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Frontiers in Immunology 2024We performed a single-arm meta-analysis to evaluate the efficacy and safety of JAK inhibitors in the treatment of dermatomyositis (DM)/ polymyositis (PM). (Meta-Analysis)
Meta-Analysis
INTRODUCTION
We performed a single-arm meta-analysis to evaluate the efficacy and safety of JAK inhibitors in the treatment of dermatomyositis (DM)/ polymyositis (PM).
METHODS
Relevant studies from four databases were systematically searched until April 25, 2023. The primary endpoint was Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI) and other outcomes were Manual Muscle Testing (MMT) and Creatine Kinase (CK). According to the type of JAK and medication regimen, we conducted subgroup analyses. The registration number in PROSPERO was CRD42023416493.
RESULTS
According to the selection criteria, we identified 7 publications with a total of 91 patients. Regarding skin lesions, the CDASI decreased by 17.67 (95% CI: -20.94 ~ -14.41). The CK increased by 8.64 U (95% CI: -28.25 ~ 45.53). About muscle lesions, MMT increased by 10.31 (95% CI: -2.83 ~ 23.46). Subgroup analysis revealed that different types of JAK inhibitors had various degrees of reduction. CDASI in patients treated with RUX had the lowest one [-20.00 (95% CI: -34.9 ~ -5.1)], followed by TOF [-18.29 (95% CI: -21.8 ~ -14.78)] and BAR [-11.2 (95% CI: -21.51 ~ -0.89)]. Additionally, the mean reduction in CDASI in patients treated with TOF alone was 16.16 (95% CI: -21.21 ~ -11.11), in combination with other immunosuppressants was 18.59 (95% CI: -22.74 ~ -14.45). For safety evaluation, one patient developed Orolabial HSV, and two patients developed thromboembolism events.
DISCUSSION
In summary, this meta-analysis demonstrated that JAK inhibitors can potentially treat DM/PM without severe adverse reactions.
SYSTEMATIC REVIEW REGISTRATION
https://www.crd.york.ac.uk/PROSPERO/display_record.php?ID=CRD42023416493, identifier CRD42023416493.
Topics: Humans; Dermatomyositis; Immunosuppressive Agents; Janus Kinase Inhibitors; Polymyositis; Skin
PubMed: 38576610
DOI: 10.3389/fimmu.2024.1382728 -
Frontiers in Immunology 2021The effectiveness of rituximab in anti-melanoma differentiation-associated gene 5 (MDA5) dermatomyositis (DM) with interstitial lung disease (ILD) has been explored only... (Meta-Analysis)
Meta-Analysis
OBJECTIVE
The effectiveness of rituximab in anti-melanoma differentiation-associated gene 5 (MDA5) dermatomyositis (DM) with interstitial lung disease (ILD) has been explored only in isolated case reports and small series. This paper aims to review the current evidence regarding rituximab (RTX) use in the treatment of ILD related to anti-MDA5 DM (anti-MDA5 DM-ILD).
METHODS
We conducted a review by searching PubMed, Web of Science, Embase, and Cochrane for articles with information on patients with anti-MDA5 DM and RTX treatment, published until August 2021, in English language. The selected studies listed variation in chest high-resolution computed tomography (HRCT) and/or pulmonary function test (PFT) as a primary outcome, in patients with anti-MDA5 DM-related ILD after using RTX.
RESULTS
Of the 145 potentially eligible articles, 17 were selected. The information gathered from a total of 35 patients with anti-MDA5 DM-ILD was reviewed, including 13 men and 22 women. Patient age at onset was 47.60 ± 13.72 years old. A total of 11.43% (4/35) of the patients were found to have chronic ILD (C-ILD) and 88.57% (31/30) exhibited rapidly progressive ILD (RP-ILD). Most patients (29/30) had typical DM rashes. Prior to RTX administration, the majority of patients (27/35) were treated with medium- or high-dose glucocorticoids and at least one additional immunotherapeutic agent. With regard to RTX efficacy for ILD in anti-MDA5 DM, 71.43% (25/35) of the patients responded to treatment. Skin rash also improved in more than half of the patients after RTX treatment. The most common side effects were infections, reported by 37.14% (13/35) of the patients after using RTX.
CONCLUSION
As a CD20 targeting drug, RTX is a promising therapeutic tool for anti-MDA5 DM-ILD, although the risk of infections should be considered before treatment. Further prospective controlled studies are required to evaluate the optimal RTX treatment regimen.
SYSTEMATIC REVIEW REGISTRATION
https://www.crd.york.ac.uk/prospero/display_record.php?ID=CRD42021289714, identifier CRD42021289714.
