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Clinical Rheumatology Nov 2023We performed a systematic review of cardiovascular risk factors in idiopathic inflammatory myopathies (IIMs) and their cardiovascular outcomes, including acute coronary... (Review)
Review
We performed a systematic review of cardiovascular risk factors in idiopathic inflammatory myopathies (IIMs) and their cardiovascular outcomes, including acute coronary syndrome and stroke. A qualitative systematic review was conducted from January 1956 to December 2022 according to the PRISMA protocol using three electronic databases: PubMed, Web of Science, and Scopus. The 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 risk factors for cardiovascular diseases in IIMs. Brief reports, reviews, papers addressing juvenile IIMs, congress proceedings, monographs, and dissertations were excluded. Twenty articles were included. According to the literature, most patients with IIMs are middle-aged North American or Asian women, with dyslipidemia and hypertension. The prevalence of the cardiovascular risk factors was generally low in IIMs, but with a high incidence of acute myocardial infarction. Further theoretical and prospective studies are needed to define the actual impact of each variable (e.g., hypertension, diabetes, smoking, alcoholism, obesity, and dyslipidemia) on the cardiovascular risk of patients with IIMs.
Topics: Female; Humans; Middle Aged; Cardiovascular Diseases; Dyslipidemias; Heart Disease Risk Factors; Hypertension; Myositis; Risk Factors
PubMed: 37191898
DOI: 10.1007/s10067-023-06633-5 -
Autoimmunity Reviews Aug 2023Rapidly progressive interstitial lung disease (RP-ILD) clearly harms the prognoses of dermatomyositis/polymyositis (DM/PM) patients, however there is a dearth of... (Meta-Analysis)
Meta-Analysis Review
Rapidly progressive interstitial lung disease (RP-ILD) clearly harms the prognoses of dermatomyositis/polymyositis (DM/PM) patients, however there is a dearth of numerical prevalence and therapy comparison in this field. Therefore, the purpose of this study was to determine the prevalence of RP-ILD in DM/PM patients and compare prognoses, including remission rate and survival data, between treatments. Studies with reports of RP-ILD in DM/PM patients and studies with definite remission and/or survival data of DM/PM-RP-ILD were included in the study. Data sources were Pubmed, Embase, and Cochrane Library without language restrictions. Two authors (WHL and WWQ) extracted independently the data. Estimates of the pooled effects were calculated using the Mantel-Haenszel technique (random effects). The prevalence meta-analysis included 18 papers with 6058 DM/PM patients, and 31 papers were analyzed for treatment effects, including remission rate, 6-month survival rate, 1-year survival rate, and 5-year survival rate. Database search yielded 1816 articles. In the DM/PM population, the combined prevalence of RP-ILD was 8.9% (95% CI, 5.8% to 12.1%). Patients with RP-ILD have a remission rate of 58.4% (95% CI, 47.3% to 69.4%), with biologic treatment with the highest remission rate, followed by triple therapy (defined as adding a third intravenous medication, including cyclophosphamide and immunoglobulin). Biologics therapy had the highest overall survival rate at six months (95% CI, 49.8% to 73.9%), followed by cDMARDs, plasma exchange, and triple therapy. The 1-year survival rate was 77.4% (95% CI, 66.7% to 88.1%), and triple therapy and cDMARDs had the best survival rates. The 5-year survival rate was 40.0% (95% CI, 10.0% to 69.9%). The prevalence of RP-ILD in DM/PM was approximately 8.9%, with a poor long-term prognosis. The use of biological agents appears to provide the best therapeutic outcomes, providing RP-ILD management with a novel evidence-based therapy. The use of strong immunosuppressive treatments may result in life-threatening side effects, thus clinicians must closely monitor the condition.
Topics: Prevalence; Polymyositis; Humans; Adult; Dermatomyositis; Lung Diseases, Interstitial; Treatment Outcome
PubMed: 37164215
DOI: 10.1016/j.autrev.2023.103335 -
Neurogastroenterology and Motility Sep 2023Dermatomyositis (DM) is a rare autoimmune disease characterized by distinctive skin rash, muscle inflammation with symmetrical and progressive muscle weakness, and... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Dermatomyositis (DM) is a rare autoimmune disease characterized by distinctive skin rash, muscle inflammation with symmetrical and progressive muscle weakness, and elevated serum levels of muscle-associated enzymes. DM may affect skeletal muscles involved in swallowing, leading to dysphagia, which can negatively impact individual's physical and psychosocial well-being. Despite this, dysphagia in patients with DM remains poorly understood. This systematic review and meta-analysis aimed to evaluate the prevalence and clinical features of dysphagia in patients with DM and juvenile DM (JDM).
