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Autoimmunity Reviews Feb 2022To perform a systematic review and meta-analysis on the efficacy and safety of intravenous (IVIg) and subcutaneous (SCIg) immunoglobulin (Ig) therapy in the treatment of... (Meta-Analysis)
Meta-Analysis Review
OBJECTIVE
To perform a systematic review and meta-analysis on the efficacy and safety of intravenous (IVIg) and subcutaneous (SCIg) immunoglobulin (Ig) therapy in the treatment of idiopathic inflammatory myopathy (IIM) and juvenile dermatomyositis (JDM).
METHODS
PubMed, Embase and SCOPUS were searched to identify studies on Ig therapy in patients with IIM and/or JDM (2010-2020). Outcome measures were complete response (CR) or partial response (PR) in terms of muscle power and extramuscular disease activity measures on the International Myositis Assessment and Clinical Studies Group (IMACS) core set domains.
RESULTS
Twenty-nine studies were included (n = 576, 544 IIM, 32 JDM). Muscle power PR with pooled Ig therapy was 88.5% (95% confidence interval (CI): 80.6-93.5, n = 499) and PR with SCIg treatment was 96.61% (95% CI: 87.43-99.15, n = 59). Pooled PR with first-line use of IVIg was 77.07% (95% CI: 61.25-92.89, n = 80). Overall, mean time to response was 2.9 months (95% CI: 1.9-4.1). Relapse was seen in 22.76% (95% CI: 14.9-33). Studies on cutaneous disease activity and dysphagia showed significant treatment responses. Glucocorticoid and immunosuppressant sparing effect was seen in 40.9% (95% CI: 20-61.7) and 42.2% (95% CI: 20.4-64.1) respectively. Ig therapy was generally safe with low risk of infection (1.37%, 95% CI: 0.1-2.6).
CONCLUSIONS
Add-on Ig therapy improves muscle strength in patients with refractory IIM, but evidence on Ig therapy in new-onset disease and extramuscular disease activity is uncertain.
Topics: Dermatomyositis; Glucocorticoids; Humans; Immunization, Passive; Immunoglobulins, Intravenous; Myositis
PubMed: 34800685
DOI: 10.1016/j.autrev.2021.102997 -
The Journal of Rheumatology Mar 2023Idiopathic inflammatory myopathy (IIM)-associated interstitial lung disease (ILD) can range from rapidly progressive disease with high mortality to indolent disease with... (Meta-Analysis)
Meta-Analysis
OBJECTIVE
Idiopathic inflammatory myopathy (IIM)-associated interstitial lung disease (ILD) can range from rapidly progressive disease with high mortality to indolent disease with minimal morbidity. This systematic review and metaanalysis describe immunological, clinical, and radiographical predictors of mortality in IIM-ILD.
METHODS
MEDLINE and Embase database searches were completed on October 18, 2021, to identify articles providing survival data according to baseline characteristics in patients with concurrent IIM and ILD. Prognostic factors common to more than 5 papers were included in the metaanalysis using a random-effects model to report odds ratios (ORs) for binary variables and Hedges for continuous variables. Risk of bias was assessed using the Newcastle-Ottawa Scale score and the Egger test for publication bias.
RESULTS
From 4433 articles, 62 papers were suitable for inclusion; among these studies, 38 different variables were considered. The OR for risk of death regarding the presence of anti-melanoma differentiation-associated protein 5 (MDA5) antibodies was 6.20 (95% CI 3.58-10.71), and anti-tRNA synthetase antibodies were found to be protective (OR 0.24, 95% CI 0.14-0.41). Neither antinuclear antibodies, anti-52-kDa Ro antigen antibodies, nor SSA significantly altered mortality, nor was MDA5 titer predictive. Examples of prognostic factors that are significantly associated with mortality in this study include the following: age; male sex; acute/subacute onset; clinically amyopathic dermatomyositis; dyspnea; ulceration; fever; raised C-reactive protein, ferritin, lactate dehydrogenase, alveolar to arterial O (A-aO) gradient, ground-glass opacity on high-resolution computed tomography (HRCT), and overall HRCT score; and reduced albumin, lymphocytes, ratio of partial pressure of oxygen in the arterial blood to fraction of inspired oxygen (PF ratio), percentage predicted transfer factor for carbon monoxide, and percentage predicted forced vital capacity. Baseline surfactant protein-D and Krebs von den Lungen-6 levels were not predictors of mortality.
CONCLUSION
Many mortality risk factors were identified, though heterogeneity was high, with a low quality of evidence and a risk of publication bias. Studies regarding anti-MDA5 antibody-positive disease and and those from East Asia predominate, which could mask risk factors relevant to other IIM subgroups or populations.
