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European Journal of Internal Medicine Jun 2021To assess the efficacy and safety of adjuvant therapies in newly diagnosed or relapsing giant cell arteritis (GCA) in terms of relapse rate at week 52 (primary outcome)... (Meta-Analysis)
Meta-Analysis
OBJECTIVES
To assess the efficacy and safety of adjuvant therapies in newly diagnosed or relapsing giant cell arteritis (GCA) in terms of relapse rate at week 52 (primary outcome) and to assess the impact of GC tapering regimen on adjuvant effectiveness.
METHODS
For this systematic review and meta-analysis, we searched PubMed, EMBASE, CENTRAL, trial registries, from inception to November 2020. We included all randomized controlled trials (RCTs) and controlled prospective studies evaluating adjuvant treatments in GCA, without date or language restriction. Two reviewers independently selected studies, extracted data and assessed risk of bias. Quality of evidence was summarised with GRADE.
RESULTS
Of the 680 records identified, 16 studies were included (1,068 participants) evaluating various adjuvant therapies compared to GC only. No study compared adjuvants with each other. Risk of bias was high in 5/7 trials evaluating our primary outcome. Risk of relapse at week 52 was reduced for only the anti-IL6 and IL6-receptor drug class versus the control (RR=0.45, 95%CI 0.30-0.66, I2=38%), particularly tocilizumab (RR=0.38, 95%CI 0.23-0.63, I2=42%) with a moderate quality of evidence. We found no significant interaction according to GC tapering regimen. Our meta-analysis did not show a significant benefit for methotrexate. Except for dapsone, ciclosporine and hydroxychloroquine, other adjuvants did not seem to show increased risk of adverse events.
CONCLUSIONS
Tocilizumab seems to reduce the relapse rate in GCA at week 52 but the quality of evidence was moderate. No other molecule has shown efficacy. No significant interaction on relapse rate by GC tapering regimen was found.
STUDY REGISTRATION
PROSPERO CRD42020172011.
Topics: Drug Therapy, Combination; Giant Cell Arteritis; Glucocorticoids; Humans; Methotrexate; Steroids
PubMed: 33879385
DOI: 10.1016/j.ejim.2021.03.040 -
Foods (Basel, Switzerland) Mar 2021This study aimed to perform a systematic review on gluten-free bread formulations using specific volumes as a quality indicator. In this systematic review, we identified... (Review)
Review
This study aimed to perform a systematic review on gluten-free bread formulations using specific volumes as a quality indicator. In this systematic review, we identified 259 studies that met inclusion criteria. From these studies, 43 met the requirements of having gluten-free bread with a specific volume greater than or equal to 3.5 cm/g. Other parameters such as the texture profile, color (crumb and crust), and sensory analysis examined in these studies were presented. The formulations that best compensated the lack of the gluten-network were based on the combination of rice flour, rice flour with low amylose content, maize flour, rice starch, corn starch, potato starch, starch with proteins and added with transglutaminase (TGase), and hydrocolloids like hydroxypropylmethylcellulose (HPMC). Of the 43 studies, three did not present risk of bias, and the only parameter evaluated in common in the studies was the specific volume. However, it is necessary to jointly analyze other parameters that contribute to the quality, such as texture profile, external and internal characteristics, acceptability, and useful life of the bread, especially since it is a product obtained through raw materials and unconventional ingredients.
PubMed: 33805719
DOI: 10.3390/foods10030614 -
Multiple Sclerosis and Related Disorders May 2021Familial Mediterranean fever (FMF) is the most frequent monogenic autoinflammatory disorder; and leads to the uncontrolled production of interleukin (IL)-1β. Multiple...
INTRODUCTION
Familial Mediterranean fever (FMF) is the most frequent monogenic autoinflammatory disorder; and leads to the uncontrolled production of interleukin (IL)-1β. Multiple sclerosis (MS) is an inflammatory disease of the central nervous system; and its development seems to be partly correlated with IL-1β levels. It is hypothesized that FMF could be associated with MS. We aim to describe the features of patients displaying both diseases and to investigate the MEFV mutation rate in MS patients.
METHODS
Patients with definite MS were retrieved from the cohort of FMF patients in the Reference Center for Rare Auto-inflammatory Diseases and Amyloidosis (CEREMAIA). We also performed a systematic literature review of articles from PubMed that were published from 1990 to 2020.
