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Clinical and Experimental Rheumatology Feb 2022Juvenile idiopathic inflammatory myopathies (JIIMs) are a heterogeneous group of systemic autoimmune diseases. Juvenile dermatomyositis (JDM) is the predominant form of...
OBJECTIVES
Juvenile idiopathic inflammatory myopathies (JIIMs) are a heterogeneous group of systemic autoimmune diseases. Juvenile dermatomyositis (JDM) is the predominant form of JIIMs, and is a rare, chronic autoimmune illness characterised by symmetric, proximal muscle damages and involvement of the skin. In the last two decades, the use of monoclonal antibodies has also been expanded to JIIMs; however, there is limited evidence on use of these treatments. We assessed the efficacy/effectiveness and safety of biologic agents in JIIMs.
METHODS
A systematic literature review was conducted using Embase®, MEDLINE®, MEDLINE®-In Process and Cochrane library to identify studies on biologics agents in JIIMs published in English language as full-text articles (1975 to December 2020) or conference abstracts (2000 to December 2020). Databases were searched with the key words regarding chronic myositis crossed with "biologic agents OR tocilizumab OR rituximab OR adalimumab OR infliximab OR anti-TNF OR etanercept". Of note, we did not include children, age, or age limits in the search as medical subject headings terms because we may have been able to extract a sub cohort of children from studies including both children and adults.
RESULTS
Of the 1633 retrieved publications, 18 articles were identified for a total of 165 patients. In real-world studies, definition of complete (CR) or partial response (PR) varied. JIIMs patients were most often treated with anti-TNF (88 pts); patients received etanercept (ETA), 48 patients infliximab (IFX), 4 patients received adalimumab (ADA). In other 15 patients IFX was followed by ADA. Rituximab (RTX) was used in 73 children. A single case series reported the use of abatacept (ABA) in 4 patients. Despite the reduced number of treated patients, complete response on myositis was reported in 29.6% (8/26) patients treated with at least one anti-TNF and in 38% (10/26) treated by RTX. Complete response of skin vasculitis has been reached in 33% (4/12) children on anti-TNF and in 36% on RTX (21/58). Anti-TNF agents might be efficient in treating calcinosis lesions.
CONCLUSIONS
Currently, the available evidence regarding the use of biologic treatment in JIIMs results quite limited but suggest a promising the use of anti-TNF agents and RTX in treating active JIIMs. Anti-TNF treatment might have a role in treating calcinosis. However, an overall very low quality of the available studies and multiple confounding factors hamper to suggest a treatment over another. Thus, randomised clinical trials are urgently required to attempt the optimal treatment in real-world setting.
Topics: Adalimumab; Adult; Antirheumatic Agents; Biological Products; Biological Therapy; Child; Etanercept; Humans; Infliximab; Myositis; Precision Medicine; Tumor Necrosis Factor Inhibitors
PubMed: 34905479
DOI: 10.55563/clinexprheumatol/ltrj4l -
Journal of Clinical Rheumatology :... Mar 2022Immune-related adverse events (irAEs) from immune checkpoint inhibitors (ICIs) are sometimes associated with autoantibodies, but we do not know how frequently or whether...
BACKGROUND
Immune-related adverse events (irAEs) from immune checkpoint inhibitors (ICIs) are sometimes associated with autoantibodies, but we do not know how frequently or whether these autoantibodies are present before ICI initiation. Our aim was to determine the positivity rate of autoantibodies in patients with organ-specific ICI-associated irAEs and determine their value as pretreatment biomarkers.
METHODS
We searched for all English, peer-reviewed publications from MEDLINE, Embase, and Cochrane Library through February 20, 2020, and included any publication describing patients with irAEs and reporting results of any autoantibody investigation. Three reviewers independently extracted data, and 1 reviewer verified all data for accuracy and quality of reporting.
