Clinical implications of seropositive and seronegative autoantibody status in rheumatoid arthritis patients: A comparative multicentre observational study.

Background and Objectives: Rheumatoid factor (RF) and anti-cyclic citrullinated protein (anti-CCP) have been used to improve the diagnosis and prognosis of rheumatoid arthritis (RA). However, their association with RA disease phenotypes, individually and in combination, is not well studied. The aim of the study was to compare patients' and disease characteristics, activity and severity in double seronegative (DNRA), single seropositive RF, single seropositive anti-CCP and double seropositive (DPRA) patients. Methods: Adults subjects with RA from Egyptian College of Rheumatology (ECR) database who had RF and anti-CCP results available were included. Demographic, clinical features, disease activity score 28 (DAS28), Health Assessment Questionnaire (HAQ) and laboratory data were collected and compared among different RA groups. Results: 5268 RA patients with mean age of 44.9±11.6 years, and 4477 (85%) were females. 2900 (55%) had DPRA, 892 (16.9%) had single positive RF, 597 (11.3%) had single positive anti-CCP while 879 (16.7%) had DNRA. Patients with DPRA had significantly high percentage of metabolic syndrome (19.3%, P < 0.001), and functional impairment using HAQ (P = 0.01). Older age (RRR [relative risk ratio]: 1.03, 95%CI: 1.0, 1.0, P = 0.029), greater DAS28 (RRR: 1.51, 95%CI: 1.2, 1.9, P < 0.001), higher steroid use (RRR: 2.4, 95%CI: 1.36, 4.25, P = 0.002) were at higher risk of DPRA while longer disease duration (RRR: 1.08, 95%CI: 1.01, 1.16, P = 0.017) and fibromyalgia syndrome (RRR: 2.54, 95%CI: 1.10, 5.88, P = 0.028) were associated with higher odds of single positive RF status. Conclusion: Dual antibody-positive status has higher disease activity and severity, and higher chance of development of metabolic syndrome; highlighting the implicated role of inflammation, atherogenesis and cardiovascular disease risk in RA.

Stroke frequency, associated factors, and clinical features in primary systemic vasculitis: a multicentric observational study.

OBJECTIVES: The cerebral vessels may be affected in primary systemic vasculitis (PSV), but little is known about cerebrovascular events (CVEs) in this population. This study aimed to determine the frequency of CVEs at the time of diagnosis of PSV, to identify factors associated with CVEs in PSV, and to explore features and outcomes of stroke in patients with PSV. METHODS: Data from adults newly diagnosed with PSV within the Diagnostic and Classification Criteria in VASculitis (DCVAS) study were analysed. Demographics, risk factors for vascular disease, and clinical features were compared between patients with PSV with and without CVE. Stroke subtypes and cumulative incidence of recurrent CVE during a prospective 6-month follow-up were also assessed. RESULTS: The analysis included 4828 PSV patients, and a CVE was reported in 169 (3.50%, 95% CI 3.00-4.06): 102 (2.13% 95% CI 1.73-2.56) with stroke and 81 (1.68% 95% CI 1.33-2.08) with transient ischemic attack (TIA). The frequency of CVE was highest in Behçet's disease (9.5%, 95% CI 5.79-14.37), polyarteritis nodosa (6.2%, 95% CI 3.25-10.61), and Takayasu's arteritis (6.0%, 95% CI 4.30-8.19), and lowest in microscopic polyangiitis (2.2%, 95% CI 1.09-3.86), granulomatosis with polyangiitis (2.0%, 95% CI 1.20-3.01), cryoglobulinaemic vasculitis (1.9%, 95% CI 0.05-9.89), and IgA-vasculitis (Henoch-Schönlein) (0.4%, 95% CI 0.01-2.05). PSV patients had a 11.9% cumulative incidence of recurrent CVE during a 6-month follow-up period. CONCLUSION: CVEs affect a significant proportion of patients at time of PSV diagnosis, and the frequency varies widely among different vasculitis, being higher in Behçet's. Overall, CVE in PSV is not explained by traditional vascular risk factors and has a high risk of CVE recurrence.

Unsupervised cluster analysis of clinical and ultrasound features reveals unique gout subtypes: Results from the Egyptian College of Rheumatology (ECR).