RESULTS
Twenty-four patients were included in the case series: five patients (1.3%) from our cohort of 364 and 19 patients from the literature. The sex ratio was 2:1. The mean age at diagnosis of FMF was 19 years old; and that for MS was 29 years old. Seven studies investigating the MEFV mutation rate in MS patients were included. Three studies found a higher mutation rate in MS patients than in the control group.
CONCLUSION
FMF and MS features were comparable to those of patients with unrelated diseases; and MEFV mutation carriage was not positively correlated with MS. However; MS prevalence in FMF patients was higher than was expected in a healthy population. To a lesser extent; FMF prevalence in MS patients was higher than expected in a healthy population and the difference might not be significant. These data suggest that FMF could be associated with MS; and further studies are needed to investigate a potential causal association.
Topics: Adult; Cohort Studies; Familial Mediterranean Fever; Humans; Multiple Sclerosis; Mutation; Pyrin; Young Adult
PubMed: 33609923
DOI: 10.1016/j.msard.2021.102834 -
The Journal of Allergy and Clinical... Feb 2021Primary immune deficiencies (PIDs) are a heterogeneous group of disorders resulting from defects in immune system. They lead to increased susceptibility to infections...
BACKGROUND
Primary immune deficiencies (PIDs) are a heterogeneous group of disorders resulting from defects in immune system. They lead to increased susceptibility to infections and immune dysregulation. The resulting chronic inflammation can induce long-term complications, including AA amyloidosis (AAA).
OBJECTIVES
To present the French cases of PID-related AAA and perform a systematic literature review to determine its main features and predisposing factors.
METHODS
A systematic literature review was performed by searching MEDLINE up until 2019. New French cases were identified with the help of the Reference Center for Auto-Inflammatory Diseases and AA Amyloidosis and the Reference Center for Hereditary Immune Deficiencies.
RESULTS
Forty patients were identified including 2 new French cases. PIDs were varied: immunoglobulin deficits (n = 30), chronic granulomatous disease (n = 3), hyper-IgM syndrome (n = 3), hereditary complete C4 deficiency (n = 1), leucocyte adhesion deficiency type 1 (n = 1), hyper-IgE syndrome (n = 1), and Chediak-Higashi syndrome (n = 1). The mean age at PID diagnosis was 22.2 ± 16.02 years. Renal involvement was the most common manifestation of AAA (80%). Infections were extremely heterogeneous; bacterial infection with pulmonary involvement was the most frequent. Bronchiectasis was particularly common (52.5%). The delay between the first symptoms of PID and AAA diagnosis was 16.18 ± 7 years. Thirteen concomitant diagnoses were made. Twenty patients died during follow-up.
CONCLUSION
AAA is a rare life-threatening complication of PID, especially in cases of long diagnostic and therapeutic delays. Bronchiectasis should be considered as a warning sign of chronic inflammation and increased risk of AAA.
Topics: Amyloidosis; Bronchiectasis; Humans; Immunoglobulins; Immunologic Deficiency Syndromes; Primary Immunodeficiency Diseases
PubMed: 33007500
DOI: 10.1016/j.jaip.2020.09.023 -
Seminars in Arthritis and Rheumatism Feb 2020Adult onset Still's disease (AOSD) is an inflammatory disorder characterized by high spiking fever, evanescent rash, polyarthritis, and many other systemic...
OBJECTIVE
Adult onset Still's disease (AOSD) is an inflammatory disorder characterized by high spiking fever, evanescent rash, polyarthritis, and many other systemic manifestations. Recurrent or persistent disease can lead to AA amyloidosis (AAA). Our objectives were to present 3 French cases and perform a systematic review of the literature, in order to determine the prevalence, characteristics, predisposing factors, and therapeutic response of AOSD-related AAA.
METHODS
A systematic literature review was performed by searching MEDLINE from 1971 to 2018. Two independent investigators selected reports of AAA complicating AOSD. New French cases were identified with the help of the Reference Center for rare Auto-Inflammatory Diseases and Amyloidosis (CEREMAIA). Patients with juvenile idiopathic arthritis were excluded.
