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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.

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.

Behcet’s disease (BD), commonly seen in the Silk road countries, is a variable vessel vasculitis with no specific investigation that reflects disease activity. The Behcet’s Disease Current Activity Form (BDCAF) is the most famous and acceptable clinical activity score. To develop a cross-cultural adaptation of the BDCAF to the Arabic language (Ar-BDCAF)—Egyptian dialect—across the country and to consider preliminary evaluation of its reliability in assessment of BD activity. The score was translated to Arabic language and revised by 3 rheumatology consultants. Reliability of Ar-BDCAF was tested among 88 BD patients from 9 Egyptian main city centers. Patients were questioned by two specialists at 30 min interval to evaluate inter-observer rating and twice by the same physician within 24 h to assess the intra-observer rating. Patients were 64 males and 24 females (2.7:1) with a mean age of 35 ± 10.3 years. The average time required by the consultant to fill in the form was 5.1 ± 2.2 min (1.5–15 min). The mean Ar-BDCAF scores were 9.81 ± 6.22 (0–25) and 9.53 ± 6.13 (0–28) with an intra-observer concordance (p = 0.28) and was 9.95 ± 6.47 (0–29) for the inter-observer rating (p = 0.89 and p = 0.66, respectively). The Ar-BDCAF is a measurable, easy to calculate, and reliable index for assessing disease activity in Egyptian BD. The Ar-BDCAF score can be used in daily clinical practice to assess BD activity and its use can be extended to other Arab countries for possible regional validation and adaptations.

Objectives To study the frequency of different autoantibodies to extractable nuclear antigens (ENAs) in rheumatoid arthritis (RA) patients and to correlate findings with clinical manifestations, disease activity and radiological damage. Methods A total of 230 RA patients were included and 75 healthy controls. In all patients rheumatological assessment was done and routine laboratory investigations and immune profile were performed in both patients and controls, including: RF, ACPA, ANA and anti-ENAs (Ro/SSA, La/SSB, U1-RNP, anti-Jo-1 and anti-Sm). Radiological damage was scored using Sharp/van der Heijde, and disease activity was evaluated by DAS28-ESR and DAS28-CRP. Results RF was positive in 101 (43.9%), ACPA in 220 (95.7%), ANA in 58 (25.2%), anti Ro in 31 (13.5%), anti-La in 10 (4.3%), anti-Jo1 in 5 (2.2%) and anti-RNP in 2 (0.9%). Anti-Ro/SSA positively correlated with sicca symptoms (p = .02), RF titer (p < .001), ANA (p < .001), DAS28-ESR (p = .026), and DAS28-CRP (p = .003). Anti-La antibodies correlated positively with SJC (p = .001), TJC (p = .001), ANA (p < .001), DAS-28 ESR (p = .007). Anti-Jo-1 correlated positively with interstitial lung disease (ILD) (p ≤ .001), RF titer (p = .037) and ANA (p ≤ .001). Anti-RNP antibodies correlated positively with disease duration (p ≤ .001), ACPA titer (p ≤ .001) and ANA (p = .014). In the controls ANA was positive in two (2.7%), anti-Ro in three (4%), and none of the controls tested positive for other autoantibodies. Conclusions In RA patients, positive ANA is frequent and positively associated with anti-Ro, anti-La and anti-Jo1 autoantibodies. Screening for autoantibodies against other anti-ENAs seems mandatory in RA patients especially when ANA is positive. RA cases with positive Anti-Jo-1 may develop anti synthetase syndrome and ILD.