BACKGROUND: Gout comprises a heterogeneous group of disorders; however, comorbidities have been the focus of most efforts to classify disease subgroups. OBJECTIVES: We applied cluster analysis using musculoskeletal ultrasound (MSUS) combined with clinical and laboratory findings in patients with gout to identify disease phenotypes, and differences across clusters were investigated. PATIENTS AND METHODS: Patients with gout who complied with the ACR/EULAR classification criteria were enrolled in the Egyptian College of Rheumatology (ECR)-MSUS Study Group, a multicenter study. Selected variables included demographic, clinical, and laboratory findings. MSUS scans assessed the bilateral knee and first metatarsophalangeal joints. We performed a K-mean cluster analysis and compared the features of each cluster. RESULTS: 425 patients, 267 (62.8 %) males, mean age 54.2 ± 10.3 years were included. Three distinct clusters were identified. Cluster 1 (n = 138, 32.5 %) has the lowest burden of the disease and a lower frequency of MSUS characteristics than the other clusters. Cluster 2 (n = 140, 32.9 %) was mostly women, with a low rate of urate-lowering treatment (ULT). Cluster 3 (n = 147, 34.6 %) has the highest disease burden and the greatest proportion of comorbidities. Significant MSUS variations were found between clusters 2 and 3: joint effusion (p < 0.0001; highest: cluster 3), power Doppler signal (p < 0.0001; highest: clusters 2), and aggregates of crystal deposition (p < 0.0001; highest: cluster 3). CONCLUSION: Cluster analysis using MSUS findings identified three gout subgroups. People with more MSUS features were more likely to receive ULT. Treatment should be tailored according to the cluster and MSUS features.

Serum pentraxin 3 in systemic lupus erythematosus: A potential indicator of cutaneous disease activity.

BACKGROUND: Although skin manifestations are common in systemic lupus erythematosus (SLE), there is still a lack of a diagnostic marker for cutaneous involvement. Pentraxin3 (PTX3) has been studied in SLE patients; however, it has not been investigated in relation to cutaneous manifestations. OBJECTIVE: To assess the serum PTX3 level in SLE patients, and to investigate its relationship with disease activity as well as with variable skin manifestations. PATIENTS AND METHODS: Thirty-four patients with SLE (17 patients with skin manifestations and 17 without) and 30 healthy subjects were included in the study. Patients were evaluated clinically for systemic and skin manifestations of SLE. Systemic Lupus Erythematosus Disease Activity Index (SLEDAI-2k) and Cutaneous Lupus Erythematosus Activity and Severity Index (CLASI) scores were calculated. Serum level of PTX3 was measured in patients and controls using ELISA. RESULTS: Higher serum PTX3 level was found in SLE patients compared to controls (p < 0.001). Patients with skin manifestations showed higher SLEDAI-2k scores and had higher PTX3 level compared to those without skin manifestations (p = 0.015 and p < 0.001, respectively). PTX3 showed higher levels in association with malar rash (p < 0.001), mucosal ulcers (p < 0.001), alopecia (p < 0.001), and purpuric eruption (p = 0.002). Moreover, PTX3 level positively correlated with CLASI scores (p < 0.001). CONCLUSION: Our results reinforce the important role of Pentraxin3 in SLE patients with skin manifestations, and it may be considered an interesting biomarker for the pattern and extent of cutaneous involvement in SLE.

Development of machine learning models for detection of vision threatening Behçet's disease (BD) using Egyptian College of Rheumatology (ECR)-BD cohort.

BACKGROUND: Eye lesions, occur in nearly half of patients with Behçet's Disease (BD), can lead to irreversible damage and vision loss; however, limited studies are available on identifying risk factors for the development of vision-threatening BD (VTBD). Using an Egyptian college of rheumatology (ECR)-BD, a national cohort of BD patients, we examined the performance of machine-learning (ML) models in predicting VTBD compared to logistic regression (LR) analysis. We identified the risk factors for the development of VTBD. METHODS: Patients with complete ocular data were included. VTBD was determined by the presence of any retinal disease, optic nerve involvement, or occurrence of blindness. Various ML-models were developed and examined for VTBD prediction. The Shapley additive explanation value was used for the interpretability of the predictors. RESULTS: A total of 1094 BD patients [71.5% were men, mean ± SD age 36.1 ± 10 years] were included. 549 (50.2%) individuals had VTBD. Extreme Gradient Boosting was the best-performing ML model (AUROC 0.85, 95% CI 0.81, 0.90) compared with logistic regression (AUROC 0.64, 95%CI 0.58, 0.71). Higher disease activity, thrombocytosis, ever smoking, and daily steroid dose were the top factors associated with VTBD. CONCLUSIONS: Using information obtained in the clinical settings, the Extreme Gradient Boosting identified patients at higher risk of VTBD better than the conventional statistical method. Further longitudinal studies to evaluate the clinical utility of the proposed prediction model are needed.

