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Rheumatology (Oxford, England) Oct 2023Dysphagia is a common debilitating clinical feature of IBM. However, the impact of dysphagia in IBM has been historically overlooked. This study aimed to identify,...
OBJECTIVES
Dysphagia is a common debilitating clinical feature of IBM. However, the impact of dysphagia in IBM has been historically overlooked. This study aimed to identify, evaluate and summarize the evidence regarding the assessment and management of dysphagia in persons with IBM undergoing treatment.
METHODS
A systematic review was conducted using a multiengine search following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses 2020 guidelines. Eligible studies had to employ an intervention for persons with IBM, report a swallowing outcome and be published in English. Quality assessments of the eligible studies were performed.
RESULTS
Of 239 studies found, 19 met the inclusion criteria. One study was rated as 'fair' and the rest as 'poor' quality, particularly due to the lack of published and validated swallowing assessment procedures and outcome measures. Cricopharyngeal (CP) dysfunction (12/19) was the most commonly reported swallowing abnormality. Interventions for disease management included pharmacological agents (10/19), followed by surgical (3/19), behavioral (1/19) and combined approaches (5/19). Interventions with immunosuppressants, botulinum toxin injection, balloon dilation and/or CP myotomy led to mixed and transient benefits. Few studies examining statins or behavioral therapies (primarily focused on respiratory function) showed no effects for dysphagia.
CONCLUSION
Various interventions have been reported to temporarily improve dysphagia in persons with IBM. However, these findings are based on limited and overall low-quality evidence. This study cautions against the generalization of these findings and emphasizes the need for further systematic research to improve the diagnosis and management of dysphagia in IBM.
Topics: Humans; Deglutition Disorders; Myositis, Inclusion Body; Pharyngeal Muscles; Endoscopy
PubMed: 37115631
DOI: 10.1093/rheumatology/kead194 -
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 -
Autoimmunity Reviews Oct 2023Juvenile idiopathic inflammatory myopathies (JIIM) are a group of connective tissue disorders characterized by muscle inflammation and variable systemic involvement,... (Review)
Review
OBJECTIVE
Juvenile idiopathic inflammatory myopathies (JIIM) are a group of connective tissue disorders characterized by muscle inflammation and variable systemic involvement, including interstitial lung disease (ILD). Available data on JIIM-associated ILD are very limited. We performed a systematic review of the available clinical, laboratory, and radiological features of JIIM-associated ILD.
METHODS
A systematic literature review was performed in accordance with Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement.
RESULTS
A total of 90 patients were identified, of whom 77.8% had JDM, 10% amyopathic JDM, 7.8% anti-synthetase syndrome, 3.3% overlap syndrome, and 1.1% juvenile polymyositis. Anti-melanoma differentiation-associated gene 5 (MDA-5/CADM-140) was the most frequently reported myositis-specific antibody (32.2%). At diagnosis of ILD, 55.5% of patients had respiratory symptoms. Ground glass opacity was the most reported radiological feature (52.9%). Thirty-three % of patients developed rapidly progressive (RP) lung disease; 26.7% were admitted to the intensive care unit (ICU); 28.9% died; all deaths were due to ILD, with a median interval of 2 months (IQR 1.5-4.7) between the onset of respiratory symptoms and death. Patients admitted to the ICU and who died of ILD were more likely to be male, to have a rapidly progressive pattern, progression of radiological features, and a higher level of KL-6.
CONCLUSIONS
MDA-5/CADM-14 is associated with RP-ILD. ILD is a rare but severe manifestation among the spectrum of systemic involvement associated with JIIM, with a high rate of ICU admission and mortality. Early recognition and aggressive treatment are needed to prevent a severe outcome.
Topics: Humans; Male; Female; Dermatomyositis; Myositis; Polymyositis; Lung Diseases, Interstitial; Lung; Autoantibodies; Retrospective Studies
PubMed: 37611886
DOI: 10.1016/j.autrev.2023.103416 -
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 -
BMJ Open Respiratory Research Nov 2023Interstitial lung disease (ILD) is an important manifestation of autoimmune diseases that can lead to morbidity and mortality. Although several autoantibodies have been... (Meta-Analysis)
Meta-Analysis
OBJECTIVES
Interstitial lung disease (ILD) is an important manifestation of autoimmune diseases that can lead to morbidity and mortality. Although several autoantibodies have been linked with ILD presentation and adverse outcomes, the association of anti-Ro52 antibody with ILD is less studied. Hence, we investigated this association in various autoimmune diseases in the current study.
DESIGN
We designed a systematic review and meta-analysis and did a comprehensive search from inception until 2 January 2023.
DATA SOURCES
A systematic search was conducted in four electronic databases: PubMed, Web of Science, Scopus and Embase.
ELIGIBILITY CRITERIA
Observational studies that reported ILD diagnosis (outcome) and anti-Ro antibody (exposure) status in any autoimmune conditions (population) were included. The association between rapidly progressive ILD (RP-ILD) and anti-Ro52 was studied in idiopathic inflammatory myopathies (IIM).
DATA EXTRACTION AND SYNTHESIS
Collected data included study characteristics and ORs with 95% CIs. Quality assessment was performed using a modified version of the Newcastle-Ottawa Scale for cross-sectional studies. Random effects meta-analysis was used to pool the effect estimates.