METHODS
Four electronic databases were systematically searched until September 2022. Studies with patients with DM or JDM and dysphagia were included. The pooled prevalence of all included studies was calculated, and the clinical characteristics of dysphagia were qualitatively analyzed.
KEY RESULTS
Thirty-nine studies with 3335 patients were included. The overall pooled prevalence of dysphagia was 32.3% (95% CI: 0.270, 0.373) in patients with DM and 37.7% (95% CI: -0.031, 0.785) in patients with JDM. Subgroup analyses revealed that Sweden had the highest prevalence (66.7% [95% CI: 0.289, 1.044]), whereas Tunisia had the lowest prevalence (14.3% [95% CI: -0.040, 0.326]). Moreover, South America had the highest prevalence (47.0% [95% CI: 0.401, 0.538]), whereas Africa had the lowest prevalence (14.3% [95% CI: -0.040, 0.326]). Dysphagia in patients with DM and JDM was characterized by both oropharyngeal and esophageal dysfunctions, with predominant difficulties in motility.
CONCLUSIONS & INFERENCES
Our findings showed that dysphagia affects one in three patients with DM or JDM. However, the documentation on the diagnosis and management of dysphagia in the literature is inadequate. Our results highlighted the need to use both clinical and instrumental assessments to evaluate swallowing function in this population.
Topics: Humans; Deglutition Disorders; Dermatomyositis; Prevalence; Esophageal Diseases; Deglutition
PubMed: 37010885
DOI: 10.1111/nmo.14572 -
International Journal of Rheumatic... Jun 2023A 58-year-old man with anti-melanoma differentiation-associated gene 5-positive dermatomyositis (MDA5-DM) developed Epstein-Barr virus (EBV)-associated malignant...
Other iatrogenic immunodeficiency-associated lymphoproliferative disorders in a patient with anti-melanoma differentiation-associated gene 5-positive dermatomyositis: A case report and systematic literature review.
A 58-year-old man with anti-melanoma differentiation-associated gene 5-positive dermatomyositis (MDA5-DM) developed Epstein-Barr virus (EBV)-associated malignant lymphoma as other iatrogenic immunodeficiency-associated lymphoproliferative disorders (OIIA-LPD) during the combined immunosuppressive therapy of high-dose prednisolone, tacrolimus, and intravenous cyclophosphamide for MDA5-DM. Serum EBV DNA was detected, and EBV-encoded small RNA was positive in the tissue sample of LPD, indicating that EBV reactivation contributed to the pathogenesis of LPD in our case. The patient underwent chemotherapy, including rituximab, promptly after discontinuation of tacrolimus and cyclophosphamide, resulting in complete remission of the malignant lymphoma, and MDA5-DM has not recurred with 3.5 mg/d of prednisolone monotherapy. We reviewed 19 cases of OIIA-LPD in patients with idiopathic inflammatory myopathies and herein report the first case of MDA5-DM complicated with OIIA-LPD. Among the 19 patients, 7 showed regression of LPD only following withdrawal of immunosuppressants, 9 took chemotherapy for LPD, and 5 died. It should be noted that patients with MDA5-DM-associated rapidly progressive interstitial lung disease could develop OIIA-LPD because they receive aggressive immunosuppressive therapy.
Topics: Male; Humans; Middle Aged; Epstein-Barr Virus Infections; Dermatomyositis; Tacrolimus; Herpesvirus 4, Human; Immunosuppressive Agents; Cyclophosphamide; Lymphoproliferative Disorders; Prednisolone; Iatrogenic Disease
PubMed: 36789793
DOI: 10.1111/1756-185X.14608 -
Autoimmunity Reviews Apr 2023Involvement of subcutaneous tissue in idiopathic inflammatory myopathies (IIM) is poorly known. (Review)
Review
INTRODUCTION
Involvement of subcutaneous tissue in idiopathic inflammatory myopathies (IIM) is poorly known.
METHODS
We conducted a systematic review of the literature regarding panniculitis and lipodystrophy/lipoatrophy in juvenile and adult IIM via PubMed/Medline, Embase and Scopus databases. Three local observations are included in this review. Epidemiological, clinical, paraclinical and therapeutic data were collected.
RESULTS
Panniculitis appears to be more common in adults than in juveniles. It was mainly localised in the upper and lower limbs. Panniculitis improved in most cases with steroids and panniculitis and myositis had a similar course in 83.3% and 72.2% of cases in juveniles and adults, respectively. Lipodystrophy appeared to be more frequent in juveniles and was only observed in dermatomyositis in both juveniles and adults. Lipodystrophy was mainly partial in juveniles and adults. The median time from myositis to the diagnosis of lipodystrophy was 6 years [0-35] and 2.5 years [0-10] in juveniles and adults, respectively. Lipodystrophy was associated with anti-TIF1 gamma auto-antibody positivity, a polycyclic/chronic course of myositis and the occurrence of calcinosis and might be an indicator of poor disease control.
