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Seminars in Arthritis and Rheumatism Apr 2021Myositis-specific autoantibodies (MSAs) are thought to be mutually exclusive in patients with idiopathic inflammatory myopathies (IIM) based on studies with...
Detection of multiple myositis-specific autoantibodies in unique patients with idiopathic inflammatory myopathy: A single centre-experience and literature review: Systematic review.
INTRODUCTION
Myositis-specific autoantibodies (MSAs) are thought to be mutually exclusive in patients with idiopathic inflammatory myopathies (IIM) based on studies with immunoprecipitation-based (IP) detection methods. Recently, detection of multiple MSAs in unique patients is increasingly reported, but the extent of this phenomenon remains unclear.
METHODS
At our centre, we reviewed results from two line immunoassays and one dot immunoassay in 145 IIM patients and 240 controls for the presence of multiple MSAs. Pubmed and Embase were systematically searched for articles mentioning detection of multiple MSAs in IIM patients, published until February 2019. We assessed the frequency, detection method, the precise combinations and clinical phenotypes of participants with multiple MSAs.
RESULTS
At our centre, detection of multiple MSAs occurred in 3.4-8.3% of patients with IIM, depending on the assay. However, no cases with full concordance across all three assays were identified. Forty-four articles reported detection of multiple MSAs, representing a total of 133 cases, including four patients with a connective tissue disease other than IIM and two healthy controls. In 101 cases all MSAs were detected using only one detection method: 40 cases with IP-based methods (most frequently used technique) and 61 cases with other assay types. In most cases the phenotype of patients with multiple MSAs matched the predicted presentation associated with one MSA and in few cases the phenotype matched with both MSAs.
CONCLUSION
Detection of multiple MSAs in unique IIM patients is less rare than commonly accepted. Specificity issues of the commercially available multiplex immunoassays may, at least partly, explain the higher frequency compared to IP-based methods. 'True multiple MSA-positive' patients may exist, though they are most likely rare.
Topics: Autoantibodies; Humans; Myositis; Phenotype; Polymyositis
PubMed: 33831755
DOI: 10.1016/j.semarthrit.2021.03.012 -
Dermatologic Therapy May 2021Refractory dermatomyositis (DM) is defined as cases that do not show improvement after initial treatment with two different immunosuppressives combined with... (Review)
Review
Refractory dermatomyositis (DM) is defined as cases that do not show improvement after initial treatment with two different immunosuppressives combined with corticosteroids with or without intravenous immunoglobulins. In recent years, few studies have reported a positive response to the use of Janus kinase inhibitors (JAK-inhibitors) for the treatment of refractory DM. A systematic literature review was performed for articles studying the use of JAK-inhibitors for the treatment of refractory DM. We identified 38 females and 15 males treated with JAK-inhibitors without serious side effects. Tofacitinib was the most frequently used JAK-inhibitor followed by ruxolitinib. Significant improvement in CDASI score, muscle strength, body weight, and skin lesions were reported in most of the studies. The duration of follow-up ranged from 1 to 15 months without relapse. Therefore, the use of JAK-inhibitors looks promising in the treatment of refractory DM and further high volume research may be required to validate the current concept. As only case reports and series were identified without direct comparison for review, there is a potential risk of bias. Despite these limitations, we believe that the result of this analysis allows a better understanding of treatment options for refractory DM and will help generate a hypothesis that can be further tested.
Topics: Dermatomyositis; Drug-Related Side Effects and Adverse Reactions; Female; Humans; Immunosuppressive Agents; Janus Kinase Inhibitors; Male
PubMed: 33713527
DOI: 10.1111/dth.14939 -
Rheumatology (Oxford, England) Jun 2021To identify clinical factors associated with cancer risk in the idiopathic inflammatory myopathies (IIMs) and to systematically review the existing evidence related to... (Meta-Analysis)
Meta-Analysis
OBJECTIVES
To identify clinical factors associated with cancer risk in the idiopathic inflammatory myopathies (IIMs) and to systematically review the existing evidence related to cancer screening.
METHODS
A systematic literature search was carried out on Medline, Embase and Scopus. Cancer risk within the IIM population (i.e. not compared with the general population) was expressed as risk ratios (RR) for binary variables and weighted mean differences (WMD) for continuous variables. Evidence relating to cancer screening practices in the IIMs were synthesized via narrative review.
