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At follow-up, 13 patients (61.9%) met the classification criteria for systemic lupus erythematosus, 1 patient (4.8%) for mixed CTD, 5 patients (23.8%) for systemic sclerosis and 2 patients (9.5%) for Sjögren's syndrome. Patients evolving towards a new diagnosis had longer disease duration (15.2±9.7 years vs. 10±5.8 years; respectively, p<0.005), had a higher prevalence of anti-Ro/SSA antibodies (63.2% vs. 28.4%; respectively, p<0.05) and anti-RNP antibodies (21.1% vs. 7.4%; respectively, p<0.05). The statistical difference was also confirmed after the multivariate analysis.

Up to 45% of UCTD patients might develop novel clinical and/or laboratory features during the follow-up, leading to evolution into a definite CTD in 1 out 5 cases.

Up to 45% of UCTD patients might develop novel clinical and/or laboratory features during the follow-up, leading to evolution into a definite CTD in 1 out 5 cases.

Systemic autoinflammatory diseases (SAIDs) are caused by the malfunctioning of the innate immune system factors. Clinical heterogeneity and undefined pathobiology are common phenomena among SAIDs. In this study, we aimed to assess the involvement of microRNAs in regulating these complex diseases.

The expression pattern of different miRNAs was compared between SAID patients with high autoinflammatory disease activity index (AIDAI) score and with low AIDAI score, and their role in inflammation-related pathways was investigated. Differentially expressed miRNAs were determined using the Multi Experiment Viewer (MEV) and Transcriptome Analysis Console (TAC) analysis tools using miRNA microarray. Potential targets of miRNAs were enriched for inflammation-related genes and validated using qRT-PCR analysis.

Upon performing microarray analysis, 40 differentially expressed miRNAs were identified between mild familial Mediterranean fever (FMF) patients and severe SAID patients. Thereafter, 21 of 40 miRNAs were found to be potentially involved in inflammatory pathways, of which, 8 were further validated through qRT-PCR. The targets of these 8 miRNAs (miR-29b-3p, miR-29c-3p, miR-30e-3p, miR-130b-3p, miR-148a-3p, miR-186-5p, miR-197-3p, and miR-374b-5p) belonged to the inflammation-related genes and pathways.

This is the first study to identify miRNAs that might be associated with a more severe disease form of monogenic autoinflammatory diseases. All these miRNAs were associated with cytokine-mediated pathways and might be used for establishing diagnostic and therapeutic methods.

This is the first study to identify miRNAs that might be associated with a more severe disease form of monogenic autoinflammatory diseases. All these miRNAs were associated with cytokine-mediated pathways and might be used for establishing diagnostic and therapeutic methods.

This systematic review and meta-analysis was aimed to evaluate the efficacy and safety of tocilizumab (TCZ) in treating severe coronavirus disease 2019 (COVID-19).

The electronic search was made using PubMed, Scopus, CENTRAL, and Google scholar to identify the retrospective observational reports. The studies published from 01 January 2020 to 30th October 2020. Participants were hospitalised COVID-19 patients. Interventions included tocilizumab versus placebo/standard of care. The comparison will be between TCZ versus standard of care (SOC)/placebo. Inconsistency between the studies was evaluated with I2 and quality of the evidences were evaluated by Newcastle-Ottawa scale.

Based on the inclusion criteria there were 24 retrospective studies involving 5686 subjects were included. The outcomes of the meta-analysis have revealed that the TCZ has reduced mortality (M-H, RE-OR -0.11(-0.18--0.04) 95% CI, p=0.001, I2 =88%) and increased the incidences of super-infections (M-H, RE-OR 1.49(1.13-1.96) 95% CI, p=0.004, I2=47%). Ipatasertib Akt inhibitor However, there is no significant difference in ICU admissions rate (M-H, RE-OR -0.06(-0.23-0.12), I2=93%), need for mechanical ventilation (M-H, RE-OR of 0.00(-0.06-0.07), I=74%), LOS (IV -2.86(-0.91-3.38), I2=100%), LOS-ICU (IV -3.93(-12.35-4.48), I2=100%), and incidences of pulmonary thrombosis (MH, RE-OR 1.01 (0.45-2.26), I2=0%) compared to SOC/control.

Based on cumulative low-to-moderate certainty evidence shows that TCZ could reduce the risk of mortality in hospitalised patients. However, there is no statistically significant difference observed between the TCZ and SOC/control groups in other parameters.

Based on cumulative low-to-moderate certainty evidence shows that TCZ could reduce the risk of mortality in hospitalised patients. However, there is no statistically significant difference observed between the TCZ and SOC/control groups in other parameters.

The importance of citrullination in rheumatoid arthritis (RA) has been reported, but the degree to which individual citrullinated proteins affect the onset and progression of RA is still unclear. We aimed to identify citrullinated proteins that may play an important role in the onset and progression of RA using an individualised anti-citrullinated protein antibody (ACPA) evaluation system with citrullinated peptides as probes.

Serum samples from 50 normal donors and 51 RA patients were evaluated using a custom MagPlexTM bead array with 13 types of citrullinated peptide. The presence/absence of ACPAs that react to each citrullinated peptide in each subject was determined using the Z-score, which was calculated based on the fluorescence intensity distribution of a sample from a normal donor. Whether the fluorescence intensity was inhibited when free citrullinated peptides were added to a system was also evaluated.

Median fluorescence intensities obtained from beads coupled with the 13 types of citrullinated peptide were all significantly higher in RA patients versus normal donors. With a Z-score ≥2 as the cut-off value for the presence of ACPAs, ACPAs that recognised five types of citrullinated peptides derived from fibrinogen A, fillagrin, clusterin, and vimentin were widely detected in RA patients. In addition, inhibition experiments showed that citrullinated vimentin, clusterin, and enolase 1A peptides inhibited coupling of ACPAs to other citrullinated peptides.