Topics: Adult; Aged; Antirheumatic Agents; Biomarkers; Dermatomyositis; Disease Management; Disease Susceptibility; Female; Humans; Interferon-Induced Helicase, IFIH1; Lung Diseases, Interstitial; Male; Middle Aged; Molecular Targeted Therapy; Rituximab; Treatment Outcome
PubMed: 35116041
DOI: 10.3389/fimmu.2021.820163 -
Seminars in Arthritis and Rheumatism Aug 2024Drug-induced dermatomyositis (DIDM) is a rare and underestimated variant of dermatomyositis (DM) characterized by muscle damage and skin rash and related to certain drug... (Review)
Review
Drug-induced dermatomyositis (DIDM) is a rare and underestimated variant of dermatomyositis (DM) characterized by muscle damage and skin rash and related to certain drug exposure. The spectrum of drugs causing DIDM has evolved over time, originally implicating hydroxyurea, penicillamine, and statins as causative agents. Tumor necrosis factor α inhibitors and immune checkpoint inhibitors have also been associated with such conditions. To bridge the gap between current literature and clinical practice, and therefore guide clinicians, we conducted a comprehensive review of English literature from Pubmed, EMBASE, and MEDLINE. Our analysis included demographic data, clinical features, laboratory findings, therapeutic outcomes, and extant research pertaining to the probable pathogenesis of DIDM induced by various drugs. Furthermore, we categorized the drugs involved in DIDM cases into biologics and traditional agents for subsequent statistical analysis. Over time, there has been a gradual accumulation of reported DIDM cases. A total of 69 published DIDM cases were documented in our study, among which 33 should be attributed to biologics and the remaining 36 to traditional drugs. Interestingly, 41 of all DIDM cases had a previous history of malignancies. Additionally, DIDM cases exhibited similar cutaneous and muscular manifestations to classic DM, with the exception of cases induced by hydroxyurea, which did not entail muscle damage. Positive antinuclear antibodies and anti-TIF1-γ autoantibodies have been predominantly observed in biologics-induced cases, while positive anti-TIF1-γ antibodies were merely reported in the cases that were primarily diagnosed with malignant diseases and exposed to ICIs afterwards. Anti-TIF1-γ antibodies may potentially serve as a red flag in the identification of co-existing malignant diseases in DM patients. We also provided a comprehensive summary and exploration of potential mechanisms lying behind drug-induced dermatomyositis. In conclusion, our review consolidates the current literature on DIDM, highlighting the evolving spectrum of medications and elucidating the differences in clinical manifestations, laboratory findings, and underlying mechanisms.
Topics: Dermatomyositis; Humans; Biological Products
PubMed: 38833729
DOI: 10.1016/j.semarthrit.2024.152478 -
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 -
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 -
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 -
Rheumatology (Oxford, England) May 2022This systemic review assesses skin tone representation in images of DM rashes in medical education literature.
OBJECTIVE
This systemic review assesses skin tone representation in images of DM rashes in medical education literature.
METHODS
A review was performed of 59 dermatology, 11 neurology, 10 neuromuscular, 7 rheumatology and 6 internal medicine textbooks published between 2011 and 2021 and 3 online image databases (UpToDate, VisualDx and DermNet NZ) that were available through an online medical school library. After extracting images, images with poor lighting or unclear rashes were removed. Authors graded skin tone independently on the Massey and Martin Skin Colour Scale (MMSCS) from 1 (very light) to 10 (very dark). The median score was taken for a final score, grouped within MMSCS 1-2, 3-4, 5-7 or 8-10. Inter-rater reliability was assessed using Kendall's coefficient of concordance (W).
RESULTS
Six hundred and twenty-one images were extracted after reviewing 93 textbooks and 3 online databases. Of the 561 images analysed, 73.1% of images represented MMSCS 1-2, followed by 3-4 (13.4%), 5-7 (11.8%) and 8-10 (1.8%). Inter-rater reliability was high (W = 0.835). Of the images in MMSCS 5-10, 59.2% were in online databases and 80.6% of textbook images were in dermatology books.
CONCLUSIONS
Patients with lighter skin tones were represented in a higher number of DM-related educational materials compared with patients with darker skin tones. Our findings add to current research implicating that darker skin tones are under-represented in cutaneous educational materials, specifically for DM. This leads to the inability to properly characterize skin involvement in DM and may lead to inappropriate exclusion from clinical trials due to erroneous skin scoring.
Topics: Dermatomyositis; Exanthema; Humans; Racial Groups; Reproducibility of Results; Skin; Skin Pigmentation
PubMed: 34718435
DOI: 10.1093/rheumatology/keab809 -
Clinical Rheumatology Aug 2019The safety and effect of physical therapy in adult patients with idiopathic inflammatory myopathies (IIMs) are currently unclear. Considering the muscle weakness...