RESULTS
We identified 515 publications. Most reports described endocrine, rheumatic, gastrointestinal/hepatic, and myositis/myasthenia/myocarditis irAEs. Autoantibodies were present in close to 50% of patients with ICI-associated endocrinopathies. Anti-BP180 was found in more than 50% of patients with skin irAEs. Antibodies were also common in patients with the triad of myositis/myasthenia/myocarditis including striational antibodies (49%), acetylcholine receptor antibodies (40%), and myositis-associated antibodies (27%). Only 11% of patients with arthritis had either rheumatoid factor or cyclic citrullinated peptide antibodies, and only 30% of patients with sicca had Sjögren antibodies. Autoantibodies were also relatively uncommon in patients with hepatitis (antinuclear antibody, 18%) and colitis (perinuclear antineutrophil cytoplasmic antibody, 19%). Some cohort studies analyzing pre-ICI seropositivity suggest there may be a role for autoantibodies as biomarkers of irAEs.
CONCLUSIONS
Reported autoantibody positivity is high in irAEs involving the endocrine organs, skin, and muscle, but lower in irAEs affecting other organ systems. Autoantibody investigations in pre-ICI treatment patients have yielded mixed results regarding their utility as a biomarker of irAEs.
Topics: Autoantibodies; Humans; Immune Checkpoint Inhibitors; Myositis; Neoplasms; Rheumatoid Factor
PubMed: 34371516
DOI: 10.1097/RHU.0000000000001777 -
Seminars in Arthritis and Rheumatism Feb 2024The biomarkers for predicting the occurrence, progression, and death of idiopathic inflammatory myopathy-associated interstitial lung disease (IIM-ILD) remain unclear.... (Meta-Analysis)
Meta-Analysis Review
OBJECTIVE
The biomarkers for predicting the occurrence, progression, and death of idiopathic inflammatory myopathy-associated interstitial lung disease (IIM-ILD) remain unclear. Serum ferritin (SF) is a potential candidate and this systematic review and meta-analysis aimed to reveal the clinical significance of SF in IIM-ILD.
METHODS
Eligible English studies were selected from PubMed, Embase, Web of science and Scopus up to 9 June 2023. The SF levels in patients with IIM-ILD were extracted and pooled. Subgroup analysis was performed based on disease types, sensitivity analysis was conducted by excluding one class of literature at a time, and publication bias was assessed by funnel plot and Egger's test.
RESULTS
Pooled analysis of 1,933 patients with IIM from 19 studies showed that SF levels were significantly higher in IIM-ILD group (WMD=263.53ng/mL, 95% CI: 146.44-380.62, p<0.001) than IIM without ILD, subgroup analysis showed that SF levels in DM-ILD (WMD = 397.67ng/mL, 95% CI:142.84-652.50, p = 0.002) and PM/DM-ILD (WMD = 117.68 ng/mL, 95% CI: 86.32-149.04, p < 0.001) were significantly higher compared to those without ILD. SF levels were significantly higher in rapidly progressive interstitial lung disease group (RP-ILD)(WMD = 484.99 ng/mL, 95% CI: 211.12-758.87, p= 0.001) than chronic ILD(C-ILD) group, subgroup analysis showed that SF levels in DM-RP-ILD (WMD= 509.75 ng/mL, 95% CI: 215.34-804.16, p=0.001) were significantly higher than those in DM-C-ILD group. SF levels were significantly higher in death group (WMD= 722.16 ng/mL, 95% CI: 572.32-872.00, p< 0.001) compared to the survival group, subgroup analysis showed that death patients with DM-ILD(WMD= 735.62 ng/mL, 95% CI:574.92-896.32, p<0.001) and PM-ILD (WMD= 632.56 ng/mL, 95% CI:217.92-1047.19, p=0.003) had significantly higher SF levels than survival group respectively.
CONCLUSION
Increased SF levels can serve as a biomarker for predicting the occurrence, progression and death of patients with IIM-ILD, which can provide early warning sign for intervention and prognosis evaluation for IIM-ILD patients.
Topics: Humans; Myositis; Lung Diseases, Interstitial; Biomarkers; Prognosis; Ferritins; Retrospective Studies
PubMed: 38086199
DOI: 10.1016/j.semarthrit.2023.152350 -
Frontiers in Immunology 2022The SARS-CoV-2 infection has been advocated as an environmental trigger for autoimmune diseases, and a paradigmatic example comes from similarities between COVID-19 and...
INTRODUCTION
The SARS-CoV-2 infection has been advocated as an environmental trigger for autoimmune diseases, and a paradigmatic example comes from similarities between COVID-19 and the myositis-spectrum disease associated with antibodies against the melanoma differentiation antigen 5 (MDA5) in terms of clinical features, lung involvement, and immune mechanisms, particularly type I interferons (IFN).
