and necrotic muscle cells. IL-22BP, which inhibits IL-22 signalling, was expressed only in some muscle fibres in PM/DM patients.Conclusion. Our findings indicate that the IL-22 pathway is activated in inflammatory myopathic tissues and may be involved in the induction of muscle inflammatory processes and muscle necrosis.
- Angiotensin-converting enzyme insertion/deletion gene polymorphism is associated with dermatomyositis. [JOURNAL ARTICLE]
- J Renin Angiotensin Aldosterone Syst 2014 Mar 3.
and objective:The cornerstone of dermatomyositis (DM) pathogenesis involves vascular disturbance that leads to hypoxia, capillary necrosis and muscle perifascicular atrophy. Hence, the hypothesis is that the angiotensin-converting enzyme (ACE) insertion/deletion (I/D) gene polymorphism could be associated with susceptibility to DM.A single centre, case control study that genotyped ACE gene in 88 DM and 99 healthy individuals. The ACE gene polymorphism was determined by melting curve analysis of real-time polymerase chain reaction products using SYBR Green.The DM and the control subjects had a comparable mean age, gender frequency and ethnicity. The frequency of the D allele was higher in DM than in the control individuals (63.6% vs 55.6%, respectively). The DM had more ACE D/D and less ACE I/D genotype when compared to the control individuals, whereas the ACE I/I genotype distribution was similar in both case and control groups. Moreover, after sex-age-adjusted analysis, the ACE D/D genotype was strongly associated with DM disease (odds ratio (OR) 2.44, 95% confidence interval (CI): 1.17-4.37), in contrast to ACE I/D genotype (OR 0.51, 95% CI: 0.28-0.93).Homozygous ACE D/D was associated significantly with the DM risk. Further investigations are required to clarify and to confirm the association of these genes with DM susceptibility.
- Severe macrophage activation syndrome and central nervous system involvement in juvenile dermatomyositis. [Journal Article]
- Scand J Rheumatol 2014; 43(2):171-3.
- Systemic Autoimmune Rheumatic Disease Prevalence in Canada: Updated Analyses Across 7 Provinces. [JOURNAL ARTICLE]
- J Rheumatol 2014 Mar 1.
To estimate systemic autoimmune rheumatic disease (SARD) prevalence across 7 Canadian provinces using population-based administrative data evaluating both regional variations and the effects of age and sex.Using provincial physician billing and hospitalization data, cases of SARD (systemic lupus erythematosus, scleroderma, primary Sjögren syndrome, polymyositis/dermatomyositis) were ascertained. Three case definitions (rheumatology billing, 2-code physician billing, and hospital diagnosis) were combined to derive a SARD prevalence estimate for each province, categorized by age, sex, and rural/urban status. A hierarchical Bayesian latent class regression model was fit to account for the imperfect sensitivity and specificity of each case definition. The model also provided sensitivity estimates of different case definition approaches.Prevalence estimates for overall SARD ranged between 2 and 5 cases per 1000 residents across provinces. Similar demographic trends were evident across provinces, with greater prevalence in women and in persons over 45 years old. SARD prevalence in women over 45 was close to 1%. Overall sensitivity was poor, but estimates for each of the 3 case definitions improved within older populations and were slightly higher for men compared to women.Our results are consistent with previous estimates and other North American findings, and provide results from coast to coast, as well as useful information about the degree of regional and demographic variations that can be seen within a single country. Our work demonstrates the usefulness of using multiple data sources, adjusting for the error in each, and providing estimates of the sensitivity of different case definition approaches.
- Clinical and serological correlates of anti-PM/Scl antibodies in systemic sclerosis: A multicenter study of 763 patients. [JOURNAL ARTICLE]
- Arthritis Rheumatol 2014 Feb 27.
Background Anti-PM/Scl autoantibodies are found in polymyositis (PM), dermatomyositis (DM), systemic sclerosis (SSc), and systemic autoimmune rheumatic disease overlap syndromes. PM1-Alpha is a major epitope of the PM/Scl complex and antibodies against PM1-Alpha can be detected using a validated ELISA. This study was undertaken to determine the prevalence and identify the clinical correlates of anti-PM1-Alpha antibodies in a large SSc cohort. Methods The sera of 763 SSc subjects enrolled in a Canadian multi-centre cohort were analyzed for the presence of antibodies against PM1-Alpha by ELISA. Associations between the presence of anti-PM1-Alpha antibodies and demographic, clinical and other serological manifestations of SSc were investigated. Results Antibodies against PM1-Alpha were present in 55 (7.2%) SSc patients, of which almost half (26/55; 3.4% of the overall cohort) had no other SSc-specific antibodies, namely anti-centromere, anti-topoisomerase I, and anti-RNA polymerase III. Features positively associated with the presence of anti-PM1-Alpha antibodies included younger age at disease onset, skeletal muscle involvement, calcinosis, inflammatory arthritis and overlap disease, while interstitial lung disease was less frequent and there were fewer gastrointestinal symptoms present in subjects with compared to without these antibodies. Conclusions Anti-PM1-Alpha antibodies are relatively common in SSc and associated with a distinct clinical phenotype consistent with that described with other anti-PM/Scl autoantibodies. Although anti-PM1-Alpha antibodies are not exclusive of other SSc-specific antibodies, they can be present in the absence thereof. Therefore, these autoantibodies are of considerable diagnostic and prognostic relevance in SSc. © 2014 American College of Rheumatology.
- The expression profile of miR-23b is not altered in peripheral blood mononuclear cells of patients with idiopathic inflammatory myopathies. [Journal Article]
- F1000Res 2013.:223.
