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Annals of the Rheumatic Diseases Jan 2024Since the publication of the EULAR recommendations for the management of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) in 2016, several...
BACKGROUND
Since the publication of the EULAR recommendations for the management of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) in 2016, several randomised clinical trials have been published that have the potential to change clinical care and support the need for an update.
METHODS
Using EULAR standardised operating procedures, the EULAR task force undertook a systematic literature review and sought opinion from 20 experts from 16 countries. We modified existing recommendations and created new recommendations.
RESULTS
Four overarching principles and 17 recommendations were formulated. We recommend biopsies and ANCA testing to assist in establishing a diagnosis of AAV. For remission induction in life-threatening or organ-threatening AAV, we recommend a combination of high-dose glucocorticoids (GCs) in combination with either rituximab or cyclophosphamide. We recommend tapering of the GC dose to a target of 5 mg prednisolone equivalent/day within 4-5 months. Avacopan may be considered as part of a strategy to reduce exposure to GC in granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA). Plasma exchange may be considered in patients with rapidly progressive glomerulonephritis. For remission maintenance of GPA/MPA, we recommend rituximab. In patients with relapsing or refractory eosinophilic GPA, we recommend the use of mepolizumab. Azathioprine and methotrexate are alternatives to biologics for remission maintenance in AAV.
CONCLUSIONS
In the light of recent advancements, these recommendations provide updated guidance on AAV management. As substantial data gaps still exist, informed decision-making between physicians and patients remains of key relevance.
Topics: Humans; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis; Antibodies, Antineutrophil Cytoplasmic; Azathioprine; Cyclophosphamide; Granulomatosis with Polyangiitis; Microscopic Polyangiitis; Remission Induction; Rituximab; Practice Guidelines as Topic
PubMed: 36927642
DOI: 10.1136/ard-2022-223764 -
Clinical Gastroenterology and... Jun 2018Celiac disease is a major public health problem worldwide. Although initially it was reported from countries with predominant Caucasian populations, it now has been... (Meta-Analysis)
Meta-Analysis
BACKGROUND & AIMS
Celiac disease is a major public health problem worldwide. Although initially it was reported from countries with predominant Caucasian populations, it now has been reported from other parts of the world. The exact global prevalence of celiac disease is not known. We conducted a systematic review and meta-analysis to estimate the global prevalence of celiac disease.
METHODS
We searched Medline, PubMed, and EMBASE for the keywords celiac disease, celiac, celiac disease, tissue transglutaminase antibody, anti-endomysium antibody, endomysial antibody, and prevalence for studies published from January 1991 through March 2016. Each article was cross-referenced with the words Asia, Europe, Africa, South America, North America, and Australia. The diagnosis of celiac disease was based on European Society of Pediatric Gastroenterology, Hepatology, and Nutrition guidelines. Of 3843 articles, 96 articles were included in the final analysis.
RESULTS
The pooled global prevalence of celiac disease was 1.4% (95% confidence interval, 1.1%-1.7%) in 275,818 individuals, based on positive results from tests for anti-tissue transglutaminase and/or anti-endomysial antibodies (called seroprevalence). The pooled global prevalence of biopsy-confirmed celiac disease was 0.7% (95% confidence interval, 0.5%-0.9%) in 138,792 individuals. The prevalence values for celiac disease were 0.4% in South America, 0.5% in Africa and North America, 0.6% in Asia, and 0.8% in Europe and Oceania; the prevalence was higher in female vs male individuals (0.6% vs 0.4%; P < .001). The prevalence of celiac disease was significantly greater in children than adults (0.9% vs 0.5%; P < .001).
CONCLUSIONS
In a systematic review and meta-analysis, we found celiac disease to be reported worldwide. The prevalence of celiac disease based on serologic test results is 1.4% and based on biopsy results is 0.7%. The prevalence of celiac disease varies with sex, age, and location. There is a need for population-based prevalence studies in many countries.