RESULTS
The prevalence of AAA in AOSD was 0.88% (95%CI [0.49-1.28]) based on 45 articles. In addition to 3 new cases from the CEREMAIA, 16 patients were assessed for clinical presentation, risk factors, and therapeutic response of AOSD-related AAA. Mean age at AOSD onset was 29.6 ± 12.6 years, with a mean delay before AAA diagnosis of 16.75±5.8 years. Renal involvement was the most common manifestation of AAA. The majority of patients presented active AOSD at AAA diagnosis. Various treatments of AOSD-related AAA were attempted including corticosteroids and biotherapies.
CONCLUSION
AAA is a rare and severe complication that may occur during the course of uncontrolled active AOSD. It could be prevented by early diagnosis and better control of AOSD, with more frequent use of biotherapies.
Topics: Adolescent; Adrenal Cortex Hormones; Adult; Amyloidosis; Humans; Still's Disease, Adult-Onset; Young Adult
PubMed: 31488308
DOI: 10.1016/j.semarthrit.2019.08.005 -
Frontiers in Immunology 2019Certain types of vasculitis occur more frequently and present differently in patients with familial Mediterranean fever (FMF). We assessed the characteristics of... (Meta-Analysis)
Meta-Analysis
Certain types of vasculitis occur more frequently and present differently in patients with familial Mediterranean fever (FMF). We assessed the characteristics of patients with FMF and systemic vasculitis through a systematic review of the literature. Medline was searched by two independent investigators until December 2017. We screened 310 articles and selected 58 of them (IgA vasculitis = 12, polyarteritis nodosa (PAN) = 25, Behçet's disease (BD) = 7, other vasculitis = 14). Clinical case reports were available for 167 patients (IgA vasculitis = 46, PAN = 61, BD = 46, other vasculitis = 14), and unavailable for 45 patients (IgA vasculitis = 38, PAN = 7). IgA vasculitis was the most common vasculitis in FMF patients with a prevalence of 2.7-7%, followed by PAN with a prevalence of 0.9-1.4%. Characteristics of FMF did not differ between patients with and without vasculitis. Patients with FMF and IgA vasculitis displayed more intussusception (8.7%) and possibly less IgA deposits on histological analysis than patients with IgA vasculitis alone. Patients with FMF and PAN had a younger age at vasculitis onset (mean age = 17.9 years), as well as more perirenal hematomas (49%) and CNS involvement (31%) than patients with PAN alone. Glomerular involvement was noted in 33% of patients diagnosed with PAN, suggesting an alternative diagnosis. Sequencing of the gene confirmed the presence of two pathogenic variants in 73% of FMF patients with IgA vasculitis or PAN. The majority of patients with BD were from one case series, and presented more skin, gastrointestinal, and CNS involvement than patients with isolated BD. In conclusion, FMF, particularly when supported by two pathogenic mutations, could predispose to IgA vasculitis, or a PAN-like vasculitis with more perirenal bleeding and CNS involvement.
Topics: Age of Onset; Disease Management; Disease Susceptibility; Familial Mediterranean Fever; Humans; Phenotype; Prevalence; Sex Factors; Vasculitis
PubMed: 31031761
DOI: 10.3389/fimmu.2019.00763 -
La Revue de Medecine Interne Apr 2018The auto-inflammatory diseases linked to NLRC4 mutations are recently described entities. Transmission is autosomal dominant in 80 % of cases; cases of somatic... (Review)
Review
The auto-inflammatory diseases linked to NLRC4 mutations are recently described entities. Transmission is autosomal dominant in 80 % of cases; cases of somatic mutation have already been reported. The disease may display two very different clinical phenotypes: the phenotype 1 (30 %), severe, is dominated by a multisystemic inflammation starting in the first year of life with symptoms of chronic inflammatory bowel disease (IBD), macrophagic actication syndrome (MAS), or even a presentation suggesting a cryopyrinopathy in its CINCA form; the mortality of this phenotype is high (25 %). The phenotype 2 (70 %), mild, usually starts after the age of 3 and is characterized by cold urticaria, arthralgia, ocular features and fever in 50 % of cases without visceral failure. Anti-interleukin-1 inhibitors are effective in most cases (83 %). Interleukin-18 (IL-18) levels are very high in both clinical forms. Interleukin-18 inhibitors and anti-interferon-gamma inhibitors were remarkably effective in two very severe phenotype 1 patients. Thus, NLRC4 mutations can induce various clinical manifestations with two distinct phenotypes. Although still rare, because very recently described, this group of diseases could be evoked by an internist in front of cold familial urticarial; probably more and more cases will be diagnosed thanks to the major progresses of genetic diagnostic tools such as next generation sequencing.