Idiopathic inflammatory myopathies (IIM) belong to a group of autoimmune disorders, primarily characterized by chronic inflammation of human skeletal muscle tissue. The etiology of these diseases is unknown, however, genetic predisposition plays a significant role in disease onset. Beside the known genetic risk located in the MHC complex, the epigenetic modifications including changes in miRNAs expression profiles have been recently implicated recently in many autoimmune diseases. Micro RNA molecules are involved in many physiological processes, including the regulation of the immune response. In our study we have focused on the miR-23b, as it represents a novel promising autoimmunity regulator molecule. Downregulation of miR-23b was recently described in patients with rheumatoid arthritis and systemic lupus erythematosus. We have measured the expression miR-23b peripheral blood mononuclear cells of patients with dermatomyositis and polymyositis. No meaningful difference was found in comparison with healthy controls.
- Rash and elevated creatine kinase in a deployed soldier. [Journal Article]
- Mil Med 2014 Feb; 179(2):e245-8.
A 24-year-old active duty soldier was evacuated from Afghanistan to the United States after persistent upper respiratory tract infection. His course was complicated by an exfoliative rash, diffuse muscle aches, and elevated creatine kinase following trimethoprim-sulfamethoxazole exposure that persisted despite withdrawal of the medication. Dermatomyositis was strongly considered, but the patient had a negative muscle biopsy and had positive serologies for acute Epstein-Barr virus infection. We present a case of acute Epstein-Barr virus infection and possible trimethoprim-sulfamethoxazole reaction mimicking dermatomyositis.
- A9.9 Prevalence and risk factors of corticosteroids self-medication. [Journal Article]
- Ann Rheum Dis 2014 Mar 1.:A95.
Corticosteroids (CTC) are semisynthetic adrenocortical derivatives hormones. They are the standard treatment for most inflammatory diseases. However, there is a severity number side effect. A good process management require a balance between inflammatory activity sufficient and adverse tolerable. CTC are anti-inflammatory not only powerful, but also rapidly effective. They are used in several pathologies with a frequent tendency to self-medicate.Evaluation of the prevalence and factors of self-medication with corticosteroids, through a survey of 125 patients treated for long term.Cross-sectional descriptive survey conducted with 125 patients, at Ibn Rochd Rheumatology department in Casablanca. Inclusions criteria were, patients receiving prolonged systemic corticosteroid. The survey contains a multiple or open choice questionnaires, established in agreement with the Biostatistics-Epidemiology and Medical Informatics. It was about the identity of patients, the disease being treated with corticosteroids, the duration and treatment dose, the patients number self-medication and its contributing factors, finally the side effects involved in taking steroids.The majority of patients were female (82.4%). The mean age was 46.5 years ± 10 years. Diseases requiring prescription CTC was rheumatoid arthritis (50.4%) and systemic Lupus (17.6%). Other indication (sarcoidosis, dermatomyositis, vasculitis) at 32%. Type of CTC was prednisone (78.4%). Patients received CTC above one year (71.2%). 80% of them were informed about effects of corticosteroids. 58 patients have been using corticosteroids for self-medication. The reasons were; rapid efficacy of corticosteroid, therapy during attacks (36.2%), Lack of information (25.8%), appointment of remote consultations (19%), easy access to pharmacists CTC (10.4%), and not expensive treatment (8.6%).Improved support and optimization patient compliance in long-term systemic corticosteroids are necessary for better medical exchanges sick. The particularities of our population are; low socio-economic level, the use of cheap treatments and CTC sale without a prescription.
- The skeletal muscle arachidonic acid cascade in health and inflammatory disease. [JOURNAL ARTICLE]
- Nat Rev Rheumatol 2014 Jan 28.
Muscle atrophy and weakness are often observed in patients with chronic inflammatory diseases, and are the major clinical features of the autoimmune myopathies, polymyositis and dermatomyositis. A general understanding of the pathogenesis of muscle atrophy and the impaired muscle function associated with chronic inflammatory diseases has not been clarified. In this context, arachidonic acid metabolites, such as the prostaglandin and leukotriene subfamilies, are of interest because they contribute to immune and nonimmune processes. Accumulating evidence suggests that prostaglandins and leukotrienes are involved in causing muscular pain and inflammation, and also in myogenesis and the repair of muscles. In this Review, we summarize novel findings that implicate prostaglandins and leukotrienes in the muscle atrophy and weakness that occur in inflammatory diseases of the muscles, with a focus on inflammatory myopathies. We discuss the role of the arachidonic acid cascade in skeletal muscle growth and function, and individual metabolites as potential therapeutic targets for the treatment of inflammatory muscle diseases.
- The clinical features, diagnosis and classification of dermatomyositis. [JOURNAL ARTICLE]
- J Autoimmun 2014 Jan 24.
Dermatomyositis (DM) is an idiopathic inflammatory myopathy (IIM) characterized by an inflammatory infiltrate primarily affecting the skeletal muscle and skin. Most common and peculiar cutaneous lesions include Gottron's papules, Gottron's sign and heliotrope rash. Different DM subsets have been identified until now encompassing classic DM, amyopathic DM, hypomyopathic DM, post-myopathic DM, and DM sine dermatitis. Patients with DM have a higher incidence rate of malignancy than the normal population. In these patients cancer occurs in about 30% of cases with higher occurrence in men and in elderly people. Bohan and Peter's diagnostic criteria, proposed in 1975, have been widely accepted and used until now. In the last ten years muscle immunopathology, myositis specific autoantibodies testing, and the use of new techniques of muscle imaging such as contrast-enhanced ultrasound or Magnetic Resonance Imaging have been introduced in the diagnostic work-up of patients with DM leading to the development of new diagnostic criteria.