CASE REPORT
We report a case of anti-MDA5 syndrome with skin manifestations, constitutional symptoms, and cardiomyopathy following a proven SARS-CoV-2 infection.
SYSTEMATIC LITERATURE REVIEW
We systematically searched for publications on inflammatory myositis associated with COVID-19. We describe the main clinical, immunological, and demographic features, focusing our attention on the anti-MDA5 syndrome.
DISCUSSION
MDA5 is a pattern recognition receptor essential in the immune response against viruses and this may contribute to explain the production of anti-MDA5 antibodies in some SARS-CoV-2 infected patients. The activation of MDA5 induces the synthesis of type I IFN with an antiviral role, inversely correlated with COVID-19 severity. Conversely, elevated type I IFN levels correlate with disease activity in anti-MDA5 syndrome. While recognizing this ia broad area of uncertainty, we speculate that the strong type I IFN response observed in patients with anti-MDA5 syndrome, might harbor protective effects against viral infections, including COVID-19.
Topics: Antigens, Differentiation; Autoimmune Diseases; Autoimmunity; Biomarkers; COVID-19; Humans; Interferon Type I; Interferon-Induced Helicase, IFIH1; Melanoma; Myositis; SARS-CoV-2
PubMed: 35833112
DOI: 10.3389/fimmu.2022.937667 -
Clinical and Experimental Rheumatology Feb 2022Dermatomyositis (DM) and juvenile dermatomyositis (JDM) are idiopathic inflammatory myopathies, which can be resistant and unresponsive to initial treatments, leading to...
OBJECTIVES
Dermatomyositis (DM) and juvenile dermatomyositis (JDM) are idiopathic inflammatory myopathies, which can be resistant and unresponsive to initial treatments, leading to severe complications and impaired quality of life. There are few randomised trials in dermatomyositis and the outcomes reported may not be consistent, which can limit decision-making. The aim of this study is to assess the scope and consistency of outcomes reported in randomised trials in dermatomyositis.
METHODS
MEDLINE, Embase, PsycINFO and clinicaltrials.gov were searched from 1993-2020 for randomised trials in children and adults with dermatomyositis. The frequency and characteristics of the outcomes reported were analysed and classified.
RESULTS
20 trials were included. Across these trials, a total of 743 outcome measures were reported, which were grouped into 34 outcome domains; of which 17 were clinical, 13 were surrogate/biochemical, and 4 were patient-reported outcomes. The top five most frequently reported outcome domains were muscle inflammation (15 trials, 46 outcome measures), physical function (14 trials, 16 outcome measures), muscle strength (13 trials, 30 outcome measures), global health (12 trials, 33 outcome measures) and immunologic marker (11 trials, 91 outcomes).
CONCLUSIONS
The majority of outcomes reported in trials in people with dermatomyositis and JDM are clinical and surrogate outcomes rather than patient-reported outcomes. The outcomes reported are very inconsistent across trials, with wide heterogeneity in the measures used. Standardised reporting of critically important outcomes is needed to strengthen the value of trials for decision-making.
Topics: Adult; Child; Dermatomyositis; Humans; Outcome Assessment, Health Care; Patient Reported Outcome Measures; Quality of Life; Randomized Controlled Trials as Topic
PubMed: 35225217
DOI: 10.55563/clinexprheumatol/3izscd -
Reumatismo May 2023The COVID-19 pandemic represents a global health problem, which has been mitigated by the opportune introduction of vaccination programs. Although we already know the...
The COVID-19 pandemic represents a global health problem, which has been mitigated by the opportune introduction of vaccination programs. Although we already know the benefit that vaccines provide, these are not exempt from adverse events which can be mild to deadly, such as idiopathic inflammatory myopathies, in which a temporal association has not been defined. It is for this reason that we carried out a systematic review of all reported cases of vaccination against COVID-19 and myositis. To identify previously reported cases of idiopathic inflammatory myopathies associated with vaccination against SARS-CoV-2 we registered this protocol on the website of PROSPERO with identification number CRD42022355551. Of the 63 publications identified in MEDLINE and 117 in Scopus, 21 studies were included, reporting 31 cases of patients with vaccination-associated myositis. Most of these cases were women (61.3%); mean age was 52.3 years (range 19-76 years) and mean time of symptom onset post-vaccination was 6.8 days. More than half of the cases were associated with Comirnaty, 11 cases (35.5%) were classified as dermatomyositis, and 9 (29%) as amyopathic dermatomyositis. In 6 (19.3%) patients another probable trigger was identified. Case reports of inflammatory myopathies associated with vaccination have heterogeneous presentations without any specific characteristics: as a consequence, it is not possible to ensure a temporal association between vaccination and the development of inflammatory myopathies. Large epidemiological studies are required to determine the existence of a causal association.