ACPAs to many citrullinated proteins exhibited cross-reactivity to citrullinated clusterin and vimentin, suggesting the importance of citrullinated clusterin and vimentin in the early stages of RA pathogenesis.

ACPAs to many citrullinated proteins exhibited cross-reactivity to citrullinated clusterin and vimentin, suggesting the importance of citrullinated clusterin and vimentin in the early stages of RA pathogenesis.

To evaluate the similarities between LBAL (adalimumab biosimilar candidate) and the adalimumab reference product (ADL) in terms of efficacy and safety, including immunogenicity, in patients with active rheumatoid arthritis despite methotrexate treatment.

This phase III, multicentre, randomised, double-blind, parallel-group, 56-week study was conducted in Japan and Korea. During the first 24 weeks, patients subcutaneously received 40 mg of LBAL or ADL every two weeks (LBAL and ADL groups). During the subsequent 28 weeks, the LBAL group patients and half of the ADL group patients received LBAL (L-L and A-L arms). The remaining ADL group patients continued to receive ADL (A-A arm). The primary efficacy endpoint was the change from baseline in disease activity score 28-erythrocyte sedimentation rate (DAS28-ESR) at Week 24. American College of Rheumatology (ACR) response rates, adverse events (AEs), and anti-drug antibody (ADA) were also assessed.

In total, 383 patients were randomised. The least squares (LS) mean changes from baseline in DAS28-ESR at Week 24 were -2.45 and -2.53 in the LBAL (n=191) and ADL (n=190) groups, respectively. The 95% confidence interval (CI; -0.139, 0.304) of the difference (0.08) was within the pre-specified equivalence margin (-0.6, 0.6). Up to Week 52, the decreases in DAS28-ESR were maintained in all three arms. No notable differences in ACR20/50/70 were observed. The AE and ADA incidences were comparable between the arms.

LBAL was equivalent in efficacy and comparable in safety, including immunogenicity, to ADL. Switching from ADL to LBAL did not impact on efficacy and safety.

LBAL was equivalent in efficacy and comparable in safety, including immunogenicity, to ADL. Switching from ADL to LBAL did not impact on efficacy and safety.

To describe local adaptations of materials derived from evidence-based recommendations in a training programme in rheumatoid arthritis (RA).

The eRA (evolving the management of rheumatoid arthritis) programme generated shared decision-making practises and a checklist for managing comorbidity in RA, among others, at the international level. Unmet needs in RA management were first identified and prioritised. Then educational materials were designed and developed to address these gaps. These materials were evaluated in detailed and discussed in small regional groups by practicing rheumatologists. Voting, open discussions and recommendations were extracted from the meetings.

Thirty-five Spanish rheumatologists discussed a comorbidity checklist and a shared decision-making tool. The results of the local meetings were synthesised as (1) a judicious commitment to check agreed comorbidities, and (2) a list of barriers and facilitators for the implementation of shared decision making in the local settings. With be used as a guidance or example for implementation elsewhere.

Familial Mediterranean fever (FMF) is an autosomal-recessive, inflammatory disorder characterised by short, recurrent attacks of fever, accompanied by pain in the abdomen, chest, or joints and complications of amyloidosis. Recently, we observed a significant association between the serum amyloid A1 (SAA1) β/β genotype and a delayed disease onset in 386 M694V homozygous FMF patients. This follow-up study was conducted to additionally analyse MEFV genotypes other than M694V/M694V for a possible influence of the SAA1 genotype on the age of disease onset.

A total of 700 Armenian patients diagnosed with FMF based on the Tel-Hashomer criteria and carrying two MEFV mutant alleles were included in this study. Patients were divided into three MEFV genotypic subgroups M694V homozygotes (M694V/M694V), M694V compound heterozygotes (M694V/Other), and patients with genotypes excluding M694V (Other/Other). MEFV and SAA1 analyses were performed by a commercial reverse-hybridisation assay, and resulting genotypes were matched against the demographic and clinical characteristics of the patients.

Within the subgroup of M694/M694 homozygotes, SAA1 genotype β/β could be identified in 115 (34.43%) and 32 (61.54%) patients with an age of onset <20 and ≥20 years, respectively(p<0.001). However, no such relationship could be observed for MEFV genotypic subgroups M694V/Other (p=0.465) and Other/Other (p=0.697).

Our data suggest, that the influence of SAA1 genotypic variation on the age of disease onset restricts to FMF patients homozygous for MEFV mutation M694V.

Our data suggest, that the influence of SAA1 genotypic variation on the age of disease onset restricts to FMF patients homozygous for MEFV mutation M694V.

To describe the clinical profile, long-term follow-up and outcome of juvenile systemic scleroderma (JSSc) from a tertiary care referral hospital in North-West India.

A review of case records was performed and children with JSSc (disease onset <14 years of age) were analysed. Diagnosis was based on the Paediatric Rheumatology European Society/American College of Rheumatology/European League against Rheumatism provisional classification criteria for JSSc.

Forty patients (28 girls and 12 boys; FM ratio= 2.31) were diagnosed with JSSc (including 22 children with overlap) in the last 25 years. Mean age at symptom onset was 7.75±3.19 years with a mean delay in diagnosis of 2.275±2.09 years. Raynaud's phenomenon was seen in 26/40 (65%) patients at presentation. Lung involvement was noted in 40% patients. Methotrexate was the most commonly used therapy, followed by oral prednisolone. Patients without overlap had higher incidence of cutaneous ulcers as compared to patients with overlap (55% vs. 18%; p-value 0.