Topics: Adolescent; Adult; Aged; Autoantibodies; Celiac Disease; Child; Child, Preschool; Female; Global Health; Humans; Infant; Intestines; Male; Middle Aged; Prevalence; Young Adult
PubMed: 29551598
DOI: 10.1016/j.cgh.2017.06.037 -
International Journal of Molecular... Nov 2022Guillain-Barré syndrome (GBS) is a rare immune-mediated acute polyradiculo-neuropathy that typically develops after a previous gastrointestinal or respiratory... (Review)
Review
Guillain-Barré syndrome (GBS) is a rare immune-mediated acute polyradiculo-neuropathy that typically develops after a previous gastrointestinal or respiratory infection. This narrative overview aims to summarise and discuss current knowledge and previous evidence regarding triggers and pathophysiology of GBS. A systematic search of the literature was carried out using suitable search terms. The most common subtypes of GBS are acute inflammatory demyelinating polyneuropathy (AIDP) and acute motor axonal neuropathy (AMAN). The most common triggers of GBS, in three quarters of cases, are previous infections. The most common infectious agents that cause GBS include , , and cytomegalovirus. is responsible for about a third of GBS cases. GBS due to is usually more severe than that due to other causes. Clinical presentation of GBS is highly dependent on the structure of pathogenic lipo-oligosaccharides (LOS) that trigger the innate immune system via Toll-like-receptor (TLR)-4 signalling. AIDP is due to demyelination, whereas in AMAN, structures of the axolemma are affected in the nodal or inter-nodal space. In conclusion, GBS is a neuro-immunological disorder caused by autoantibodies against components of the myelin sheath or axolemma. Molecular mimicry between surface structures of pathogens and components of myelin or the axon is one scenario that may explain the pathophysiology of GBS.
Topics: Humans; Amantadine; Autoantibodies; Axons; Campylobacter jejuni; Guillain-Barre Syndrome
PubMed: 36430700
DOI: 10.3390/ijms232214222 -
Archives of Gynecology and Obstetrics Jul 2023Systemic lupus erythematosus (SLE)-a most common disorder in women of reproductive age-has been described to be associated with adverse pregnancy outcomes. Despite the... (Review)
Review
Systemic lupus erythematosus (SLE)-a most common disorder in women of reproductive age-has been described to be associated with adverse pregnancy outcomes. Despite the increased health risks for the mother (preeclampsia, lupus flare, arterial hypertension, gestational diabetes mellitus and thrombotic risk when antiphospholipid antibodies are present) and fetus (miscarriage, stillbirth, premature birth, intrauterine growth restriction and neonatal lupus), the majority of patients can deliver healthy neonates. With appropriate management by a multidisciplinary team, composing rheumatologists, obstetricians and neonatologists, women with SLE can achieve better pregnancy outcomes by monitoring associated predictive indicators, raising major concern for severe complications and somewhat early delivery if necessary. In this review, we summarize the latest advances in secondary infertility and pregnancy-related risk perception for lupus patients, with an emphasis on the safety of biological agents (mainly belimumab and rituximab) and traditional therapeutic regimens.
Topics: Pregnancy; Infant, Newborn; Humans; Female; Lupus Erythematosus, Systemic; Symptom Flare Up; Pregnancy Outcome; Pregnancy Complications; Antibodies, Antiphospholipid
PubMed: 35913558
DOI: 10.1007/s00404-022-06718-7 -
Seminars in Arthritis and Rheumatism Apr 2022Anti-melanoma differentiation-associated gene 5 (MDA5) antibody-positive clinically amyopathic dermatomyositis (CADM) is frequently associated with rapidly progressive... (Review)
Review
INTRODUCTION
Anti-melanoma differentiation-associated gene 5 (MDA5) antibody-positive clinically amyopathic dermatomyositis (CADM) is frequently associated with rapidly progressive interstitial lung disease (RP-ILD) and high mortality rates. There is a lack of data on management of this often fatal condition. The aim of this systematic review was to evaluate current evidence that assesses the available management options and discuss the associated management challenges.