Topics: Humans; Male; Autoantibodies; Dermatomyositis; Disease Progression; Lung Diseases, Interstitial; Myositis; Prognosis; Retrospective Studies; Female
PubMed: 36379584
DOI: 10.3899/jrheum.220383 -
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 -
Modern Rheumatology Apr 2023To evaluate the efficacy and safety of intravenous immunoglobulin (IVIG) in the treatment of dermatomyositis (DM) and polymyositis (PM). (Meta-Analysis)
Meta-Analysis
OBJECTIVES
To evaluate the efficacy and safety of intravenous immunoglobulin (IVIG) in the treatment of dermatomyositis (DM) and polymyositis (PM).
METHODS
We searched PubMed, Embase, and the China National Knowledge Infrastructure for relevant studies from July 1919 to May 2021.
RESULTS
Seventeen papers pertinent to our questions were found: In a meta-analysis, we found that IVIG significantly improved the level of CK (SMD (STD. Mean Difference) = -0.69; 95%CI -0.93, -0.46; P < 0.0001), Manual Muscle Test (SMD = 1.12; 95%CI 0.77, 1.47; P < 0.00001), Medical Research Council (SMD = 1.59; 95%CI 0.86, 2.33; P < 0.00001), Activities of Daily Living (SMD = 1.07; 95%CI 0.59, 1.56; P < 0.0001). The CK levels in DM and PM were also significantly improved after IVIG (SMD = -0.73; 95%CI -1.12, -0.34; P = 0.0002 and SMD = -3.29; 95%CI -5.82, -0.76; P < 0.0001, respectively). The meta-analysis of three RCTs showed that there was a statistically significant improvement after IVIG (SMD = 0.63; 95%CI 0.22, 1.03; P = 0.002). In a random effects model, pooled muscle power improvement rate was 77% (95% CI: 66.0-87.0%). Meta-analyses of IVIG as first-line therapy showed a significant improvement of the CK level (SMD = -0.71; 95%CI -1.12, -0.30; P = 0.0007). The polled improvement rate of oesophageal disorders was 88% (95% CI: 80.0-95.0%). There was no statistically significant difference in the rate of improvement between the number of courses <2 and ≥2 (0.80% vs. 0.80%, P = 0.9). The proportion of corticosteroid-sparing success reached 81.8%. Adverse reactions following IVIG administration are usually mild and transient. Seven patients developed serious adverse events.
CONCLUSION
IVIG seems to be an effective drug for DM/PM, improving muscle strength, CK levels, and oesophageal involvement, and it is well tolerated by patients.
Topics: Humans; Immunoglobulins, Intravenous; Dermatomyositis; Activities of Daily Living; Polymyositis; Adrenal Cortex Hormones
PubMed: 35660927
DOI: 10.1093/mr/roac057 -
Seminars in Arthritis and Rheumatism Oct 2023To determine the prognostic factors of dermatomyositis with anti-melanoma differentiation-associated gene 5 (MDA5) antibody, a rare disease and often complicated by... (Meta-Analysis)
Meta-Analysis
OBJECTIVES
To determine the prognostic factors of dermatomyositis with anti-melanoma differentiation-associated gene 5 (MDA5) antibody, a rare disease and often complicated by life-threatening, rapidly progressive interstitial lung disease.
METHODS
Herein, we searched the Medline, Embase, and Cochrane Library databases and extracted studies published before August 23, 2022. Pooled analysis of hazard ratios (HRs) or odds ratios was used to identify prognostic factors for mortality among patients with anti-MDA5 antibody-positive dermatomyositis (MDA5+ DM).
RESULTS
Twenty-nine cohorts with 2,645 patients were included in this meta-analysis. Factors related to poor prognosis included old age (HR 1.54, 95% confidence interval (CI) 1.41-1.69, p < 0.01), male sex (HR 2.07, 95% CI 1.34-3.18, p < 0.01), rapidly progressive interstitial lung disease (RP-ILD) (HR 9.34, 95% CI 6.39-13.6, p < 0.01), high levels of ferritin (HR 1.05, 95% CI 1.01-1.08, p < 0.01), C-reactive protein (CRP) (HR 1.12, 95% CI 1.06-1.19, p < 0.01), creatine kinase (HR 1.05, 95% CI 1.03-1.07, p < 0.01), and lactate dehydrogenase (LDH) (HR 1.27, 95% CI 1.12-1.45, p < 0.01), whereas oxygen index (HR 0.990, 95% CI 0.988-0.992, p < 0.01), partial pressure of oxygen (HR 0.933, 95% CI 0.906-0.961, p < 0.01), forced vital capacity (HR 0.962, 95% CI 0.928-0.998, p = 0.038), and lymphocyte count (HR 0.421, 95% CI 0.282-0.629, p < 0.01) were associated with better outcomes.