Topics: Humans; Female; Infant, Newborn; Infant; Male; SARS-CoV-2; Pandemics; COVID-19; Myositis; Vaccination
PubMed: 37154256
DOI: 10.4081/reumatismo.2023.1548 -
Frontiers in Immunology 2021The idiopathic inflammatory myopathies (IIM) are characterized by muscular weakness, cutaneous manifestations, muscle damage revealed by increase of muscular enzymes,...
The idiopathic inflammatory myopathies (IIM) are characterized by muscular weakness, cutaneous manifestations, muscle damage revealed by increase of muscular enzymes, muscle biopsy, electromyography and changes on magnetic resonance imaging. However, the hallmark of these IIM, is the development of myositis specific antibodies (MSA) or myositis associated antibodies (MAA). The theories about their presence in the serum of IIM is not known. Some studies have suggested that some of these MSA, such as anti-Mi-2 increases according to the intensity of UV radiation. There is scarce information about the environmental factors that might contribute in order to be considered as triggering factors as UV radiation might be. In this review, we analyzed the reported prevalence of MSAs and MAAs regarding to their geographical location and the possible relation with UV radiation. We collected the prevalence data of fifteen MSA and thirteen MAA from 22 countries around the world and we were able to observe a difference in prevalence between countries and continents. We found differences in anti-PL7, anti-Ro52, anti-La and anti-Ku prevalence according to UV radiation level. Otherwise, we observed that anti-Mi-2 prevalence increases near to the Equator meanwhile anti-MJ/NXP2 and anti-ARS prevalence had an opposite behavior increasing their prevalence in the geographical locations farther to the Equator. Our results highlighted the importance to include the UV radiation and other environmental factors in IIM studies, in order to clarify its association with MSA and MAA prevalence as well as its possible role in the immunopathogenesis of these diseases.
Topics: Autoantibodies; Autoimmune Diseases; Geography; Humans; Myositis; Prevalence
PubMed: 33968081
DOI: 10.3389/fimmu.2021.672008 -
Journal of Orthopaedic Case Reports 2018Treatment and risk factors for Parvimonas micra spinal infections are scarcely researched. This study reports a case and presents a systematic review of the literature...
INTRODUCTION
Treatment and risk factors for Parvimonas micra spinal infections are scarcely researched. This study reports a case and presents a systematic review of the literature to provide evidence-based ground for diagnosis and treatment of P. micra spinal infections.
CASE REPORT
This is a case of a 78-year-old male with severe back and leg pain. Advanced imaging demonstrated the destruction of L2-L3 with an extensive fluid collection in the remaining intervertebral space, paravertebral myositis, and multiple abscesses. A decompression of L2 and L3 and a posterior spondylodesis from T12 to L5 was performed. Intraoperative cultures showed P. micra. The postoperative treatment consisted of intravenous penicillin for 2 weeks and subsequent oral clindamycin for 4 weeks. At 1-year follow-up, the patient was in good health and reported only occasional back pain.
CONCLUSIONS
A total of 15 additional cases of P. micraspinal infections were identified. The antibiotic treatment showed a great variety in the treated patients. Nevertheless, the outcome of these patients was good concerning relapse of the infection and pain. Spinal infections caused by P. micra are rare, but can be successfully treated according to the guidelines for spinal infection.
PubMed: 30740380
DOI: 10.13107/jocr.2250-0685.1216 -
Hellenic Journal of Nuclear Medicine 2021The purpose of current investigation was to evaluate the diagnostic accuracy of fluorine-18-fluorodeoxyglucose (F-FDG) positron emission tomography/computed tomography... (Meta-Analysis)
Meta-Analysis
OBJECTIVE
The purpose of current investigation was to evaluate the diagnostic accuracy of fluorine-18-fluorodeoxyglucose (F-FDG) positron emission tomography/computed tomography (PET/CT) for the determination of disease activity of idiopathic inflammatory myopathies (IM) using diagnostic accuracy test.