MATERIAL AND METHODS
This systematic review was conducted according to PRISMA guidelines. Online databases were searched from inception to April of 2021 using the search terms: "dermatomyositis" OR "amyopathic dermatomyositis" OR "clinically amyopathic dermatomyositis" AND "MDA-5″ OR "melanoma differentiation-associated gene-5″ OR "CADM-140″ AND "management" OR "treatment" OR "therapy" OR "therapeutics". Articles assessing the use of pharmacologic agents on 10 or more patients with MDA5-antibody positive CADM associated with ILD were included. Narrative or systematic reviews and meta-analyses were not eligible for inclusion.
RESULTS
A total of 15 eligible studies and 399 unique patients were selected. We identified only one open-label randomized controlled trial (RCT) that examined the management of anti-MDA5 antibody CADM/DM-ILD. Further, 3 cohort studies with prospective arms matched against historical controls, 10 retrospective cohort studies, and 1 retrospective case series were included. A combined therapeutic regimen of high-dose systemic glucocorticoids and other immunosuppressive agents such as calcineurin inhibitors and/or cyclophosphamide, administered early, appears to give the highest rates of survival in those with RP-ILD, while additional therapies such as plasma exchange can be added for refractory disease. Further, tofacitinib and rituximab might have a place in the therapeutic armamentarium of this challenging to treat condition. Early detection and treatment are of extreme importance, given the risk for rapid decline and high mortality in this subset of patients.
CONCLUSION
There are limited RCTs evaluating the treatment of ILD associated with MDA5-antibody positive CADM. Initiating a combined immunosuppressive therapeutic regimen early in the disease course improves overall morbidity and mortality. RCTs and larger prospective studies are needed to provide high-quality evidence to inform future treatment guidelines.
Topics: Autoantibodies; Dermatomyositis; Disease Progression; Humans; Immunosuppressive Agents; Lung Diseases, Interstitial
PubMed: 35134633
DOI: 10.1016/j.semarthrit.2022.151959 -
Clinical and Experimental Rheumatology Feb 2022Anti-synthetase syndrome (ASSD) is a heterogeneous autoimmune disease characterised by multi-system involvement with a wide variety of manifestations. Validated... (Meta-Analysis)
Meta-Analysis
OBJECTIVES
Anti-synthetase syndrome (ASSD) is a heterogeneous autoimmune disease characterised by multi-system involvement with a wide variety of manifestations. Validated classification criteria are necessary to improve recognition and prevent misclassification, especially given the lack of reliable and standardised autoantibody testing. We systematically reviewed the literature to analyse proposed ASSD criteria, characteristics, and diagnostic performance.
METHODS
We searched PubMed and Embase databases (01/01/1984 to 06/11/2018) and the ACR and EULAR meeting abstracts (2017-2018). Sensitivities, specificities, positive, negative likelihood ratios and risk of bias were calculated for ASSD criteria and key variables reported in the literature. We performed meta-analysis when appropriate.
RESULTS
We retrieved 4,358 studies. We found 85 proposed ASSD criteria from a total of 82 studies. All but one study included anti-synthetase autoantibody (ARS) positivity in the ASSD criteria. Most studies required only one ASSD feature plus anti-ARS to define ASSD (n=64, 78%), whereas 16 studies required more than one ASSD variable plus anti-ARS. The only criteria not including anti-ARS positivity required 5 ASSD clinical features. We found limited data and wide variability in the diagnostic performance of each variable and definition proposed in the literature. Given these limitations we only meta-analysed the performance of individual muscle biopsy and clinical variables in diagnosing ASSD, which performed poorly.
CONCLUSIONS
The current ASSD criteria include a variety of serological, clinical, and histological features with wide variability amongst proposed definitions and the performance of these definitions has not been tested. This systematic literature review suggests the need for additional data and consensus-driven classification criteria for ASSD.
Topics: Autoantibodies; Humans; Ligases; Syndrome
PubMed: 35225224
DOI: 10.55563/clinexprheumatol/8xj0b9 -
Autoimmunity Reviews Oct 2021To identify and assess the magnitude of effect of pregnancy outcome predictors in women with antiphospholipid syndrome (APS) by means of systematic review and... (Meta-Analysis)
Meta-Analysis Review
OBJECTIVE
To identify and assess the magnitude of effect of pregnancy outcome predictors in women with antiphospholipid syndrome (APS) by means of systematic review and meta-analysis.