CONCLUSIONS
Old age, male sex, hypoxemia, low forced vital capacity, lymphocytopenia, and high levels of ferritin, CRP, creatine kinase, and LDH are risk factors for mortality in patients with MDA5+ DM. However, a cautious interpretation of these results and further quality investigation are warranted.
Topics: Humans; Male; Autoantibodies; Dermatomyositis; Disease Progression; Ferritins; Interferon-Induced Helicase, IFIH1; Lung Diseases, Interstitial; Prognosis; Retrospective Studies; Risk Factors
PubMed: 37348186
DOI: 10.1016/j.semarthrit.2023.152231 -
Autoimmunity Reviews Jun 2022We conducted a systematic review, on behalf of the EULAR Study Group on Microcirculation in Rheumatic Diseases (EULAR SG MC/RD), to investigate the value of nailfold... (Review)
Review
Standardised interpretation of capillaroscopy in autoimmune idiopathic inflammatory myopathies: A structured review on behalf of the EULAR study group on microcirculation in Rheumatic Diseases.
OBJECTIVE
We conducted a systematic review, on behalf of the EULAR Study Group on Microcirculation in Rheumatic Diseases (EULAR SG MC/RD), to investigate the value of nailfold videocapillaroscopy (NVC) in idiopathic inflammatory myopathies (IIM).
METHODS
Three electronic databases were systematically searched to find all relevant manuscripts reporting NVC outcomes in IIM patients. Articles were assessed based on study design, population, NVC methodology and description of NVC results. To allow comparison between the articles, all NVC results were interpreted according to standardised capillaroscopic terminology, as previously consented by the EULAR SG MC/RD and the Scleroderma Clinical Trials Consortium (SCTC) Group on Capillaroscopy.
RESULTS
Of the 653 identified records; five were retained after critical appraisal on title, abstract and manuscript level. A marked difference in NVC was observed between (juvenile) dermatomyositis [(j)DM] versus polymyositis, healthy controls and systemic sclerosis patients. In addition, reduced capillary density and scleroderma pattern seem to be associated with active disease in (j)DM, while immunosuppressive treatment appears to reduce NVC abnormalities.
CONCLUSION
This is the first systematic review investigating NVC in IIM, interpreting the results according to an international consented standardised manner, as proposed by the EULAR SG MC/RD and SCTC Group on Capillaroscopy. We can conclude that NVC presents a promising asset in the diagnosis of (j)DM. Moreover, NVC could be a biomarker for organ involvement and follow-up. Large multicentre prospective standardised studies are further needed to definitely describe associations with clinical and laboratory parameters in the different IIM subtypes.
Topics: Autoimmune Diseases; Capillaries; Dermatomyositis; Humans; Microcirculation; Microscopic Angioscopy; Myositis; Nails; Prospective Studies; Rheumatic Diseases; Scleroderma, Localized; Scleroderma, Systemic
PubMed: 35421608
DOI: 10.1016/j.autrev.2022.103087 -
Current Rheumatology Reports Feb 2024We performed a systematic review of the literature on the epidemiology, pathogenesis, clinical and laboratory characterization, and treatment of calcinosis in patients... (Review)
Review
PURPOSE OF REVIEW
We performed a systematic review of the literature on the epidemiology, pathogenesis, clinical and laboratory characterization, and treatment of calcinosis in patients with juvenile dermatomyositis (JDM). A qualitative systematic review was conducted from January 1975 to April 2023 according to the PRISMA protocol using three electronic databases: PubMed, Web of Science, and Scopus. Studies were analyzed based on the following eligibility criteria: at least one combination of the terms described in the search strategy appeared in the title, written in English, Portuguese, or Spanish, and addressed the epidemiology, pathogenesis, diagnosis, and treatment of calcinosis in juvenile dermatomyositis. Systematic or scoping reviews, letters, clinical images, book chapters, abstracts, inflammatory myopathy in other connective tissue diseases, idiopathic inflammatory myopathies in adults, and purely qualitative studies were excluded.
RECENT FINDINGS
Seventy-five studies were included. According to the literature, calcinosis is common in women, around five years old, with three years of disease in association with osteoarticular, cutaneous, pulmonary manifestations, and fever. The pathogenesis is still unknown, but the participation of interleukin 1 and 6, tumor necrosis factor alpha, and innate immunity dysregulation seem to be involved. Common autoantibodies are anti-NXP-2, anti-MDA-5, and anti-Mi-2, and their treatment remains controversial. Prospective, randomized, controlled studies are needed to evaluate treatment protocols and map the natural history of this serious complication. Calcinosis seems to be more common in White female children with muscle weakness, fever, arthritis, severe pulmonary, and skin involvement with anti-NXP-2, anti-MDA-5, and anti-Mi-2 autoantibodies. The multitargets and aggressive treatment is recommended.