Background: Women with rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE) are at high risk of cardiovascular diseases (CVD). The atherogenic index of plasma (AIP) is a new marker for the assessment of CVD. Objective: This study aimed to determine the predictive value of AIP with long-term CVD risk among women with RA and SLE. Methods: This is a cross-sectional study of 99 RA and 59 SLE women. Demographic, clinical, and biochemical data were obtained, and disease activities were calculated. For each patient, the longterm risk of CVD was calculated using the Framingham risk score (FRS); AIP was derived according to the logarithmic (triglycerides/high-density lipoproteins cholesterol). Results: The mean age of the RA and SLE patients was 47.97 ± 8.78 and 36.75 ± 9.09 years, respectively. The median (interquartile range) of AIP values in RA and SLE patients were 0.34 (-0.15, 1.02) and 0.33 (-0.53, 0.96), respectively, while FRS values of RA patients and SLE patients were 6.38 ± 5.58 and 4.86 ± 4.5, respectively (p >0.05). There was a moderate correlation between AIP and FRS in RA and SLE patients (r=0.42, p=0.002 and r=0.33, p=0.007, respectively). According to the multivariate regression analyses, we found that AIP value is an independent factor for FRS in RA (β: 4.13, 95% confidence interval; 1.71, 6.18; p=0.008) and in SLE patients (β: 6.19, 95% confidence interval; 2.58, 9.81; p Conclusions: : We reported that AIP can be used as an independent indicator for long-term CVD risk in RA and SLE patients.

Rationale: There are limited tools to identify which children are at greatest risk for developing sleep-disordered breathing (SDB)-associated behavioral morbidity.Objectives: To examine association...

Objective Immunosuppressant drugs can increase the risk of hepatitis B virus (HBV) and hepatitis C virus (HCV) and tuberculosis (TB) reactivation. Using the American College of Rheumatology's Rheumatology Informatics System for Effectiveness (RISE) registry, we examined pre-treatment screening among new users of biologic or targeted synthetic disease modifying drugs (DMARDs). Methods Data, derived from RISE, included patients ≥ 18 years old who were new users of biologic or targeted synthetic DMARDs. We developed quality measures related to pre-treatment screening for HBV, HCV, and TB in addition to a “composite” measure for all applicable tests. We assessed patient-level screening rates, practice-level variation among practices reporting on ≥ 20 patients, and the frequency of positive results. Results We included 26,802 patients across 213 rheumatology practices nationwide. Patients were 58 (14) years old, 75.9% female; 59.6% had rheumatoid arthritis, and TNFi were the most common index DMARDs (64.9%). Overall, 44.8% and 40.5% patients had any documented HBV or HCV screening, respectively, prior to the index date; 29.7% had TB screening in the year prior to drug start. Only 15.5% had documentation of screening for all appropriate infections prior to drug start. Practice-level performance on the composite measure was low (range 0 to 48.3%). 2.4% of screening tests were positive. Conclusion We found gaps in documentation of key safety measures among practices participating in RISE. Given the small but significant number of patients with active or latent infections that pose safety risks, developing standardized and reliable strategies to capture safety screening measures is paramount.

Rheumatoid arthritis (RA) is a chronic inflammatory disease, characterized by polyarthritis and systemic manifestations. RA-fatigue is a significant problem and adds on disease burden. Sleep disturbance, depression, and disease activity are suggested contributing factors to RA-fatigue; however, their combined role did not examine before among Egyptian RA patients. The objective of the study was to investigate the presence of fatigue, sleep and mood disturbances in RA patients. Also, to evaluate the possible association of poor sleep, depression, and disease activity with RA-fatigue.This cross-sectional study included 115 RA patients diagnosed according to the 2010 ACR-EULAR criteria and 46 age and sex matched controls. Fatigue using the Multidimensional Assessment of Fatigue-Global Fatigue Index, sleep using the Pittsburgh Sleep Quality Index and mood status using Beck Depression Inventory were assessed for all participants. RA disease activity was evaluated using disease activity score-28 joints.RA patients had higher mean fatigue, sleep disturbance, and depression scores (27.2±8.9, 6.4±3.6, and 12.8±7.3; respectively) than controls (22.7±7, 4.8±3, 7.8±5.9; respectively) (P.05). Poor sleep, depression and higher disease activity were significantly correlated with fatigue (r=0.4, r=0.65, r=0.55; respectively) (P.001). The three variables may explain up to 49.1% of the variation in fatigue on multiple regression analysis.Fatigue, poor sleep, and depression are more common in Egyptian patients with RA. A remarkably higher fatigue was associated with poor sleep, depression, and high disease activity, thus monitoring these silent comorbidities in clinical practice is required.