RESULTS
Sixty-nine studies were included in the meta-analysis. DM subtype (RR 2.21), older age (WMD 11.19), male sex (RR 1.53), dysphagia (RR 2.09), cutaneous ulceration (RR 2.73) and anti-transcriptional intermediary factor-1 gamma positivity (RR 4.66) were identified as being associated with significantly increased risk of cancer. PM (RR 0.49) and clinically amyopathic DM (RR 0.44) subtypes, Raynaud's phenomenon (RR 0.61), interstitial lung disease (RR 0.49), very high serum creatine kinase (WMD -1189.96) or lactate dehydrogenase (WMD -336.52) levels, and anti-Jo1 (RR 0.45) or anti-EJ (RR 0.17) positivity were identified as being associated with significantly reduced risk of cancer. Nine studies relating to IIM-specific cancer screening were included. CT scanning of the thorax, abdomen and pelvis appeared to be effective in identifying underlying asymptomatic cancers.
CONCLUSION
Cancer risk factors should be evaluated in patients with IIM for risk stratification. Screening evidence is limited but CT scanning could be useful. Prospective studies and consensus guidelines are needed to establish cancer screening strategies in IIM patients.
Topics: Adenosine Triphosphatases; Age Factors; Antibodies, Antinuclear; Creatine Kinase; DNA-Binding Proteins; Deglutition Disorders; Dermatomyositis; Female; Guidelines as Topic; Humans; L-Lactate Dehydrogenase; Lung Diseases, Interstitial; Male; Myositis; Neoplasms; Publication Bias; Raynaud Disease; Risk; Sex Factors; Skin Ulcer; Tomography, X-Ray Computed; Transcription Factors
PubMed: 33599244
DOI: 10.1093/rheumatology/keab166 -
Contemporary Oncology (Poznan, Poland) 2020Dermatomyositis (DM) is defined as an autoimmune inflammatory disease that affects the skin, the blood vessels and the muscles. It typically presents with erythema... (Review)
Review
Dermatomyositis (DM) is defined as an autoimmune inflammatory disease that affects the skin, the blood vessels and the muscles. It typically presents with erythema affecting mostly the eyes and the hands as well as proximal muscle weakness. It has been also correlated with various types of cancer, including ovarian cancer. A systematic PubMed and Scopus search was conducted. A total of 110 women were included in our review. The median age of the patients was 52.5 years (8-85). The most frequent histological type of malignancy was epithelial (87 cases, 79.1%) and in only one patient (0.9%) DM was co-existing with a cancer recurrence. A clinical diagnosis of DM or PM preceded the diagnosis of ovarian cancer in 69.1% (76/110), while paraneoplastic DM after the diagnosis of ovarian cancer was reported in 31% (34/110). Serum antibodies were present in 22.5% (25/110) and the median creatine kinase during first evaluation was 886 (56.6-16,596). Postoperative improvement of the symptoms was observed in 24.6% (27/110) while also 24.6% (27/110) needed post-treatment rheumatological management. Neoadjuvant chemotherapy or radiotherapy was necessary in 58.2% (64/110) and a cancer recurrence was identified in 28.2% (31/110), with a median follow up of 24.5 months (5-210). Finally, 52 (47.3%) deaths were reported in a median follow-up period of 16 months (0-210). It is crucial that DM patients should receive a thorough evaluation for ovarian cancer, among other malignancies, encompassing an abdominal CT or MRI scan and serum Ca-125 marker measurements. Treatment of ovarian cancer is usually accompanied by remission of DM symptoms in most of the cases.
PubMed: 33531873
DOI: 10.5114/wo.2020.102814 -
Journal of the American Academy of... Jan 2022
Topics: Biological Therapy; Dermatomyositis; Humans
PubMed: 33529702
DOI: 10.1016/j.jaad.2021.01.087 -
Seminars in Arthritis and Rheumatism Feb 2021Interstitial lung disease (ILD) is the most important prognostic factor for mortality in patients with polymyositis (PM) and dermatomyositis (DM), but the prevalence of... (Meta-Analysis)
Meta-Analysis Review
OBJECTIVE
Interstitial lung disease (ILD) is the most important prognostic factor for mortality in patients with polymyositis (PM) and dermatomyositis (DM), but the prevalence of ILD in PM/DM may vary between countries. The aim of this study was to determine the overall prevalence of ILD in global patients with PM/DM.
METHODS
We performed a systematic literature review of studies published from Jan 1, 2000 to April 30, 2020 on ILD and PM/DM. We extracted data and pooled the prevalence by using a random-effect model due to high heterogeneity. Heterogeneity was assessed by subgroup analysis and sensitivity analysis.