METHODS
PubMed and Embase were searched (13th June 2020) for studies reporting on pre-pregnancy risk factors of pregnancy outcomes in APS patients. Literature screening and data extraction were conducted by two reviewers independently, in a blinded standardized manner. Pooled univariate odds ratios (OR) were computed using a random effects model. Heterogeneity was assessed by I%.
RESULTS
The search yielded 3013 unique results; 27 records were included in this meta-analysis. Previous thrombosis was associated with a decreased live birth risk (OR 0.60, p < 0.01, I = 40%), increased neonatal mortality (OR 15.19, p < 0.01, I = 0%), an increased risk of antenatal or postpartum thrombosis (OR 6.26, p < 0.01, I = 0%) and an increased risk of delivering a small for gestational age neonate (SGA) (OR 2.60, p = 0.01, I = 0%). Patients with an APS laboratory category I (double or triple positivity) profile had a decreased live birth risk (OR 0.66, p < 0.01, I = 0%), an increased risk of SGA (OR 1.86, p = 0.01, I = 43%) and preterm birth (OR 1.35, p < 0.01, I = 49%). Triple positivity was associated with a decreased live birth risk (OR 0.33, p < 0.01, I = 68%), an increased risk of preeclampsia (OR 2.43, p = 0.02, I = 35%) and SGA (OR 2.47, p = 0.04, I = 61%). Patients with lupus anticoagulant positivity had an increased risk of preeclampsia (OR 2.10, p = 0.02, I = 48%), SGA (OR 1.78, p < 0.01, I = 0%) and preterm birth (OR 3.56, p = 0.01, I = 48%). Risk of bias assessment suggested considerable bias on study participation and statistical methods.
CONCLUSIONS
The results of this meta-analysis identified previous thrombosis, laboratory category I, triple positivity and lupus anticoagulant positivity as the most important predictors of adverse pregnancy outcomes. This up-to-date knowledge, can be used in preconception counseling and tailoring of obstetric care.
Topics: Antiphospholipid Syndrome; Female; Humans; Infant, Newborn; Lupus Coagulation Inhibitor; Pre-Eclampsia; Pregnancy; Pregnancy Outcome; Premature Birth
PubMed: 34280554
DOI: 10.1016/j.autrev.2021.102901 -
Frontiers in Endocrinology 2023Hashimoto's thyroiditis (HT) is the most common autoimmune disease. HT may be associated with nonthyroidal autoimmune diseases, including celiac disease (CD) or other... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Hashimoto's thyroiditis (HT) is the most common autoimmune disease. HT may be associated with nonthyroidal autoimmune diseases, including celiac disease (CD) or other gluten-related conditions (GRC). In the last years, interest about gluten-free diet (GFD) has increased for its supposed extraintestinal anti-inflammatory effect; thus, many patients with HT initiate GFD on their own.
OBJECTIVES
The aim of this meta-analysis is to examine all available data in literature about the effect of a GFD on TgAb, TPOAb, TSH, FT4, and FT3 levels in patients with HT and no symptoms or histology of CD.
METHODS
The study was conducted according to MOOSE (Meta-analysis Of Observational Studies in Epidemiology). The search was performed on databases PubMed and Scopus. The last search was performed on 7 February 2023. Quality assessment was performed. Meta-analyses were performed using the random-effect model. Hedges' was used to measure the effect size (ES). Statistical analyses were performed using StataSE 17.
RESULTS
The online search retrieved 409 articles, and 4 studies with a total of 87 patients were finally included for quantitative analysis. The risk of bias was generally low. The mean period of GFD was almost 6 months. The meta-analyses showed reduction in antibody levels with ES: -0.39 for TgAb (95% CI: -0.81 to +0.02; = 0.06; ² = 46.98%) and -0.40 for TPOAb (95% CI: -0.82 to +0.03; = 0.07; ² = 47.58%). TSH showed a reduction with ES: -0.35 (95% CI: -0.64 to -0.05; = 0.02; ² = 0%) and FT4 showed an increase with ES: +0.35% (95% CI: 0.06 to 0.64; = 0.02; ² = 0%). FT3 did not display variations (ES: 0.05; 95% CI: -0.38 to +0.48; = 0.82; ² = 51%). The heterogeneity of TgAb, TPOAb, and FT3 data was solved performing sub-analyses between patients with or without GRC (TgAb = 0.02; TPOAb = 0.02; FT3 = 0.04) and only for FT3, performing a sub-analysis between patients taking and not taking LT4 ( = 0.03).