The safety and effect of physical therapy in adult patients with idiopathic inflammatory myopathies (IIMs) are currently unclear. Considering the muscle weakness resulting from disease activity as well as from the administered drugs, these patients could benefit from an evidence-based physical therapy program. To perform a systematic review to assess safety and effects of physical therapy on the functional outcome of patients with idiopathic inflammatory myopathies in both active and quiescent disease: Pubmed, Embase, and Cochrane. Patients with one of the following idiopathic inflammatory myopathies: polymyositis, dermatomyositis, immune-mediated necrotizing myopathy, and/or overlap myositis. The intervention included several types of rehabilitation programs, from strength and resistance training to endurance training, with a minimal duration of 1 month. Studies reporting intervention-related adverse events, disease activity, and functional outcomes were eligible. The risk of bias was assessed using the Cochrane guidelines. We included five randomized controlled and seven open-label non-randomized non-controlled trials. Data on statistical significance were extracted for all the trials. Included trials were of medium-quality evidence given the low number of patients and some risk of bias factors. Physical therapy does not have a negative effect on the disease activity of idiopathic inflammatory myopathies in quiescent disease and could improve functional outcome. The physical therapy program should minimally include endurance training. A combination with resistance training might be beneficial.
Topics: Adult; Dermatomyositis; Evidence-Based Medicine; Exercise Therapy; Humans; Muscle Weakness; Myositis; Physical Therapy Modalities; Randomized Controlled Trials as Topic; Rehabilitation; Resistance Training; Treatment Outcome
PubMed: 31115788
DOI: 10.1007/s10067-019-04571-9 -
Journal of Clinical Rheumatology :... Jun 2021There is a well-known association between malignancy and dermatomyositis but reports of an association between malignancies of the thyroid gland and dermatomyositis are...
OBJECTIVE
There is a well-known association between malignancy and dermatomyositis but reports of an association between malignancies of the thyroid gland and dermatomyositis are very few. Here we describe a recent case of dermatomyositis found to have thyroid cancer during screening and review the clinical features of the similar cases in literature.
METHODS
The case history, treatment responses and follow up data are described. We performed a systematic literature review using the keywords (thyroid cancer OR thyroid carcinoma) AND dermatomyositis.
RESULTS
35 year old male presented with an acute onset of symptoms including facial rash, widespread myalgia, muscle weakness and dysphagia. Patient was diagnosed with dermatomyositis based on clinical findings, laboratory, electromyography and imaging results and immunosuppressive treatment initiated. Screening for cancer revealed a papillary thyroid carcinoma. Thyroidectomy was performed and immunosuppressive medication was gradually tapered and stopped. At 24 months following the diagnosis patient remains in remission. This is the 14th reported case of dermatomyositis and thyroid cancer in the English literature. Analysis of data from these 14 cases while revealing conflicting insights about the link between dermatomyositis and thyroid cancer do not rule out this possibility. Treatment of thyroid cancer appeared to have a significant influence on the course of dermatomyositis in at least six of these cases.
CONCLUSION
Better recognition of the link between dermatomyositis and thyroid cancer may allow physicians to protect some dermatomyositis patients from morbidity and mortality associated with immunosuppression.
Topics: Adult; Dermatomyositis; Humans; Male; Muscle Weakness; Thyroid Cancer, Papillary; Thyroid Neoplasms; Thyroidectomy
PubMed: 32803919
DOI: 10.1097/RHU.0000000000001048 -
Clinical Rheumatology Mar 2022Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) which preferentially infiltrates into subcutaneous adipose tissue is rare, however may mimic autoimmune diseases... (Review)
Review
Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) which preferentially infiltrates into subcutaneous adipose tissue is rare, however may mimic autoimmune diseases from the aspect of clinical manifestations. Here, we describe a 16-year-old young man, who initially presented with eyelid erythema and swelling, accompanied by fever and muscle and bone marrow involvement. He was preliminarily considered as a patient with classical dermatomyositis (DM), but finally diagnosed as SPTCL concomitant with paraneoplastic inflammatory myositis, confirmed by in total 8 times repeated biopsies at different sites. After systematically reviewing the literatures, we summarized the main features of SPTCL mimicking DM with eyelid edema as the presenting manifestation. The cautionary tale reminds rheumatologists of considering mimickers in patients with atypical autoimmune-like manifestations. Suitable biopsy is critical for diagnosis and improving prognosis.
Topics: Adolescent; Dermatomyositis; Diagnosis, Differential; Erythema; Eyelids; Humans; Lymphoma, T-Cell; Male; Panniculitis
PubMed: 34786628
DOI: 10.1007/s10067-021-05992-1