Rheumatoid arthritis study of the Egyptian College of Rheumatology (ECR): nationwide presentation and worldwide stance.

To depict the spectrum of rheumatoid arthritis (RA) in Egypt in relation to other universal studies to provide broad-based characteristics to this particular population. This work included 10,364 adult RA patients from 26 specialized Egyptian rheumatology centers representing 22 major cities all over the country. The demographic and clinical features as well as therapeutic data were assessed. The mean age of the patients was 44.8 ± 11.7 years, disease duration 6.4 ± 6 years, and age at onset 38.4 ± 11.6 years; 209 (2%) were juvenile-onset. They were 8750 females and 1614 males (F:M 5.4:1). 8% were diabetic and 11.5% hypertensive. Their disease activity score (DAS28) was 4.4 ± 1.4 and health assessment questionnaire (HAQ) 0.95 ± 0.64. The rheumatoid factor (RF) and anti-cyclic citrullinated peptide (anti-CCP) were positive in 73.7% and 66.7% respectively. Methotrexate was the most used treatment (78%) followed by hydroxychloroquine (73.7%) and steroids (71.3%). Biologic therapy was received by 11.6% with a significantly higher frequency by males vs females (15.7% vs 10.9%, p = 0.001). The least age at onset, F:M, RF and anti-CCP positivity were present in Upper Egypt (p < 0.0001), while the highest DAS28 was reported in Canal cities and Sinai (p < 0.0001). The HAQ was significantly increased in Upper Egypt with the least disability in Canal cities and Sinai (p = 0.001). Biologic therapy intake was higher in Lower Egypt followed by the Capital (p < 0.0001). The spectrum of RA phenotype in Egypt is variable across the country with an increasing shift in the F:M ratio. The age at onset was lower than in other countries.

Metabolic Syndrome in Behçets Disease Patients: Keep an Eye on the Eye.

BACKGROUND: Metabolic syndrome (MetS) is characterized by insulin resistance, high blood pressure/ sugar, dyslipidemia, and obesity. Whether MetS and its components affect the development of Behçet's Disease (BD) remains unclear. AIMS: The aim was to determine the frequency of MetS among BD patients and to study its relationship with disease characteristics. METHODS: The study included 1028 adult BD patients recruited from 18 specialized rheumatology centers. 51 healthy matched control were considered. Behçet Disease Current Activity Form (BDCAF) and the BD damage index (BDI) were estimated. Adult Treatment Panel-III criteria were used to define MetS. RESULTS: The mean age of patients was 36.8 ± 10.1 years, M:F 2.7:1 and disease duration 7.01 ± 5.2 years. Their mean BDCAF was 5.1 ± 4.6 and BDI 5.5 ± 2.8. MetS was present in 22.8% of patients and in 5.9% of control (3.9 fold higher-risk). Patients with MetS had a significantly increased age at onset (31.8 ± 9.2 vs. 29 ± 8.5 years) and higher frequency of genital ulcers (96.2% vs. 79.7%), skin involvement (73.1% vs. 50.4%), arthritis (48.3% vs. 29.1%) (p<0.0001) and CNS manifestations (18.8% vs. 13%) (p=0.042) compared to those without it. Eye involvement was significantly increased in those with MetS (82.1% vs. 74.2%) (p=0.003) with increased frequency of posterior uveitis (67.1% vs. 43.5%), retinal vessel occlusion (35.9% vs. 21.3%), retinal vasculitis (41.9% vs. 26.4%) (p<0.0001) and vitritis (37.2% vs. 24%) (p=0.001). BDCAF was significantly lower (3.9 ± 4.3 vs. 5.6 ± 4.6) and BDI higher (7.4 ± 2.7vs5 ± 2.6) (p<0.0001). CONCLUSION: BD patients with MetS are predisposed to mucocutaneous, musculoskeletal, neuropsychiatric and ocular manifestations with consequently increased damage. The involvement of the deeper structures of the eye should alarm rheumatologists to keep in mind that all patients should have an eye examination, especially those with MetS.

Monitoring and Achievement of Target Serum Urate Among Gout Patients Receiving Long-Term Urate-Lowering Therapy in the American College of Rheumatology RISE Registry.

