UNASSIGNED: The American College of Rheumatology (ACR) 1997, Systemic Lupus International Collaborating Clinics (SLICC) 2012, and European League Against Rheumatism (EULAR)/ACR 2019 SLE criteria are often used to classify patients with adult-onset and childhood-onset systemic lupus erythematosus (SLE) in clinical practice because there are currently no diagnostic criteria for SLE. However, there is scarce evidence regarding which criteria are best for diagnosing patients with adult-onset and childhood-onset SLE.
UNASSIGNED: We searched Medline and Scopus databases for English-language articles from inception through October 2021. Data were extracted from the included publications by two independent reviewers. We performed bivariate meta-analysis with a random-effects model to pool diagnostic parameters. Meta-regression and subgroup analyses were performed to explore heterogeneity sources. We used network meta-analysis to compare diagnosis performance among the three criteria and ranked them in descending order. Publication bias was assessed using Deeks\' funnel plot.
UNASSIGNED: We included 29 studies for systematic review and meta-analysis. Of these, 18 studies involved adult-onset SLE and 11 studies involved childhood-onset SLE. The pooled sensitivities of the three criteria for diagnosis of adult-onset SLE were comparable between SLICC 2012 and EULAR/ACR 2019 [95.86, 95% confidence interval (CI) 92.28-97.81 vs. 94.79, 95% CI 92.03-96.63]; pooled specificity was highest in ACR 1997 (92.24, 95% CI 87.06-95.46). In childhood-onset SLE, pooled sensitivity was highest in SLICC 2012 (93.76, 95% CI 89.45-96.39), and pooled specificity was highest in ACR 1997 (95.89, 95% CI 91.73-98.00). In network meta-analysis, the pooled diagnostic odds ratio ranked highest for EULAR/ACR 2019 (131.570, 95% CI 61.50-281.47) in adult-onset SLE and ranked highest for SLICC 2012 (191.07, 95% CI 76.06-480.01) in childhood-onset SLE. Deeks\' funnel plot showed no publication bias.
UNASSIGNED: We found that the diagnostic performance of the ACR 1997, SLICC 2012, and EULAR/ACR 2019 criteria differed between adult-onset and childhood-onset SLE. EULAR/ACR 2019 performed best for adult-onset SLE and SLICC 2012 was best for childhood-onset SLE in classifying patients with SLE.
UNASSIGNED: [www.ClinicalTrials.gov], identifier [CRD420 21281586].

Systemic sclerosis is a complex, often progressive, multisystem autoimmune disease. It is commonly categorized into limited cutaneous or diffuse cutaneous systemic sclerosis. There is near universal involvement of skin fibrosis and gastrointestinal dysfunction, but lung disease is not only common but also a most serious complication. Severe lung disease is the top cause of mortality, displacing scleroderma renal crisis as the leading cause of death. Whether there is limited cutaneous or diffuse cutaneous manifestations can be predictive of what type of lung disease that can present in the patient. Limited cutaneous systemic sclerosis patients tend to have pulmonary hypertension whereas diffuse cutaneous systemic sclerosis patients tend to have interstitial lung disease. There are more rare phenotypes associated with antibodies Th/To and U3RNP that can have both pulmonary hypertension and interstitial lung disease concomitantly. There are inherent challenges in the management for both pulmonary hypertension and interstitial lung disease but with the focus on early diagnosis for each of these lung complications, treatment may have a higher chance of efficacy.

Although the original purpose of the systemic lupus erythematosus (SLE) classification criteria was to distinguish SLE from other mimic diseases, and to facilitate sample selection in scientific research, they have become widely used as diagnostic criteria in clinical situations. It is not known yet if regarding classification criteria as diagnostic criteria, what problems might be encountered? This is the first study comparing the three sets of classification criteria for SLE, the 1997 American College of Rheumatology (ACR\'97), 2012 Systemic Lupus International Collaborating Clinics (SLICC\'12) and 2019 European League Against Rheumatism/American College of Rheumatology (EULAR/ACR\'19), for their ability to distinguish patients with SLE from patients with pure mucocutaneous manifestations (isolated cutaneous lupus erythematosus without internal disease, i-CLE) in the lupus disease spectrum. 1,865 patients with SLE and 232 patients with i-CLE were recruited from a multicenter study. We found that, due to low specificity, none of the three criteria are adept at distinguishing patients with SLE from patients with i-CLE. SLICC\'12 performed best among the original three criteria, but if a positive ANA was removed as an entry criterion, EULAR/ACR\'19 would performed better. A review of previous studies that compared the three sets of criteria was presented in this work.