Systemic lupus erythematosus (SLE) and Sjogren’s syndrome (SS) may coexist and carry a higher risk for future comorbidities. Although 14-3-3η protein is recently a known diagnostic marker in rheumatoid arthritis (RA), its role has not been investigated in SLE. The aim of this study was to compare serum 14-3-3η protein level in SLE and RA patients and to examine its association with clinical and laboratory features in SLE patients. Eighty-four SLE patients and 39 RA patients were included. Sociodemographic, SLE disease activity index (SLEDAI), and damage index were assessed for SLE patients. Data about secondary SS were collected. 14-3-3η was measured by ELISA; titres above 0.19 ng/ml were considered positive. Serum 14-3-3η protein in SLE was significantly lower than in RA (0.37 ± 0.09 vs 1.5 ± 0.51; p

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.

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.

Key Points Question Are there socioeconomic disparities in functional status among individuals with rheumatoid arthritis seen in rheumatology practices? Findings In this national cohort of 83 965 individuals with rheumatoid arthritis, functional status was statistically significantly worse across each successively lower quintile of socioeconomic status (SES). In addition, the probability of functional decline over the study period was statistically significantly higher in individuals with low SES (18.9% in the lowest SES quintile) compared with individuals with high SES (14.1% in the highest SES quintile). Meaning These findings suggest that disparate health outcomes exist among individuals with rheumatoid arthritis seen in rheumatology practices., This cohort study examines the association between socioeconomic status and functional status among patients with rheumatoid arthritis and evaluates the association between socioeconomic status and functional declines over time in patients who received at least some rheumatology care., 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.

Introduction Rheumatoid arthritis (RA) is the most widespread chronic inflammatory rheumatic disease over the world. It is characterized by chronic proliferation of synovium, cartilage destruction, and periarticular erosion/bone loss. We investigated the serum levels of the C-telopeptide of type II collagen (CTX-II), Dickkopf-1 (DKK1), and cartilage oligomeric matrix protein (COMP) in relationship to the disease activity. Material and methods Serum COMP, CTX-II, and DKK1 levels were measured in 63 RA patients and 50 person age and gender matched as a healthy controls by ELISA test. Disease activity score (DAS) were calculated. Results The mean level of and COMP and CTX-II were significantly higher in patients with RA than in healthy controls (5.71 ±7.04 vs. 2.70 ±1.31 ng/ml, and 0.45 ±0.27 vs. 0.23 ±0.16 ng/ml, respectively; p < 0.001). Also, DKK1 serum levels were significantly higher in patients with RA than in healthy controls (6970.68 ±7566.68 vs. 3276.96 ±1306.77 pg/m; p < 0.001). There was a positive significant correlation between DKK1 and swollen joint (r = 0.42, p < 0.001). There were no significant differences in the number of patients, gender, the duration of RA disease, DAS, and RF. Sensitivity was 58.7% and specificity was 85.7% at a cut-off point (> 3.6 ng/ml) for serum COMP in RA patients, while, sensitivity was 100% and specificity was 52.4% at a cut-off point (> 0.15 ng/ml) for serum CTX-II and sensitivity was 68.3% and specificity was 95.2 % at a cut-off point (> 4876 pg/ml) for serum DKK1. Conclusions Measurement of some serological biomarkers such as CTX-II, COMP, and DKK1 that reflect bone and cartilage destruction in RA patients could be used to indicate disease activity and early joint affection.