OBJECTIVE: The American College of Rheumatology's (ACR) 2020 guidelines for the management of gout recommend using a treat-to-target approach to lower serum urate (SU). Using the ACR's Rheumatology Informatics System for Effectiveness registry, we examined the use of a treat-to-target approach among gout patients receiving long-term urate-lowering therapy (ULT) and followed longitudinally by rheumatologists. METHODS: Included patients had one or more diagnoses for gout in 2018-2019 and continuous use of ULT for ≥12 months. We assessed the proportions of patients with SU monitoring and, among those tested, who achieved SU <6.0 mg/dl during the measurement year. Multilevel logistic regression adjusting for sociodemographics, comorbidities, region, and health care utilization was used to determine factors associated with SU monitoring and achievement of target SU. RESULTS: A total of 9,560 were included. The mean ± SD age was 67.2 ± 12.7 years, 73.5% of patients were male, and 32.3% were non-White. Fifty-six percent of patients had at least 1 SU recorded during the measurement year; among patients with at least 1 SU recorded, 74% achieved the SU target. In multivariate analyses, non-White patients were slightly less likely to be tested or achieve a target SU. CONCLUSION: Among gout patients receiving long-term ULT followed longitudinally by rheumatologists, more than half had a documented SU, and among those tested, three-quarters achieved the recommended SU target. Routine monitoring of SU is a first step toward improving quality of care for patients with gout.

Evaluating the Use of Glucocorticoids Among Belimumab-Treated Patients With Systemic Lupus Erythematosus in Real-World Settings Using the Rheumatology Informatics System for Effectiveness Registry.

OBJECTIVE: Glucocorticoids are part of standard therapy for systemic lupus erythematosus (SLE), despite adverse effects associated with long-term treatment. Belimumab improved clinical manifestations of SLE and reduced glucocorticoid doses in clinical trials and clinical practice; however, associations have not been examined using multi-institutional electronic health record (EHR) data. Using the Rheumatology Informatics System for Effectiveness registry, we examined glucocorticoid use patterns among belimumab-treated adults with SLE. METHODS: This retrospective analysis (GSK Study 209267) used EHR prescription records of patients with SLE managed by rheumatologists. Eligible patients had an index date (first belimumab prescription) between January 2014 and June 2018. The primary analysis compared patients' mean daily oral glucocorticoid (prednisone equivalent) dose over the 6 months preindex versus 6 months post index. An exploratory analysis assessed glucocorticoid doses at 12 and 24 months post index for patients with extended follow-up. RESULTS: Of the 1987 patients receiving their first belimumab prescription, 767 had available glucocorticoid prescribing data, whereas 204 (primary analysis population) had glucocorticoids prescribed in the 6 months preindex and received belimumab according to the prescribing information for the first 8 weeks post index. The mean (SD) glucocorticoid dose was 12.5 (13.5) mg/day 3 months preindex, reducing to 10.3 (10.6) mg/day over the 6 months post index, and 8.7 (9.4) and 9.0 (9.3) mg/day at 12 and 24 months post index. CONCLUSION: This study showed reductions in mean daily glucocorticoid dose after belimumab initiation. Several limitations of EHRs for real-world effectiveness research were identified, which limited interpretation of results and may inform future study designs.

Diagnostic value of salivary gland ultrasonography for secondary Sjögren syndrome in patients with systemic lupus erythematosus.

BACKGROUND: Salivary gland ultrasound (SGUS) is a reliable technique for assessing the salivary glands in patients with primary Sjögren's syndrome (SS); however, the role of SGUS for diagnosis of secondary SS (sSS) in patients with systemic lupus erythematosus (SLE) was not examined. OBJECTIVE: To assess the diagnostic value of SGUS for sSS in patients with SLE, and to investigate the relationship between SGUS findings with clinical and laboratory characteristics of patients with SLE. PATIENTS AND METHODS: This cross-sectional study included 49 patients with SLE. The diagnosis of sSS was confirmed according to the 2016 ACR/EULAR criteria. Salivary gland US was performed for all patients and graded using a validated Hocevar scoring system. A complete clinical and laboratory workup for SLE was assessed. Schirmer's test and the ocular staining were performed. RESULTS: Of the 49 patients with a mean age of 30.2 ± 9.6 years, 98% were female. 19 (38.8%) had sSS. SGUS changes consistent with sSS (≥17) were found in 29 (59.2%) of the patients. Patients with higher SGUS score had more sicca findings as well as positive anti-Ro, anti-La antibodies, and poorer psychological stress (p < 0.05). The SGUS (≥17) showed a sensitivity of 84.2% and a specificity of 56.7% for sSS diagnosis, with an area under the curve of 0.77 (95% CI: 0.63, 0.91). CONCLUSION: We propose salivary gland ultrasound as a non-invasive method in the diagnostic workup for sSS in patients with SLE. Further studies to confirm the diagnostic value of SGUS in a larger sample of patients with sSS will be necessary.