SUBJECTS AND METHODS
The PubMed and EMBASE database, from the earliest available date of indexing through August 31, 2020, were searched for results investigating the diagnostic accuracy of F-FDG PET/CT for the determination of disease activity of IM. We calculated the pooled sensitivities and specificities of included studies, calculated positive and negative likelihood ratios (LR+ and LR-), and obtained summary receiver operating characteristic (SROC) curves.
RESULTS
Across 4 studies with 5 results (90 patients), the pooled sensitivity was 0.94 (95% CI; 0.87-0.97) without heterogeneity (I=0.0, P=0.8) and a pooled specificity was 0.90 (95% CI; 0.72-0.97) with heterogeneity (I=65.1, P=0.02). Likelihood ratio (LR) syntheses showed an overall positive likelihood ratio (LR+) of 9.2 (95% CI; 3.1-28.0) and negative likelihood ratio (LR-) of 0.07 (95% CI; 0.03-0.16). The pooled DOR was 131 (95% CI; 26-664). The hierarchical SROC curve shows the areas under the curve of 0.96 (95% CI; 0.94-0.98).
CONCLUSION
Fluorine-18-FDG PET/CT has a good performance for the detection of active disease status in patients with IM. Although there are no guidelines for adopting imaging techniques, more objective and updated criteria for the assessment of disease activity incorporated with F-FDG PET/CT should be introduced and validated. Further studies are necessary to determine if 18F-FDG PET/CT-based treatment of IM can improve outcomes.
Topics: Fluorodeoxyglucose F18; Humans; Myositis; Positron Emission Tomography Computed Tomography; Positron-Emission Tomography; Radiopharmaceuticals; Sensitivity and Specificity
PubMed: 34352048
DOI: 10.1967/s002449912353 -
Journal of Autoimmunity Feb 2021The diverse clinical manifestations of COVID-19 is emerging as a hallmark of the Severe Acute Respiratory Syndrome Coronavirus-2 (SARS-CoV-2) infection. While the...
The diverse clinical manifestations of COVID-19 is emerging as a hallmark of the Severe Acute Respiratory Syndrome Coronavirus-2 (SARS-CoV-2) infection. While the initial target of SARS-CoV-2 is the respiratory tract, it is becoming increasingly clear that there is a complex interaction between the virus and the immune system ranging from mild to controlling responses to exuberant and dysfunctional multi-tissue directed autoimmune responses. The immune system plays a dual role in COVID-19, being implicated in both the anti-viral response and in the acute progression of the disease, with a dysregulated response represented by the marked cytokine release syndrome, macrophage activation, and systemic hyperinflammation. It has been speculated that these immunological changes may induce the loss of tolerance and/or trigger chronic inflammation. In particular, molecular mimicry, bystander activation and epitope spreading are well-established proposed mechanisms to explain this correlation with the likely contribution of HLA alleles. We performed a systematic literature review to evaluate the COVID-19-related autoimmune/rheumatic disorders reported between January and September 2020. In particular, we investigated the cases of incident hematological autoimmune manifestations, connective tissue diseases, antiphospholipid syndrome/antibodies, vasculitis, Kawasaki-like syndromes, acute arthritis, autoimmune-like skin lesions, and neurologic autoimmune conditions such as Guillain-Barré syndrome. We screened 6263 articles and report herein the findings of 382 select reports which allow us to conclude that there are 2 faces of the immune response against SARS-CoV-2, that include a benign virus controlling immune response and a many faceted range of dysregulated multi-tissue and organ directed autoimmune responses that provides a major challenge in the management of this viral disease. The number of cases for each disease varied significantly while there were no reported cases of adult onset Still disease, systemic sclerosis, or inflammatory myositis.
Topics: Animals; Autoimmune Diseases; COVID-19; Chronic Disease; Humans; Immunity; Incidence; Inflammation; Janus Kinases; SARS-CoV-2
PubMed: 33401171
DOI: 10.1016/j.jaut.2020.102592