Topics: CARD Signaling Adaptor Proteins; Calcium-Binding Proteins; Female; Genetic Predisposition to Disease; Hereditary Autoinflammatory Diseases; Humans; Inflammation; Male; Mutation; Phenotype
PubMed: 29496273
DOI: 10.1016/j.revmed.2018.02.003 -
The British Journal of Nutrition Oct 2015Rice is an important staple food for more than half of the world's population. Especially in Asian countries, rice is a major contributor to dietary glycaemic load (GL).... (Review)
Review
Rice is an important staple food for more than half of the world's population. Especially in Asian countries, rice is a major contributor to dietary glycaemic load (GL). Sustained consumption of higher-GL diets has been implicated in the development of chronic diseases such as type 2 diabetes mellitus. Given that a reduction in postprandial glycaemic and insulinaemic responses is generally seen as a beneficial dietary change, it is useful to determine the variation in the range of postprandial glucose (PPG) and insulin (PPI) responses to rice and the primary intrinsic and processing factors known to affect such responses. Therefore, we identified relevant original research articles on glycaemic response to rice through a systematic search of the literature in Scopus, Medline and SciFinder databases up to July 2014. Based on a glucose reference value of 100, the observed glycaemic index values for rice varieties ranged from 48 to 93, while the insulinaemic index ranged from 39 to 95. There are three main factors that appear to explain most of the variation in glycaemic and insulinaemic responses to rice: (1) inherent starch characteristics (amylose:amylopectin ratio and rice cultivar); (2) post-harvest processing (particularly parboiling); (3) consumer processing (cooking, storage and reheating). The milling process shows a clear effect when compared at identical cooking times, with brown rice always producing a lower PPG and PPI response than white rice. However, at longer cooking times normally used for the preparation of brown rice, smaller and inconsistent differences are observed between brown and white rice.
Topics: Amylopectin; Amylose; Blood Glucose; Chemical Phenomena; Cooking; Food Handling; Glycemic Index; Glycemic Load; Humans; Insulin; Oryza; Postprandial Period; Randomized Controlled Trials as Topic; Starch; Whole Grains
PubMed: 26310311
DOI: 10.1017/S0007114515001841 -
Annals of the Rheumatic Diseases Apr 2015Familial Mediterranean fever (FMF) is a disease of early onset which can lead to significant morbidity. In 2012, Single Hub and Access point for pediatric Rheumatology... (Review)
Review
Familial Mediterranean fever (FMF) is a disease of early onset which can lead to significant morbidity. In 2012, Single Hub and Access point for pediatric Rheumatology in Europe (SHARE) was launched with the aim of optimising and disseminating diagnostic and management regimens for children and young adults with rheumatic diseases. The objective was to establish recommendations for FMF focusing on provision of diagnostic tools for inexperienced clinicians particularly regarding interpretation of MEFV mutations. Evidence-based recommendations were developed using the European League against Rheumatism standard operating procedure. An expert committee of paediatric rheumatologists defined search terms for the systematic literature review. Two independent experts scored articles for validity and level of evidence. Recommendations derived from the literature were evaluated by an online survey and statements with less than 80% agreement were reformulated. Subsequently, all recommendations were discussed at a consensus meeting using the nominal group technique and were accepted if more than 80% agreement was reached. The literature search yielded 3386 articles, of which 25 were considered relevant and scored for validity and level of evidence. In total, 17 articles were scored valid and used to formulate the recommendations. Eight recommendations were accepted with 100% agreement after the consensus meeting. Topics covered were clinical versus genetic diagnosis of FMF, genotype-phenotype correlation, genotype-age at onset correlation, silent carriers and risk of amyloid A (AA) amyloidosis, and role of the specialist in FMF diagnosis. The SHARE initiative provides recommendations for diagnosing FMF aimed at facilitating improved and uniform care throughout Europe.
Topics: Child; Cytoskeletal Proteins; Evidence-Based Medicine; Familial Mediterranean Fever; Humans; Practice Guidelines as Topic; Pyrin; Young Adult
PubMed: 25628446
DOI: 10.1136/annrheumdis-2014-206844