The Emerging Era of Interventional Imaging in Rheumatology: An Overview During the Coronavirus Disease-2019 (COVID-19) Pandemic.

Imaging has long been taking its place in the diagnosis, monitor, and prognosis of rheumatic diseases. It plays a vital role in the appraisal of treatment. Key progress in the clinical practice of rheumatology is the innovation of advanced imaging modalities; such as musculoskeletal ultrasound (MSUS), computerized tomography (CT) and magnetic resonance imaging (MRI). These modalities introduced a promising noninvasive method for visualizing bone and soft tissues to enable an improved diagnosis. The use of MSUS in rheumatology is considered a landmark in the evolution of the specialty and its ease of use and many applications in rheumatic diseases make it a forerunner instrument in the practice. The use of MSUS among rheumatologists must parallel the development rate of the excellence revealed in the specialty. Moreover, innovative interventional imaging in rheumatology (III-R) is gaining fame and key roles in the near future for a comprehensive management of rheumatic diseases with precision. This review article throws light on the emergence of these robust innovations that may reshape the guidelines and practice in rheumatology, in particular, efforts to enhance best practice during the coronavirus disease 2019 (COVID-19) pandemic are endorsed.

Rheumatologists' knowledge and perception of COVID-19 and related vaccines: the vaXurvey2 online survey.

The study aimed to explore the experience of coronavirus disease-2019 (COVID-19) infection and vaccine adverse events (AEs) among rheumatologists. A validated questionnaire was distributed as a Google form to rheumatologists across the country via social networking sites from late December 2021 till early January 2022. The questionnaire included questions regarding participants' socio-demographic details, COVID-19 infection and vaccination details with special emphasis on AEs. Out of 246 responses, 228 were valid. 200 (81.3%) responders had received the vaccine. The mean age of the 228 participants was 37.9 ± 8.5 years, 196 were females and 32 males (F:M 6.1:1) from 18 governorates across the country. Comorbidities were present in 54 subjects (27%). There was a history of highly suspicious or confirmed COVID-19 infection in 66.7% that were all managed at home. The COVID-19 vaccine was received by 200 and a booster dose of 18.5%. Obesity and musculoskeletal involvement co-morbidities were present only in those with AEs (9.1% and 5.5% respectively). AEs were present in 82%; 66.7% had injection-site tenderness, 50% fatigue, 35.5% fever, 15% chills, 42.5% myalgia, 14.5% arthralgia, 8% low back pain, headache 31%, dizziness 10%, sleepliness 16% and 15% developed post-vaccine. There were no differences according to the geolocation regarding the occurrence of COVID-19 infection (p = 0.19) or AEs post-vaccine (p = 0.58). The adverse events were mostly mild to moderate and tolerable which makes this work in agreement with other studies that support the broad safety of the vaccine in favor of the global benefit from mass vaccination.

Improvement in disease activity among patients with rheumatoid arthritis who switched from intravenous infliximab to intravenous golimumab in the ACR RISE registry.

Infliximab and golimumab are intravenously (IV) administered tumor necrosis factor inhibitors approved to treat moderate-to-severe rheumatoid arthritis (RA) with concomitant methotrexate. Owing to differences in biologic construct, patients with IV-infliximab treatment failure may benefit from switching to IV-golimumab. Utilizing the ACR's Rheumatology Informatics System for Effectiveness (RISE), a large electronic health records registry based in the USA, we assessed RA disease activity in patients switching from IV-infliximab to IV-golimumab. This retrospective, longitudinal, single-arm study included adults (≥ 18 years) with ≥ 1 RA diagnosis code between 2014 and 2018 and ≥ 1 IV-infliximab prescription within 6 months of a new IV-golimumab order (index date). Longitudinal assessments of disease activity using the Clinical Disease Activity Index (CDAI) were calculated in patients continuing IV-golimumab for 6-9- and 9-12-months post-switch. Paired t-tests evaluated significance of mean improvements during the follow-up periods. Most RA patients with disease activity assessments during the 6-month follow-up (N = 100; mean age: 65.3 years; 81% female; 74% white) demonstrated moderate-to-high disease activity (CDAI: 73% [38/52]) at enrollment. On average, patients showed significant improvement in disease activity within 6-9 months of switching; mean CDAI scores improved from 21.3 to 14.1 (p < 0.0001) and were durable through 9-12 months of treatment. Real-world patients with moderate-to-high disease activity who switched from IV-infliximab to IV-golimumab demonstrated significant and sustained improvements post-switch as measured by the CDAI. Key Points • This study used real-world data from the Rheumatology Informatics System for Effectiveness (RISE) registry to evaluate the efficacy of directly switching from intravenous (IV)-infliximab to IV-golimumab to control rheumatoid arthritis (RA) disease activity. • Most IV-infliximab patients had moderate-to-high disease activity at the time of the switch. • On average, IV-golimumab was effective in improving RA disease activity after switching from IV-infliximab as measured by the Clinical Disease Activity Index. • These data suggest that real-world RA patients with persistent symptoms despite treatment with IV-infliximab may realize improved disease control with a switch to IV-golimumab.