RESULTS
A total of 34 studies with 10,130 patients were included in our meta-analysis. Pooled data demonstrated that the global prevalence of ILD in patients with PM/DM was 0.41 (95% confidence interval [CI] 0.35-0.48). However, this prevalence varied with geographical locations and time trends. The prevalence of ILD in PM/DM was 0.5 (95% CI 0.42-0.57) in Asia, 0.23 (95% CI 0.15-0.31) in America, and 0.26 (95% CI 0.18-0.34) in Europe. A higher prevalence of ILD was reported in studies published in 2011-2015 (0.43, 95% CI 0.34-0.52) and 2016-2020 (0.45, 95% CI 0.35-0.54), compared with those published in 2000-2010 (0.27, 95% CI 0.16-0.39). The pooled prevalence of ILD in patients with DM, PM, and clinically amyopathic dermatomyositis subtype was 0.42 (95% CI 0.35-0.49), 0.35 (95% CI 0.27-0.42), and 0.53 (95% CI 0.32-0.74), respectively. Patients with anti-Jo-1 and anti-melanoma differentiation-associated gene 5 antibodies were more likely to develop ILD than other myositis-specific autoantibodies.
CONCLUSION
The global prevalence of ILD in patients with PM/DM was approximately 41% and the condition was predominant in Asians. This highlights potential genetic and environmental differences in the pathogenesis of ILD in patients with PM/DM. More studies are required to elucidate the specific associations.
Topics: Dermatomyositis; Humans; Lung Diseases, Interstitial; Polymyositis; Prevalence; Retrospective Studies
PubMed: 33383294
DOI: 10.1016/j.semarthrit.2020.11.009 -
Neurology International Nov 2020to find the most up-to-date evidence of the effectiveness and safety of supervised physical therapy in polymyositis/dermatomyositis patients. (Review)
Review
OBJECTIVE
to find the most up-to-date evidence of the effectiveness and safety of supervised physical therapy in polymyositis/dermatomyositis patients.
METHODS
a systematic review of the literature in the main scientific databases was carried out. We searched for randomized controlled trials concerning supervised physical therapy and polymyositis/dermatomyositis. The PICOS method was used for the formulation of the clinical query. Methodological quality and the level of evidence of the included studies were assessed using the modified Jadad scale and the Oxford Centre for Evidence-Based Medicine Levels of Evidence guide, respectively.
RESULTS
a total of 2591 articles were found. By applying the inclusion/exclusion criteria, six randomized controlled clinical trials were admitted to the final phase of the review. The compared approaches concerned supervised exercise programs based on strategies of muscle strengthening or aerobic work. Following these exercises, an increase in the maximum rate of oxygen consumption, a decrease in creatine phosphokinase levels, an enhancement in the patient's aerobic performance and an improvement in the quality of life indexes were registered. The methodological quality of the included studies ranged from 3 to 4.5. All the studies were classified as presenting an evidence level of 2b.
CONCLUSIONS
supervised physical therapy in polymyositis/dermatomyositis is an effective, safe and free-of-contraindications tool to be used both in the acute and in the established phases of the pathology. However, further and higher-quality studies are necessary to confirm those findings, to clarify the timing of exercise delivery and to guide the choice towards different types of muscle contraction exercises.
PubMed: 33255200
DOI: 10.3390/neurolint12030015 -
Best Practice & Research. Clinical... Apr 2020Idiopathic inflammatory myopathies (IIM) and systemic lupus erythematosus (SLE) are inflammatory connective tissue diseases (CTDs) with common features of arthritis,...
Idiopathic inflammatory myopathies (IIM) and systemic lupus erythematosus (SLE) are inflammatory connective tissue diseases (CTDs) with common features of arthritis, muscle impairment, skin rash, and heart- and lung involvement. Exercise is becoming an important part of the treatment in patients with IIM and SLE; however, there is a need for evidence-based exercise recommendations on patient-relevant outcomes. To evaluate the evidence and to present evidence-based exercise recommendations on patient-relevant outcomes in patients with IIM and SLE. A systematic literature search of five databases was performed at two time points, 2016 going back all years, and an update in 2019. Inclusion criteria: RCTs including exercise, physical activity intervention, and patient-relevant outcomes. Systematic reviews and meta-analysis was also included. Grading of evidence was done according to the GRADE system. Five RCTs and 1 systematic review were identified in patients with IIM and eight RCTs, 6 systematic reviews, and 2 meta-analysis for patients with SLE. Aerobic exercise and resistance training on moderate-high intensity can improve aerobic capacity, muscle impairment, activity limitation, quality of life, and disease activity (limited evidence) in patients with established polymyositis (PM) and dermatomyositis (DM). Moderate-high intensity aerobic exercise can improve aerobic capacity (moderately strong evidence) and improve fatigue and depressive symptoms (limited evidence) without changing disease activity in patients with mild/inactive SLE with low/no organ damage. There is insufficient evidence for effects of exercise in patients with recent onset PM/DM and IBM. Exercise performed in line with American College of Sports Medicine recommendations can improve aerobic capacity, patient-reported outcomes in patients with nonactive PM/DM and mild/inactive SLE. More well-designed studies are needed to increase the scientific evidence. Studies with additional focus on evaluating effects of exercise in patients with higher disease activity, in patients with vital-organ involvement and in patients with IBM are needed.