RESULTS
A total of 2353 studies were identified, from which 59 articles met the eligibility criteria. Anti-Ro52/SSA positivity was associated with ILD in all autoimmune disease subgroups: IIM (OR=3.08; 95% CI: 2.18 to 4.35; p value<0.001; I=49%), systemic lupus (OR=2.43; 95% CI: 1.02 to 5.79; p=0.046; I=71%), Sjogren (OR=1.77; 95% CI: 1.09 to 2.87; p=0.021; I=73%), systemic sclerosis (OR=1.71; 95% CI: 1.04 to 2.83; p=0.036; I=43%), mixed connective tissue disease (OR=3.34; 95% CI: 1.82 to 6.13; p<0.001; I=0%). Additionally, anti-Ro52-positive myopathy patients were more likely to have simultaneous RP-ILD (OR=2.69; 95% CI:1.50 to 4.83; p<0.001; I=71%).
CONCLUSION
Anti-Ro52/SSA positivity is associated with a higher frequency of ILD diagnosis in various autoimmune diseases. Anti-Ro52/SSA is also linked with a more severe lung involvement (RP-ILD). Future studies can investigate the benefits of screening for anti-Ro52 and its association with ILD development.
PROSPERO REGISTRATION NUMBER
CRD42022381447.
Topics: Humans; Autoantibodies; Cross-Sectional Studies; Autoimmune Diseases; Lung Diseases, Interstitial; Scleroderma, Systemic; Myositis
PubMed: 38030264
DOI: 10.1136/bmjresp-2023-002076 -
Frontiers in Pediatrics 2023Circumscribed or pseudomalignant myositis ossificans (MO) is a rare and benign condition characterized by heterotopic bone formation in soft tissues. The clinical...
INTRODUCTION
Circumscribed or pseudomalignant myositis ossificans (MO) is a rare and benign condition characterized by heterotopic bone formation in soft tissues. The clinical presentation of MO, imaging investigations, histological findings, and treatment strategies are unclear, especially in the pediatric population.
MATERIALS AND METHODS
A literature search was conducted in PubMed, Scopus, and Google Scholar electronic databases to identify original articles and reviews in English or French of traumatic and non-traumatic MO. Studies were selected by 2 independent reviewers following the PRISMA recommendation and descriptive data were extracted. We harvest in each case the sex, age at diagnosis, location, presence of initial trauma, pre-emptive diagnosis, modalities of imagery used, realized biopsy, treatment performed, and type of follow-up.
RESULTS
Sixty pediatric cases of MO were identified between 2002 and 2023. Twenty-three patients (38.3%) were diagnosed with idiopathic/pseudomalignant and 37 patients (61.7%) with circumscribed. The mean age at diagnosis was 9.5 years (range 0.2-17 years), with a male-to-female ratio of 1:1. The initial pre-emptive diagnosis was neoplasia in 13 patients (21.7%). The biopsy was percutaneous in 9 patients (15%) and incisional in 7 patients (11.7%). Histological analysis was achieved in 35 cases (57%). Surgical excision was the first line treatment in 46.7% of patients, and non-surgical in the remaining patients. The follow-up strategy was clinical in 16 patients (26.7%) or based on imaging investigation in 23 patients (38.3%).
DISCUSSION
Although MO in children is described as a rare pathology, identifying the benignity of the condition is essential to avoid unnecessary invasive treatment and to avoid delaying the treatment of a potentially life-threatening entity. It seems that there is no consensus established concerning the proper imaging for diagnosis. Clinicians should acknowledge that the absence of a triggering trauma tends to direct the investigation and the management toward a surgical attitude. Conservative management is key, however, surgical excision can be proposed on matured lesions on a case-by-case basis. The absence of recurrence is not excluded. Therefore, a close clinical follow-up is suggested for all cases. The true benefit of a radiological is questioned in a question known to be self-resolving.
PubMed: 38161430
DOI: 10.3389/fped.2023.1295212 -
Vaccines Oct 2023A comprehensive, up-to-date systematic review (SR) of the new-onset rheumatic immune-mediated inflammatory diseases (R-IMIDs) following COVID-19 vaccinations is lacking.... (Review)
Review
A comprehensive, up-to-date systematic review (SR) of the new-onset rheumatic immune-mediated inflammatory diseases (R-IMIDs) following COVID-19 vaccinations is lacking. Therefore, we investigated the demographics, management, and prognosis of new R-IMIDs in adults following SARS-CoV-2 vaccinations. A systematic literature search of Medline, Embase, Google Scholar, LitCovid, and Cochrane was conducted. We included any English-language study that reported new-onset R-IMID in adults following the post-COVID-19 vaccination. A total of 271 cases were reported from 39 countries between January 2021 and May 2023. The mean age of patients was 56 (range 18-90), and most were females (170, 62.5%). Most (153, 56.5%) received the Pfizer BioNTech COVID-19 vaccine. Nearly 50% of patients developed R-IMID after the second dose of the vaccine. Vasculitis was the most prevalent clinical presentation (86, 31.7%), followed by connective tissue disease (66, 24.3%). The mean duration between the vaccine's 'trigger' dose and R-IMID was 11 days. Most (220, 81.2%) received corticosteroids; however, 42% (115) received DMARDs such as methotrexate, cyclophosphamide, tocilizumab, anakinra, IV immunoglobulins, plasma exchange, or rituximab. Complete remission was achieved in 75 patients (27.7%), and 137 (50.6%) improved following the treatment. Two patients died due to myositis. This SR highlights that SARS-CoV-2 vaccines may trigger R-IMID; however, further epidemiology studies are required.
PubMed: 37896974
DOI: 10.3390/vaccines11101571 -
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 -
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 -
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