BACKGROUND: Familial Mediterranean fever (FMF) is a genetic disease of childhood and adulthood which is relatively rare in Germany. It is characterized by recurrent febrile attacks, peritonitis, pleuritis and arthritis. The established treatment with colchicine is effective and well-tolerated by most patients; however, some patients do not adequately respond or do not tolerate this treatment. Biologics can be considered for some of these patients. The Society for Pediatric and Adolescent Rheumatology (GKJR) and the German Society for Rheumatology (DGRh) have agreed to develop joint recommendations for this specific clinical situation.
OBJECTIVE: Implementation of a systematic literature search (SLR) on the basis of the EULAR recommendations published in 2016 as the foundation for the development of evidence-based treatment recommendations for FMF patients with insufficient response or intolerance to colchicine.
METHODS: The SLR was performed using references from various databases as an update of the SLR carried out by EULAR up to 2014, whereby all articles must have been published between 1 January 2015 and 31 December 2017. The Rayyan abstract tool for the preselection and the classification of the Oxford Centre for Evidence Based Medicine 2009 were used for the preparation of the evidence tables.
RESULTS: The search yielded 360 hits and after duplicate matching 263. A total of 88 publications were included (34%) and 102 excluded (39%), a review of the full publication was necessary for a further 73 (28%) and 43 were discussed more intensively. Finally, 64 publications (24%) remained. A total of 4 case-control studies, 31 cohort studies, 8 case series, 7 controlled studies (including 5 abstracts), 10 reviews, 4 meta-analyses and systematic reviews were accepted.
CONCLUSIONS: The SLR was carried out in a scientifically accurate and transparent manner according to international standards. The SLR proved to be a good basis for a consensus on the 5 overarching principles and the 10 recommendations, so that the joint activity of the GKJR and DGRh was successfully and even promptly concluded. The recommendations are a solid basis for treating patients of all ages with FMF. The explanations on the problem of colchicine resistance play an important role here.

Psoriatic arthritis (PsA) is a very heterogeneous immune-mediated disease that usually involves skin and joints but can also affect entheses and extra-articular structures during the disease course. Furthermore, it can also be linked with other associated diseases. Therefore, the individualized selection of an effective and patient-oriented treatment must be carried out taking the extent of various manifestations of the PsA itself and also of other influencing factors into consideration. Various recommendations for selection and control of the suitable treatment of PsA are available for clinical use. The recommendations of the European League Against Rheumatism (EULAR) and the Group for Research and Assessment of Psoriasis and Psoriatic Arthritis (GRAPPA) are the two recommendations that are frequently used and internationally acknowledged. Both recommendations were updated in 2016. Specific German treatment recommendations are currently missing. In analogy to the treat-to-target strategy for rheumatoid arthritis, at least minimal disease activity (MDA) should be achieved in PsA patients with the use of specific therapeutic interventions if remission as the maximum therapeutic goal cannot be reached. New treatment options, which target different specific molecules, offer possibilities for a more differentiated personalized medicinal treatment for improvement of the care of PsA patients. This particularly applies to a focus on personalized strategies for optimal treatment of various manifestation forms and patterns.

OBJECTIVE: This study aims to evaluate the effects of passive smoking on disease activity in female patients with rheumatoid arthritis (RA).
METHODS: Among a total of 191 female patients with RA (mean age 59.1±12.5 years; range 21 to 87 years) consecutively recruited, 100 female patients (mean age 56.1±13.4 years; range 21 to 87 years) completed the study with mean 17.3 months of follow-up. Patients were classified according to smoking status: current, never, passive, or ex-smoker. Clinical response between never and passive smokers was assessed by disease- activity score 28 (DAS28)-erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) (DAS28-ESR and DAS28-CRP) and by the European League Against Rheumatism response criteria.
RESULTS: Among the 100 female RA patients analyzed, the distribution of smoking status was as follows: current (n=3), never (n=55), passive (n=34), and ex-smokers (n=8). There was no difference of DAS28-ESR and DAS28-CRP between never and passive smokers at baseline. At the time of follow-up, the values of DAS28-ESR and DAS28-CRP in never smokers were significantly decreased than those in passive smokers (p=0.019 and p=0.023, respectively). Patients who never smoked showed a trend to have good or moderate European League Against Rheumatism response without statistical significance, compared to passive smokers (52.7% vs. 32.4%, respectively; p=0.060).
CONCLUSIONS: This preliminary study implicates that passive smoking might be responsible for higher disease activity in female RA patients and never smoking might induce good clinical response in RA.

Methotrexate (MTX) is used widely as a first-line drug for the treatment of rheumatoid arthritis (RA) worldwide. There are large interindividual differences in the therapeutic response to MTX, but it is not known which factors influence them. We therefore investigated predictive factors associated with the therapeutic response to MTX in a hospital-based cohort study. Japanese adult RA outpatients prescribed MTX were enrolled and their characteristics were collected from the electronic medical records. The European League Against Rheumatism (EULAR) response criteria were used as the response to MTX therapy. The observation period was 1 year after beginning MTX administration. Sixteen types of single-nucleotide polymorphisms were investigated using the real-time PCR method. Associations between the MTX response and patient characteristics were evaluated using the multivariate logistic regression model. Among 70 Japanese adult RA outpatients, 52 were classified as MTX responders. In multivariate analysis, patients with the solute carrier family 19 member 1 (SLC19A1) 80G>A A/A genotype had a better response than those with the A/G or G/G genotype, and patients with the C allele of γ-glutamyl hydrolase (GGH) 16T>C had a better response than those with the T/T genotype.This study showed that the therapeutic response to MTX in Japanese RA patients was associated with the genetic polymorphisms of SLC19A1 80G>A and GGH 16T>C in actual clinical practice.

The BAFF system plays a key role in the development of autoimmunity, especially in systemic lupus erythematosus (SLE). This often leads to the assumption that BAFF is mostly a B cell factor with a specific role in autoimmunity. Focus on BAFF and autoimmunity, driven by pharmaceutical successes with the recent approval of a novel targeted therapy Belimumab, has relegated other potential roles of BAFF to the background. Far from being SLE-specific, the BAFF system has a much broader relevance in infection, cancer and allergy. In this review, we provide the latest views on additional roles of the BAFF system in health and diseases, as well as an update on BAFF and autoimmunity, with particular focus on current clinical trials.