Aim of the work: The aim of the present work was to present the frequency of ocular surface manifestations in rheumatoid arthritis (RA) patients and to determine its correlation with disease activity and duration. Patients and methods: This cross-sectional study included 70 RA patients. Disease activity score (DAS-28) was scored. All patients underwent complete ophthalmic evaluation including assessment of visual acuity. Ocular surface assessment by Rose Bengal (RB) stain, and Schirmer test (ST) was carried out. Results: Seventy patients (137 eyes) were studied; 3 eyes were excluded due to previous eye surgery. Patients were 67 women and 3 men with a mean age of 47.96 ± 10.8 years, and disease duration 9.19 ± 6.26 years. The mean DAS28 was 4.1 ± 0.77. Rheumatoid factor was positive in 39 (55.7%). Mean best-corrected visual acuity was 0.47 ± 0.33, ST was 10.6 ± 6.1 mm/5 min and RB score was 6.8 ± 3.6. 70% had ocular manifestations in the form of dry eye (54.7%), pterygium (16.1%), posterior blepharitis (5.8%), scleritis (1.3%), corneal thinning (13.9%), filamentary keratitis (8.7%) and maculopathy (11.7%). There was a significant correlation between ST and disease duration (r = −0.17, p = 0.04). Morning stiffness had a significant correlation with both ST (r = −0.2, p = 0.02) and RB (r = 0.22, p = 0.01) and remained significant on regression analysis. DAS-28 was not significantly associated with any of the ocular surface abnormalities. Conclusion: Ocular manifestations are significant in patients with RA. The most common ocular manifestation is dry eye. Ocular manifestations are significantly associated with the disease duration and should be expected regardless of disease activity in RA patients. Keywords: Rheumatoid arthritis, Ocular manifestations, DAS-28, Dry eye, Rose Bengal, Schirmer test

Objectives: The aim of the study is to examine the association between 14-3-3η protein levels in both serum and synovial fluid (SF) with various parameters in a longitudinal cohort of patients with...

The present work was conducted to estimate the prevalence of adult Behcet’s disease (BD) in adult Egyptian and to study the clinical pattern and influence of age at-onset and sex on disease phenotype. Also, we investigated the spectrum of presentation and frequencies along the north-to-south gradient of the country. The population-based, multicenter, cross-sectional study included 1526 adult BD patients from 26 specialized Egyptian rheumatology centers. Demographic, clinical, and therapeutic data are assessed for all patients. The mean age of patients was 35.7 ± 9.84 years, disease duration 6.58 ± 5.25 years, and age at onset 29.37 ± 8.6 years; 91 were juvenile-onset (JoBD). There were 1102 males and 424 females (M:F 2.6:1). Regarding co-morbidities, 19.92% were diabetic, and 26.05% were hypertensive. The mean body mass index was 27.57 ± 5.24 (43.1% overweight; 25.9% obese). The mean BD current activity form was 4.48 ± 4.28. Regarding the medications use, systemic steroid and colchicine were the most common drugs used (947 (90.2%) and 611 (82.7%), respectively). The overall estimated prevalence of BD in Egypt was 3.6/100,000 population being highest in the two main cities: Alexandria (15.27) and Cairo (8.72). Pathergy test was positive in 43.4%. 90.2% were receiving systemic steroids and 8.3%, biologics. Disease characteristics were comparable between JoBD and adult-onset BD cases. Central nervous system (CNS), deep venous thrombosis (DVT), and gastrointestinal (GIT) involvement were significantly higher in males (p = 0.01, p = 0.001, and p = 0.001 respectively) while joint affection (p = 0.001) and disease activity (p = 0.011) were increased in females. This study provides current prevalence of BD in Egypt; 3.6/100,000 with no remarkable north-to-south gradient. The sex influences the disease phenotype with the CNS, DVT, and GIT involvement are higher in males, while the joint affection and disease activity were increased in females. • The prevalence and phenotype of Behcet’s disease across Egypt is presented in a multicenter nationwide study. • The potential influence of the age at onset and sex on disease phenotype is highlightened. • A review of the literature worldwide is presented allowing comparisons with studies from other nations.