Topics: Exercise Therapy; Exercise Tolerance; Humans; Lupus Erythematosus, Systemic; Myositis; Quality of Life
PubMed: 32819833
DOI: 10.1016/j.berh.2020.101547 -
International Journal of Rheumatic... Dec 2020Patients with rheumatic diseases are increasingly using internet-based information to inform healthcare utilization and make treatment decisions. Our aim was to assess...
AIM
Patients with rheumatic diseases are increasingly using internet-based information to inform healthcare utilization and make treatment decisions. Our aim was to assess the readability and quality of internet-based information on dermatomyositis (DM) and polymyositis (PM).
METHOD
Key words "Dermatomyositis" and "Polymyositis" were searched on 3 commonly used search engines (Google, Yahoo and Bing). The first 3 pages (~30) of search results were examined from each search engine. Readability of information was assessed using 4 readability formulae (Flesch Reading Ease Score, Flesch-Kincaid Grade Level, the Simplified Measure of Gobbledygook index, the Coleman-Liau index). Quality of information was assessed using the DISCERN tool, Journal of The American Medical Association (JAMA) benchmark criteria and Health on The Net Code (HoN code). We also examined Google Trends data to determine if there were obvious temporal search patterns.
RESULTS
Thirty-two websites were included in the study after duplicates were removed and exclusion criteria were applied. The overall quality was low including DISCERN with a median overall score of 38/80 (interquartile range 12.25), only 4/32 (13%) websites fulfilled all 4 JAMA benchmark criteria, and 9/32 (28%) had HoN code. Readability of information was assessed using 4 readability formulae (Flesch Reading Ease Score, Flesch-Kincaid Grade Level, the Simplified Measure of Gobbledygook index, the Coleman-Liau index. There was no obvious temporal trend in searches on analysis of Google Trends data.
CONCLUSION
The overall quality and readability of internet-based information relating to DM and PM is poor. Patients require appropriate information of high quality and readability throughout the course of their disease in order to make informed decisions on their condition including treatment.
Topics: Decision Making; Dermatomyositis; Humans; Internet; Patient Acceptance of Health Care; Polymyositis; Quality Improvement
PubMed: 32812386
DOI: 10.1111/1756-185X.13929 -
The Journal of Rheumatology Jan 2021Rheumatoid arthritis (RA) and other rheumatic diseases may present with ocular manifestations.The purpose of our work was to determine the prevalence and type of eye... (Meta-Analysis)
Meta-Analysis
OBJECTIVE
Rheumatoid arthritis (RA) and other rheumatic diseases may present with ocular manifestations.The purpose of our work was to determine the prevalence and type of eye involvement in RA and other connective tissue diseases through a metaanalysis and literature review.
METHODS
A systematic review of the literature was performed using Medline, Web of Science, and the Cochrane Library from their inceptions until January 7, 2019. Conjunctivitis, keratoconjunctivitis sicca, xeropthalmia, uveitis, eye hemorrhage, optic neuritis, papilledema, orbital disease, retinal artery/vein occlusion, macular edema, retinitis, chorioretinitis, scleritis, iridocyclitits, choroid hemorrhage, blindness, and amaurosis fugax were searched for prevalence in patients with RA, systemic lupus erythematosus (SLE), antiphospholipid syndrome (APS), dermatomyositis, polymyositis, systemic sclerosis, Sjögren syndrome (SS), undifferentiated connective tissue disease, giant cell arteritis, granulomatosis polyangiitis (GPA; formerly Wegener granulomatosis), systemic vasculitis, and sarcoidosis.
RESULTS
There were 3394 studies identified and 65 included. The prevalence of eye involvement was 18% in RA, 26% in GPA, 27% in giant cell arteritis, 27% in sarcoidosis, 31% in SLE, and 35% in APS. The most common manifestation was dry eye syndrome ("dry eye"; keratoconjunctivitis sicca) in most diseases analyzed, with an especially high frequency of 89% in SS. Anterior and posterior uveitis were the most common ocular complications in sarcoidosis, occurring in 16% (95% CI 3-28) and 6% (95% CI 3-9) of patients, respectively.
CONCLUSION
Eye involvement is present in approximately one-fifth of patients with RA, and a one-quarter to one-third of patients with connective tissue diseases (other than SS at 89%) and vasculitis.
Topics: Arthritis, Rheumatoid; Connective Tissue Diseases; Granulomatosis with Polyangiitis; Humans; Lupus Erythematosus, Systemic; Rheumatic Diseases
PubMed: 32358156
DOI: 10.3899/jrheum.190768