Currently, there are no guidelines for the treatment of rheumatoid arthritis (RA) tailored to the context of the Kingdom of Saudi Arabia (KSA). Adaptation of guidelines accounts for contextual factors and becomes more efficient than de novo guideline development when relevant, good quality, and up-to-date guidelines are available. The objective of this study is to describe the methodology used for the adolopment of the 2021 American College of Rheumatology (ACR) guidelines for the treatment of RA in the KSA.
We followed the \'Grading of Recommendations Assessment, Development and Evaluation\' (GRADE)-ADOLOPMENT methodology. The adolopment KSA panel included relevant stakeholders and leading contributors to the original guidelines. We developed a list of five adaptation-relevant prioritization criteria that the panelists applied to the original recommendations. We updated the original evidence profiles with newly published studies identified by the panelists. We constructed Evidence to Decision (EtD) tables including contextual information from the KSA setting. We used the PanelVoice function of GRADEPro Guideline Development Tool (GDT) to obtain the panel\'s judgments on the EtD criteria ahead of the panel meeting. Following the meeting, we used the PANELVIEW instrument to obtain the panel\'s evaluation of the process.
The KSA panel prioritized five recommendations, for which one evidence profile required updating. Out of five adoloped recommendations, two were modified in terms of direction, and one was modified in terms of certainty of the evidence. Criteria driving the modifications in direction were valuation of outcomes, balance of effects, cost, and acceptability. The mean score on the 7-point scale items of the PANELVIEW instrument had an average of 6.47 (SD = 0.18) across all items.
The GRADE-ADOLOPMENT methodology proved to be efficient. The panel assessed the process and outcome positively. Engagement of stakeholders proved to be important for the success of this project.

Our aim was to compare the efficacy of rituximab, tocilizumab, and abatacept in individuals with rheumatoid arthritis (RA) refractory to treatments with MTX or TNFi agents.
We searched 6 databases until January 2023 for phase 2-4 RCTs evaluating patients with RA refractory to MTX or TNFi therapy treated with rituximab, abatacept, and tocilizumab (intervention arm) compared to controls. Study data were independently assessed by two investigators. The primary outcome was considered as achieving ACR70 response.
The meta-analysis included 19 RCTs, with 7,835 patients and a mean study duration of 1.2 years. Hazard ratios for achieving an ACR70 response at six months were not different among the bDMARDs, however, we found high heterogeneity. Three factors showing a critical imbalance among the bDMARD classes were identified: baseline HAQ score, study duration, and frequency of TNFi treatment in control arm. Multivariate meta-regression adjusted to these three factors were conducted for the relative risk (RR) for ACR70. Thus, heterogeneity was attenuated (I2 = 24%) and the explanatory power of the model increased (R2 = 85%). In this model, rituximab did not modify the chance of achieving an ACR70 response compared to abatacept (RR = 1.773, 95%CI 0.113-10.21, p = 0.765). In contrast, abatacept was associated with RR = 2.217 (95%CI 1.554-3.161, p < 0.001) for ACR70 compared to tocilizumab.
We found high heterogeneity among studies comparing rituximab, abatacept, and tocilizumab. On multivariate metaregressions, if the conditions of the RCTs were similar, we estimate that abatacept could increase the chance of reaching an ACR70 response by 2.2-fold compared to tocilizumab.

Takayasu arteritis (TA) is a large vessel vasculitis that involves the aorta and its major branches. The disease has a female preponderance, and it presents with a wide variety of symptoms including skin manifestations, mainly ulcerative nodules, pyoderma gangrenosum, and erythema nodosum-like lesions. We report a case of a 50-year-old female who presented to the outpatient department with multiple ulcerative lesions over both upper extremities and chest. On physical examination, the patient had pulseless upper limbs. Laboratory investigations revealed positive antinuclear antibodies (ANA) and raised inflammatory markers. CT angiography of the aorta showed thickened aortic arch with the obliterated lumen of the left common carotid and left subclavian arteries. A biopsy of the skin lesion revealed surface ulceration and densely inflamed granulation tissue with a fibroblastic proliferation of deeper tissues. The patient had three out of six features of the American College of Rheumatology 1990 (ACR-1990) criteria for the classification of TA and was diagnosed with TA associated with pyoderma gangrenosum. The patient was managed with steroids and immunosuppressants along with gentle wound debridement with grafting of skin wounds. Since TA has varying presentations, its diagnosis is often challenging and requires a combined approach including clinical signs and symptoms, as well as laboratory and radiological workup. The disease also requires long-term follow-up due to its remitting and relapsing course.

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].