CONCLUSION
Adipose tissue involvement, particularly lipodystrophy, occurs almost exclusively in dermatomyositis. The insidious onset and lack of awareness of the diagnosis may underestimate its prevalence. Larger studies are needed to identify possible risk factors in these patients, to better potential underlying pathophysiological process, in order to discuss potential therapeutic targets.
Topics: Adult; Humans; Dermatomyositis; Subcutaneous Tissue; Autoantibodies; Myositis; Panniculitis; Lipodystrophy
PubMed: 36736986
DOI: 10.1016/j.autrev.2023.103284 -
European Journal of Internal Medicine Apr 2023To evaluate the efficacy and safety of tacrolimus for dermatomyositis (DM) and polymyositis (PM) treatment. (Meta-Analysis)
Meta-Analysis
OBJECTIVE
To evaluate the efficacy and safety of tacrolimus for dermatomyositis (DM) and polymyositis (PM) treatment.
METHODS
We searched the Embase, PubMed, the Cochrane Central Register of Controlled Trials, and China National Knowledge Infrastructure were used as searching tools from inception up to October 2022. Two authors independently selected studies. The available studies were comprehensively reviewed and investigated.
RESULTS
A total of 9 studies, including 350 patients, were analysed. Pooled results showed a higher overall survival rate in tacrolimus therapy group. Creatine kinase (CK) levels and forced vital capacity (FVC) showed significant improvement after tacrolimus therapy. The incidence of adverse events including infection and renal dysfunction showed no significant differences between the tacrolimus therapy group and conventional therapy group.
CONCLUSION
The results of this meta-analysis indicated that GC therapy in combination with tacrolimus therapy could help improving overall survival rate, pulmonary function and had similar safety outcomes compared to conventional therapy in DM and PM patients.
Topics: Humans; Dermatomyositis; Drug Therapy, Combination; Immunosuppressive Agents; Polymyositis; Tacrolimus
PubMed: 36725399
DOI: 10.1016/j.ejim.2023.01.018 -
Medicine Jan 2023Chronic graft versus host disease (cGVHD) is a systemic immune-mediated complication that occurs in approximately half of patients undergoing allogeneic hematopoietic...
RATIONALE
Chronic graft versus host disease (cGVHD) is a systemic immune-mediated complication that occurs in approximately half of patients undergoing allogeneic hematopoietic stem cell transplantation (allo-HCT), and remains the leading cause of late morbidity and mortality. cGVHD involves a heterogeneous group of organic manifestations, many of which mimic autoimmune diseases such as scleroderma, primary biliary cholangitis, Sjögren syndrome and polymyositis.
PATIENT CONCERNS
A 60-years-old female with a history of allo-HCT developed de novo cGVHD 11 months after allo-HCT with isolated liver involvement. The patient presented with jaundice, cytolysis, cholestasis and concomitant acute digital ischemia. Liver biopsy and autoimmunity tests were performed and were found to be compatible with immune-mediated liver damage. Nailfold capillaroscopy revealed microangiopathy, characterized by avascular areas and some enlarged capillaries resembled an early systemic sclerosis pattern.
DIAGNOSIS
Biliary cholangitis-like and digital ischemia related to cGVHD.
INTERVENTIONS
The patient was treated with high-dose prednisone and ursodeoxycholic acid, and extracorporeal photopheresis. The patient required hospital admission for administration of intravenous prostacyclin due to refractory Raynaud syndrome.
OUTCOMES
After 6 to 8 weeks, the patient achieved a good response, with evident clinical improvement and progressive normalization of liver function.
LESSONS
cGVHD is a multiorgan pathological condition, and this case emphasizes that a multidisciplinary team, including rheumatologists, should be involved in the follow-up of allo-transplant patients to ensure that the clinical complications are adequately addressed. Early intervention is critical for improving patient' prognosis.In addition, we performed a systemic literature review based on published case articles on hepatic cGVHD and digital ischemia published up to August 2022. To the best of our knowledge, this is the first reported case of such an association.