The clinical utility of faecal calprotectin in patients with differentiated and undifferentiated spondyloarthritis: Relevance and clinical implications.

OBJECTIVES: There is cumulative evidence in the literature supporting a potential role of faecal calprotectin (FCP) as a biomarker for gut inflammation in spondyloarthritis (SpA). However its relevance in undifferentiated SpA (USpA) is still uncertain. The aim of the current study is to assess the diagnostic significance of FCP levels in patients with differentiated and undifferentiated SpA. MATERIAL AND METHODS: A total of 52 differentiated SpA, 33 USpA and 50 controls could be included. For all patients, clinical evaluation, routine laboratory investigations, FCP levels, and occult blood in stool were performed. When indicated imaging and/or endoscopies were performed. RESULTS: The differentiated SpA patients were 12 (23.1%) with ankylosing spondylitis, 21 (40.4%) with psoriatic arthritis, 13 (25%) with ulcerative colitis, 5 (9.6%) with Crohn's disease (CD) and one (1.9%) with reactive arthritis. The mean FCP level in 85 patients correlated with CRP and ESR. Within the SpA group ulcerative colitis and Crohn's disease patients had increased FCP levels compared to other SpA subgroups and USpA patients (p<0.001). The mean FCP levelwas significantly higher in the SpA patients compared to USpA and controls (p<0.001). CONCLUSIONS: Elevated FCP levels may identify patients who are most likely to have SpA already in the unclassified phase of the disease. Further studies in different series of patients are needed to evaluate the potential diagnostic and prognostic roles of FCP in both differentiated and undifferentiated phases of the disease.

Treatment of Sarcoidosis in US Rheumatology Practices: Data From the American College of Rheumatology's Rheumatology Informatics System for Effectiveness (RISE) Registry.

OBJECTIVE: Sarcoidosis is often treated with glucocorticoids, although the use of biologics is growing. Prescribing patterns for biologics for patients with sarcoidosis in US rheumatology practices have never been examined. Given that there are no steroid-sparing US Food and Drug Administration-approved therapies for sarcoidosis, we sought to characterize the real-world treatment of sarcoidosis and to assess practice-level variation in prescribing patterns. METHODS: We conducted an observational study of patients with sarcoidosis using data from the Rheumatology Informatics System for Effectiveness (RISE) registry (2014-2018). The RISE registry represents an estimated 32% of the US clinical rheumatology workforce. Adult patients with ≥2 codes for sarcoidosis ≥30 days apart were included. We examined sarcoidosis-specific medication use at any time during the study period. Data were analyzed at the practice level. RESULTS: A total of 3,276 patients with sarcoidosis from 184 practices were included. Of those patients, 75.1% were women, with a mean age of 59.0 ± 12.5 years; 48.3% were White and 27.6% were Black. Overall, 59.3% of patients were prescribed glucocorticoids, and 24.7% received prolonged glucocorticoid therapy (≥10 mg/day for ≥90 days). In all, 12.1% received a biologic or targeted synthetic disease-modifying antirheumatic drug (tsDMARD), most commonly tumor necrosis factor inhibitors. There was wide practice-level variation among 31 practices with ≥30 patients with sarcoidosis; biologic use ranged from 15.6% to 69.2%. Infliximab represented the most common biologic prescribed. CONCLUSION: In a large sample of US rheumatology practices, 12.1% of patients with sarcoidosis received biologics or tsDMARDs. We found high variability in biologic use across practices. The significant use of long-term glucocorticoids suggests unmet therapeutic needs in this patient population.

Clinical Impact of the ABO Blood Type in Patients with Rheumatic Diseases: Is there a Link to the ABO and Rhesus?