In the development of rheumatoid arthritis, the cytokine interleukin-6 plays a role. An interleukin-6 cytokine-specific monoclonal antibody called olokizumab directly targets this cytokine. OKZ effectiveness and safety are being evaluated through this meta-analysis.
I looked up every published randomized controlled study on Clinicaltrials.gov, Scopus, Web of Science, Cochrane, and PubMed. I conducted the study using both the Mantel-Haenszel and inverse variance approaches. I evaluated bias in the included studies using the risk of bias tool 2.
In this meta-analysis, five trials totalling 2227 participants, were examined. In contrast to the placebo group, the olokizumab group had a significantly higher incidence of American College of Rheumatology 20; RR = 1.83, 95% CI [1.69, 1.99], P < 0.00001. Regarding Health Assessment Questionnaire-Disability Index improvement, olokizumab significantly outperformed the placebo group; MD = -0.28, 95% CI [-0.32, -0.24], P < 0.00001. The incidence of treatment-emergent adverse events was significantly higher in the olokizumab group than in the placebo group; RR = 1.10, 95% CI [1.04, 1.17], P = 0.0006. Furthermore, the incidence of treatment-emergent serious adverse events did not differ significantly between the olokizumab group and the placebo group; RR = 0.85, 95% CI [0.60, 1.20], P = 0.35.
In patients with rheumatoid arthritis, olokizumab combined methotrexate is well tolerated and more effective than placebo plus methotrexate.

BACKGROUND: The 2021 American College of Rheumatology (ACR) rheumatoid arthritis (RA) guideline considers the specific context of the United States which differs from that of Saudi Arabia in many aspects that may impact recommendations. The objective of this project was to adapt a set of prioritized recommendations from the 2021 ACR guideline for the treatment of rheumatoid arthritis RA for the context of Saudi Arabia, by the Saudi Society for Rheumatology (SSR).
METHODS: The process followed the GRADE-ADOLOPMENT methodology, and the reporting adhered to the RIGHT-Ad@pt checklist. Working groups included a coordination group and a 19-member panel representing different stakeholder groups. The Evidence to Decision (EtD) tables included evidence on health effects from the source guideline and contextual information from the Saudi setting.
RESULTS: The panel prioritized and adapted five recommendations from the source guideline. The process led to modifying two out of the five prioritized recommendations, all listed here. In naive patients with low disease activity, methotrexate (MTX) is conditionally recommended over sulfasalazine (SSZ) (modified direction); hydroxychloroquine (HCQ) is conditionally recommended over SSZ (unmodified). Initiation of csDMARDs with short-term glucocorticoids is conditionally recommended over csDMARDs alone in naive patients with moderate to high disease activity (modified direction). Switch to subcutaneous MTX is conditionally recommended over addition/switch to alternative DMARD(s) in patients taking oral MTX who are not at target (unmodified). Discontinuation of MTX is conditionally recommended over gradual discontinuation of the bDMARD or tsDMARD for patients taking MTX plus a bDMARD or tsDMARD who wish to discontinue a DMARD (unmodified).
CONCLUSIONS: Rheumatologists practicing in Saudi Arabia can use the adoloped recommendations generated by this project while adopting the rest of the recommendations from the 2021 ACR guidelines.

To review: 1) degree of conformity to the American College of Rheumatology neuropsychological battery (ACR-NB) among studies that used a NB, 2) review definitions of cognitive impairment (CI) from studies that used a NB, and 3) characterize measurement tools used to assess CI in systemic lupus erythematosus (SLE).
The literature search was conducted in Ovid Medline, Embase, and PsycINFO for articles on CI in adult SLE patients. We reviewed studies that used a NB and compared their tests to the ACR-NB to assess the degree of conformity. Definitions of CI from studies that used a NB were reviewed when sufficient information was available. We reviewed and categorized CI measurement tools into four broad categories: NB, screening, incomplete/mixed batteries, and computerized batteries.
Of 8727 references, 118 were selected for detailed review and 97 were included in the final analysis. Of 43 studies that used a NB, none of the studies used the ACR-NB exactly as published. Many studies supplemented with other tests. Overall, there was inconsistent use of ACR-NB tests. Definitions for CI varied, with cut-offs ranging from 1 to 3 standard deviations below normative values on domains/tests varying in type and number. The most frequently used measurement tool for assessing CI in SLE was a NB. Use of screening tests and computerized batteries have also increased over the last decade.
The assessment and definition of CI in SLE remains heterogeneous. A consensus meeting to address existing inconsistencies should be considered to harmonize the field of CI in SLE.

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.

Juvenile idiopathic arthritis (JIA) is the most common but extremely heterogeneous group of rheumatic diseases of childhood. There are no reliable, well-researched and published biomarkers for diagnosis or monitoring in juvenile idiopathic arthritis as there are for rheumatoid arthritis (RA) in adults. Biomarkers are not utilized in classifying JIA as they are in adult RA, making the JIA classifications less clinically effective and informative. The situation presents a lost opportunity for early aggressive therapy in JIA patients. Various researchers have used diverse biomarkers anecdotally in JIA and more systematically in RA patients and have drawn inferences on their utility from their experiences. The experience with biomarkers from RA patients cannot necessarily be extrapolated for JIA patients because they are dissimilar diseases. This article reconnoiters the comparative utility of various arthritis biomarkers in adult as well as in JIA patients. In contrast to RA, JIA is in itself a diverse group of arthritis with clinically overlapping subgroups with diverse etiology. The difference in the etiopathogenesis of arthritis subgroups demands identifying subgroup-specific biomarkers for diagnosis/monitoring and subgroup-specific therapies for management. The diagnostic/prognostic value of the individual biomarker could be different in different types of arthritis and in different types of hosts. Understanding the utility of individual biomarkers and careful selection of the assay are important to achieve the best disease outcomes.