Topics: Humans; Female; Middle Aged; Transplantation, Homologous; Hematopoietic Stem Cell Transplantation; Graft vs Host Disease; Cholangitis; Bronchiolitis Obliterans Syndrome; Scleroderma, Systemic; Ischemia; Chronic Disease
PubMed: 36637943
DOI: 10.1097/MD.0000000000032495 -
Frontiers in Immunology 2022Idiopathic inflammatory myopathies (IIMs) are a heterogeneous group of autoimmune diseases with various subtypes, myositis-specific antibodies, and affect multiple... (Meta-Analysis)
Meta-Analysis
OBJECTIVE
Idiopathic inflammatory myopathies (IIMs) are a heterogeneous group of autoimmune diseases with various subtypes, myositis-specific antibodies, and affect multiple systems. The treatment of IIMs remains challenging, especially for refractory myositis. In addition to steroids and traditional immunosuppressants, rituximab (RTX), a B cell-depleting monoclonal antibody, is emerging as an alternative treatment for refractory myositis. However, the therapeutic response to RTX remains controversial. This meta-analysis aimed to systematically evaluate the efficacy and safety of RTX in patients with IIMs, excluding sporadic inclusion body myositis.
METHODS
PubMed, Embase, Cochrane Library, China National Knowledge Infrastructure, and WanFang Data were searched for relevant studies. The overall effective rate, complete response rate, and partial response rate were calculated to assess the efficacy of RTX. The incidences of adverse events, infection, severe adverse events, severe infection, and infusion reactions were collected to evaluate the safety of RTX. Subgroup analyses were performed using IIM subtypes, affected organs, continents, and countries. We also performed a sensitivity analysis to identify the sources of heterogeneity.
RESULTS
A total of 26 studies were included in the quantitative analysis, which showed that 65% (95% confidence interval [CI]: 54%, 75%) of patients with IIMs responded to RTX, 45% (95% CI: 22%, 70%) of patients achieved a complete response, and 39% (95% CI: 26%, 53%) achieved a partial response. Subgroup analyses indicated that the overall efficacy rates in patients with refractory IIMs, dermatomyositis and polymyositis, as well as anti-synthetase syndrome were 62%, 68%, and 62%, respectively. The overall efficacy rates for muscle, lungs, and skin involvement were 59%, 65%, and 81%, respectively. In addition, studies conducted in Germany and the United States showed that patients with IIMs had an excellent response to RTX, with an effective rate of 90% and 77%, respectively. The incidence of severe adverse events and infections was 8% and 2%, respectively.
CONCLUSION
RTX may be an effective and relatively safe treatment choice in patients with IIMs, especially for refractory cases. However, further verification randomized controlled trials is warranted.
Topics: Humans; Rituximab; Myositis; Polymyositis; Immunosuppressive Agents; Autoimmune Diseases
PubMed: 36578492
DOI: 10.3389/fimmu.2022.1051609 -
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 -
Seminars in Arthritis and Rheumatism Dec 2022To evaluate the role of myositis-specific autoantibodies (MSAs) in interstitial lung disease (ILD), management of idiopathic inflammatory myopathies (IIM) associated... (Review)
Review
OBJECTIVE
To evaluate the role of myositis-specific autoantibodies (MSAs) in interstitial lung disease (ILD), management of idiopathic inflammatory myopathies (IIM) associated ILD, and if there is a role for MSA specific management of ILD.
METHODS
A systematic review was performed examining how MSAs relate to ILD manifestations in IIM patients and comparing treatment outcomes with varying immunosuppressive regimens.
RESULTS
112 papers were included in this analysis. Patients with anti-aminoacyl tRNA synthetase (anti-ARS) and anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibodies had consistently higher rates of ILD than other MSA groups. Anti-ARS positive patients had higher rates of chronic ILD whereas anti-MDA5 positive patients had higher rates of rapidly progressive ILD (RP-ILD). The most common high-resolution computed tomography (HRCT) patterns for ILD in anti-ARS and anti-MDA5 positive patients were nonspecific interstitial pneumonia (NSIP) and unclassifiable respectively. Anti-transcription intermediary factor 1-gamma (anti-TIF1-γ), anti-Mi-2, anti-nuclear matrix protein 2 (anti-NXP-2), and anti-3-hydroxy-3-methylglutaryl coenzyme A reductase (anti-HMGCR) antibodies were associated with a decreased risk of ILD. Small sample sizes, a lack of head-to-head trials, and non-randomized designs prevented drawing meaningful conclusions with respect to immunosuppressive management.
CONCLUSION
Clear relationships exist with regards to the ILD manifestations of certain MSAs. Standard therapy for IIM associated ILD (IIM-ILD) is glucocorticoids with the addition of others immunosuppressives in patients with or at risk of RP-ILD as well as in refractory cases. Immunosuppressives should be preferentially used in MSA populations in which they have been studied and shown to be efficacious.
Topics: Humans; Myositis; Lung Diseases, Interstitial; Autoantibodies; Immunosuppressive Agents; Retrospective Studies
PubMed: 36116345
DOI: 10.1016/j.semarthrit.2022.152088