OBJECTIVES: Several studies have shown associations of ABO and Rh blood groups with various diseases; however, the relationship of ABO and Rh blood groups with rheumatic diseases are scarce. The aim of the present study was to examine whether there is an association between ABO and Rh blood groups and the types of rheumatic diseases. METHOD: In this multi-centre cross-sectional study, sociodemographic data, type of rheumatic disease, and type ABO and Rh blood groups were examined for patients with different rheumatic diseases. RESULTS: A total of 304 patients; 207 (68.1%) were diagnosed with rheumatoid arthritis, and 40 (13.2%) had systemic lupus erythematosus. The patients were assessed for blood types; 37.8% patients had A type, 27.6% had B type, 19.1% had O type, and 15.4% had AB type. The Rh (+) blood group was more prevalent (89.1%) than Rh (-). Blood group A was more prevalent in patients with rheumatic disease, followed by B, O, and AB respectively, although there was no significant difference in the distribution of ABO groups among rheumatic diseases. Female gender, smoking, and anti-cyclic citrullinated peptide are significantly different between the blood groups within rheumatic diseases. CONCLUSION: The A and Rh (+) blood groups were more commonly observed in patients with rheumatic diseases. There was lack of association between types of rheumatic diseases and ABO blood groups. The study provides knowledge for the interaction between ABO blood groups and several risk factors related to rheumatic diseases and may serve a guide for future clinical studies.

The Relationship Between Electronic Health Record System and Performance on Quality Measures in the American College of Rheumatology's Rheumatology Informatics System for Effectiveness (RISE) Registry: Observational Study.

BACKGROUND: Routine collection of disease activity (DA) and patient-reported outcomes (PROs) in rheumatoid arthritis (RA) are nationally endorsed quality measures and critical components of a treat-to-target approach. However, little is known about the role electronic health record (EHR) systems play in facilitating performance on these measures. OBJECTIVE: Using the American College Rheumatology's (ACR's) RISE registry, we analyzed the relationship between EHR system and performance on DA and functional status (FS) quality measures. METHODS: We analyzed data collected in 2018 from practices enrolled in RISE. We assessed practice-level performance on quality measures that require DA and FS documentation. Multivariable linear regression and zero-inflated negative binomial models were used to examine the independent effect of EHR system on practice-level quality measure performance, adjusting for practice characteristics and patient case-mix. RESULTS: In total, 220 included practices cared for 314,793 patients with RA. NextGen was the most commonly used EHR system (34.1%). We found wide variation in performance on DA and FS quality measures by EHR system (median 30.1, IQR 0-74.8, and median 9.0, IQR 0-74.2), respectively). Even after adjustment, NextGen practices performed significantly better than Allscripts on the DA measure (51.4% vs 5.0%; P<.05) and significantly better than eClinicalWorks and eMDs on the FS measure (49.3% vs 29.0% and 10.9%; P<.05). CONCLUSIONS: Performance on national RA quality measures was associated with the EHR system, even after adjusting for practice and patient characteristics. These findings suggest that future efforts to improve quality of care in RA should focus not only on provider performance reporting but also on developing and implementing rheumatology-specific standards across EHRs.

Systemic lupus erythematosus children in Egypt: Homeland spectrum amid the global situation.

OBJECTIVES: This study aims to present the manifestations of juvenile systemic lupus erythematosus (JSLE) across Egypt, to focus on age at onset and gender-driven influence on disease characteristics, and to compare findings to other countries. METHODS: The study included 404 Egyptian children with systemic lupus erythematosus (SLE) presenting to one of the specialized rheumatology centers corresponding to 13 major governorates. Juvenile cases age was ≤ 16°years at the time of recruitment. The SLE Disease Activity Index (SLEDAI) and damage index (DI) were assessed. RESULTS: The mean age was 13.2 ± 2.4°years; 355 females and 49 males (7.2:1), and the disease duration was 2.3 ± 1.6 years, while age at disease onset was 11.1 ± 2.5°years. Their SLEDAI was 13.5 ± 12.3, and DI, 0.36 ± 0.78. The overall estimated prevalence of childhood-SLE patients in the recruited cohort in Egypt was 1/100,000 population (0.24/100000 males and 1.8/100000 females). 7.4% developed pre-pubertal SLE (≤ 7 years); 73.3%, peri-pubertal; and 19.3% during early adolescence. The differences according to age group were equal for gender and clinical manifestations except skin lesions present in 59.3% of pre-pubertal onset, 74.6% of peri-pubertal, and 84.2% of adolescents (p = 0.029), and renal involvement in 73.8% of peripubertal, 62.1% of pre-pubertal and 58.9% of adolescents (p = 0.03). Laboratory investigations, SLEDAI, and DI were similar among age categories. Lupus nephritis was more common in Egypt compared to JSLE from other countries. CONCLUSION: Our large multicenter study identified that female gender influenced disease characteristics with more frequent skin involvement. Skin lesions were significantly higher in adolescents, while renal involvement in peri-pubertal children.