CONCLUSION
This is the first meta-analysis investigating the effect of GFD on HT. Our results seem to indicate a positive effect of the gluten deprivation on thyroid function and its inflammation, particularly in patients with HT and GRC. However, current lines of evidence are not yet sufficient to recommend this dietary approach to all patients with a diagnosis of HT.
Topics: Humans; Thyroiditis, Autoimmune; Diet, Gluten-Free; Celiac Disease; Autoantibodies; Hashimoto Disease; Thyrotropin
PubMed: 37554764
DOI: 10.3389/fendo.2023.1200372 -
Arthritis Care & Research Aug 2021To provide evidence-based recommendations and expert guidance for the management of antineutrophil cytoplasmic antibody-associated vasculitis (AAV), including...
OBJECTIVE
To provide evidence-based recommendations and expert guidance for the management of antineutrophil cytoplasmic antibody-associated vasculitis (AAV), including granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA).
METHODS
Clinical questions regarding the treatment and management of AAV were developed in the population, intervention, comparator, and outcome (PICO) format (47 for GPA/MPA, 34 for EGPA). Systematic literature reviews were conducted for each PICO question. The Grading of Recommendations Assessment, Development and Evaluation methodology was used to assess the quality of evidence and formulate recommendations. Each recommendation required ≥70% consensus among the Voting Panel.
RESULTS
We present 26 recommendations and 5 ungraded position statements for GPA/MPA, and 15 recommendations and 5 ungraded position statements for EGPA. This guideline provides recommendations for remission induction and maintenance therapy as well as adjunctive treatment strategies in GPA, MPA, and EGPA. These recommendations include the use of rituximab for remission induction and maintenance in severe GPA and MPA and the use of mepolizumab in nonsevere EGPA. All recommendations are conditional due in part to the lack of multiple randomized controlled trials and/or low-quality evidence supporting the recommendations.
CONCLUSION
This guideline presents the first recommendations endorsed by the American College of Rheumatology and the Vasculitis Foundation for the management of AAV and provides guidance to health care professionals on how to treat these diseases.
Topics: Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis; Antibodies, Antineutrophil Cytoplasmic; Biomarkers; Clinical Decision-Making; Consensus; Decision Support Techniques; Evidence-Based Medicine; Humans; Immunosuppressive Agents; Rheumatology; Severity of Illness Index; Treatment Outcome
PubMed: 34235880
DOI: 10.1002/acr.24634 -
Autoimmunity Reviews Apr 2022Many systemic immune-mediated diseases (SIDs) may involve the heart and present as myocarditis with different histopathological pictures, i.e. lymphocytic, eosinophilic,... (Review)
Review
Many systemic immune-mediated diseases (SIDs) may involve the heart and present as myocarditis with different histopathological pictures, i.e. lymphocytic, eosinophilic, granulomatous, and clinical features, ranging from a completely asymptomatic patient to life-threatening cardiogenic shock or arrhythmias. Myocarditis can be part of some SIDs, such as sarcoidosis, systemic lupus erythematosus, systemic sclerosis, antiphospholipid syndrome, dermato-polymyositis, eosinophilic granulomatosis with polyangiitis and other vasculitis syndromes, but also of some organ-based immune-mediated diseases with systemic expression, such as chronic inflammatory bowel diseases. The aim of this review is to describe the prevalence, main clinical characteristics and prognosis of myocarditis associated with SIDs.
Topics: Churg-Strauss Syndrome; Granulomatosis with Polyangiitis; Humans; Myocarditis; Prevalence; Prognosis
PubMed: 34995763
DOI: 10.1016/j.autrev.2022.103037