Topics: Child; Adult; Humans; Female; Child, Preschool; Dermatomyositis; Prospective Studies; Autoantibodies; Myositis; Calcinosis
PubMed: 38060107
DOI: 10.1007/s11926-023-01126-5 -
JAMA Dermatology Feb 2024While several medications are known to induce dermatomyositis (DM), most existing studies are case reports or small case series from a single institution. There is also...
IMPORTANCE
While several medications are known to induce dermatomyositis (DM), most existing studies are case reports or small case series from a single institution. There is also limited information on DM induced by immune checkpoint inhibitors, which are increasingly used in oncologic therapy.
OBJECTIVE
To characterize causes and clinical presentation of drug-induced DM based on the current literature.
EVIDENCE REVIEW
A systematic review was performed in PubMed according to Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) reporting guidelines, from inception to August 22, 2022. Articles meeting preestablished inclusion criteria (written in English and classified as original articles, case reports, literature reviews, and observation letters) were selected and data abstracted. Articles that met the scope of the review were also added from reference lists. When possible, study results were quantitatively combined.
FINDINGS
In 134 studies (114 from the literature search and 20 additional studies pulled from reference lists) describing 165 cases, 88 patients (53.3%) were female, and the median (IQR) age was 61 (49-69) years. Among the cases of drug-induced DM, the most common associated medications were hydroxyurea (50 [30.3%]), immune checkpoint inhibitors (27 [16.4%]), statins (22 [13.3%]), penicillamine (10 [6.1%]), and tumor necrosis factor inhibitors (10 [6.1%]). Histopathologic testing, when undertaken, helped establish the diagnosis. There was a median (IQR) of 60 (21-288) days between drug initiation and drug-induced DM onset. History of cancer was reported in 85 cases (51.6%).
CONCLUSIONS AND RELEVANCE
In this systematic review, drug-induced DM was associated with multiple types of medications, including chemotherapies and immunotherapies. It is essential that dermatologists promptly recognize and diagnose drug-induced DM so that they can guide management to minimize interruption of therapy when possible.
Topics: Humans; Female; Middle Aged; Aged; Male; Dermatomyositis; Immune Checkpoint Inhibitors; Neoplasms; Hydroxyurea
PubMed: 38198130
DOI: 10.1001/jamadermatol.2023.5418 -
Frontiers in Oncology 2023The idiopathic inflammatory myopathies (IIM) are a collection of autoimmune diseases that have a substantial impact on the entire body and include conditions such as... (Review)
Review
BACKGROUND
The idiopathic inflammatory myopathies (IIM) are a collection of autoimmune diseases that have a substantial impact on the entire body and include conditions such as dermatomyositis (DM), polymyositis (PM), sporadic inclusion body myositis, and immune-mediated necrotizing myopathy. These disorders are characterized by symptoms such as muscular weakness, pain, and dermal rash. This systematic review is intended to explore the potential link between bladder cancer and DM/PM.
METHODS
We performed a comprehensive systematic search on PubMed and Scopus until August 2022 to identify relevant research studies. The studies that met our inclusion criteria focused on patients with urinary bladder cancer and dermatomyositis, and/or polymyositis.
RESULTS
The patients' median age was 65.5 years (47-79), with the majority being male (15, 39.47%). Bladder cancer manifested before PM/DM in 5 (13.15%) patients, while in the majority of cases occurred after the cancer diagnosis. The stage of cancer at the time of the initial PM/DM diagnosis were mostly locally (11/20, 50%).During the first presentation, the patients had a median creatine kinase level of 2227 U/L, ranging between 44 and 10471. In one case, anti-TIF-1γ antibodies were found to be present. Among the cases with reported medical history (20/38), treatment immediately improved DM symptoms in 16 patients(53.8%) and in 3 patients(15%), symptoms of DM resurfaced during the period after the operation. Death was reported in 14 (36.8%) patients.
CONCLUSION
In conclusion, our study provides knowledge and understanding for identifying specific risk factors in patients with the coexistence of bladder cancer and DM/PM and their management. During the initial and follow-up screening, age, gender, and the clinicopathological subgroup of myositis should be considered to ensure proper management of the condition.
PubMed: 38023222
DOI: 10.3389/fonc.2023.1223627 -
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