Epidemiology and treatment of Behçet's disease in the USA: insights from the Rheumatology Informatics System for Effectiveness (RISE) Registry with a comparison with other published cohorts from endemic regions.

BACKGROUND: Behçet's disease (BD), a chronic systemic vasculitis, has distinct geographical and ethnic variation. Data regarding the epidemiology of patients with BD in the U.S. are limited; therefore, we sought to describe BD patient characteristics and medication use in the U.S., and compared them with data from patients from endemic regions. METHODS: We conducted a cross-sectional study using data from the RISE registry (2014-2018). Patients aged ≥ 18 years with BD were included. Sociodemographic and treatment information was extracted. We compared patients from the RISE registry to data from other published studies of patients with BD from endemic areas. RESULTS: One thousand three hundred twenty-three subjects with BD from the RISE registry were included. Mean age was 48.7 ± 16.3 years, female to male ratio was 3.8:1, and 66.7% were White. The most frequently used medications included glucocorticoids (67.6%) and colchicine (55.0%). Infliximab and adalimumab were the most used biologics (14.5% and 14.1%, respectively); 3.2% of patients used apremilast. The RISE registry had more women (79.3%), and patients were older compared to previously published BD studies from endemic areas. Methotrexate and TNFi were more commonly reported in RISE (21.8% and 29.4%) compared to studies from Egypt and Turkey. Colchicine, cyclosporine, and cyclophosphamide were more commonly used in cohorts from Egypt, Turkey, and Iran. CONCLUSIONS: Findings from the largest BD dataset in the U.S. suggest that BD patients are predominantly female. Further research is needed to explore the reasons for the higher prevalence of BD among women in the U.S. and its possible impact on disease severity and management.

Socioeconomic Disparities in Functional Status in a National Sample of Patients With Rheumatoid Arthritis.

Importance: Little is known about the association of poverty with functional status (FS) in patients with rheumatoid arthritis (RA) who use rheumatology care. Objectives: To examine the association between socioeconomic status (SES) and FS among patients with RA and to evaluate the association between SES and functional declines over time in patients who received at least some rheumatology care. Design, Setting, and Participants: This cohort study used data from the American College of Rheumatology's Rheumatology Informatics System for Effectiveness (RISE) registry between January 1, 2016, and December 31, 2018. Analyses included all adult patients with a confirmed RA diagnosis (ie, had ≥2 encounters associated with RA International Classification of Diseases codes ≥30 days apart) and at least 1 FS score documented between 2016 and 2018 seen at participating rheumatology practices. Data analysis was conducted from April to December 2020. Exposures: The Area Deprivation Index (ADI), a zip code-based indicator of neighborhood poverty, was used as a proxy for SES. ADI scores were categorized into quintiles. Main Outcomes and Measures: FS measures included Multidimensional Health Assessment Questionnaire (MDHAQ), Health Assessment Questionnaire Disability index, and Health Assessment Questionnaire-II. Cross-sectionally, mean FS scores were compared across ADI quintiles. Longitudinally, among patients with at least 2 FS scores, multilevel multivariate regression computed the probability of functional decline, defined as a change greater than the minimum clinically important difference, across ADI quintiles. In a subgroup analysis, whether disease activity mediated the association between SES and functional decline was examined. Results: Of the 83 965 patients included in the study, 66 649 (77%) were women, and 60 037 (72%) were non-Hispanic White. Mean (SD) age was 63.4 (13.7) years. MDHAQ was the most reported FS measure (56 928 patients [67.8%]). For all measures, mean (SD) FS score was worse at lower SES levels (eg, for MDHAQ quintile 1: 1.79 [1.87]; quintile 5: 2.43 [2.17]). In longitudinal analyses, the probability of functional decline was 14.1% (95% CI, 12.5%-15.7%) in the highest SES quintile and 18.9% (95% CI, 17.1%-20.7%) in the lowest SES quintile. The association between SES and functional decline was partially mediated (7%; 95% CI, 4%-22%) by disease activity. Conclusions and Relevance: In this cohort study of patients with RA, worse FS and faster declines in functioning over time were observed in patients with lower SES. These findings provide a framework for monitoring disparities in RA and for generating evidence to spur action toward achieving health equity.