Objectives The aim of the present work was to explore the perspectives of Egyptian Rheumatology staff members as regards the coronavirus disease-19 (COVID-19) vaccine. Methods The survey is composed of 25 questions. Some questions were adapted from the global rheumatology alliance COVID-19 survey for patients. Results 187 rheumatology staff members across Egypt from 18 universities and authorizations actively participated with a valid response. The mean time needed to complete the survey was 17.7 ± 13 min. Participants were 159 (85%) females (F:M 5.7:1). One-third agreed that they will be vaccinated once available, 24.6% have already received at least one dose, 29.4% are unsure while 16% will not take it. Furthermore, 70.1% agreed that they will recommend it to the rheumatic diseases (RD) patients once available, 24.1% are not sure while 5.9% will not recommend it. RD priority to be vaccinated against COVID-19 in descending order include SLE (82.9%), RA (55.1%), vasculitis (51.3%), systemic sclerosis (39.6%), MCTD (31.6%), Behcet's disease (28.3%). The most common drugs to be avoided before vaccination included biologics (71.7%), DMARDs (44.4%), biosimilars (26.7%), IVIg (17.1%) and NSAIDs (9.1%). Conclusions The results of the study and specifically the low rate of acceptability are alarming to Egyptian health authorities and should stir further interventions to reduce the levels of vaccine hesitancy. As rheumatic disease patients in Egypt were not systematically provided with the vaccine till present, making the vaccine available could as well enhance vaccine acceptance. Further studies to investigate any possible side effects, on a large scale of RD patients are warranted. Supplementary Information The online version contains supplementary material available at 10.1007/s00296-021-04941-0.

Background Behcet's disease (BD) is a chronic multisystem variable vessel vasculitis. Disease damage is irreversible and permanent. Validated tools evaluating damage are limited. Enhancements in the clinical treatment of vasculitis will take place from the development of refined and exclusive indices for individual vasculitic syndromes including BD and attempting their international validation. Objectives This aim was to develop and validate a simple BD Damage Index (BDI). Methods This was a nationwide study including 1252 BD patients. The work consisted of 3 stages. Stage 1: items generation for score content. Stage 2: items selection for the draft score was performed by an expert rheumatologist. Stage 3: the content validity of the draft score was assessed and BDI, Vasculitis Damage Index (VDI), Antineutrophil cytoplasmic antibody-associated Vasculitis Index of Damage (AVID) and Combined Damage Assessment Index (CDAI) were calculated and compared. Results The mean age of the BD patients was 36.1 ± 9.9 years. Stages 1 and 2 resulted in a BDI instrument containing 73 items with a maximum score of 100. Stage 3, the VDI, CDAI, AVID, and BDI were 2.9 ± 2.2, 3.1 ± 2.3, 3.1 ± 2.3 and 5.1 ± 2.9, respectively. High correlations (r = .9) between comparable damage scores assured acceptable concurrent validity. Conclusion The proposed BDI represents a new robust and potentially useful tool when dealing with BD chronic status.

Background Cardiovascular disease (CVD) is a leading cause of early mortality among young adults with cerebral palsy. While low physical activity in childhood has been hypothesized as a potential contributor to increased CVD risk in early adulthood, little is known about timing of vascular disease progression and the presence of subclinical atherosclerosis has not been extensively evaluated in children with cerebral palsy. Objective The aim of this study was to determine if measures of vascular structure and function are different between children and adolescents with and without cerebral palsy. Methods In this cross-sectional study, we measured carotid intima-media thickness (CIMT), and brachial artery flow-mediated dilation (FMD) of children with and without cerebral palsy. Group means for CIMT and brachial artery FMD absolute (FMDA) and percent of relative change (FMDR%) were compared using Wilcoxon rank-sum tests. Results A total of 26 children and adolescents with cerebral palsy (46.1% girls, mean age = 15.0 ± 2.0 years) and 19 controls (68.4% girls, mean age = 13.3 ± 2.6 years) participated. Children with cerebral palsy had significantly greater average CIMT (0.43 ± 0.02 mm) than children without cerebral palsy (0.41 ± 0.01 mm, p = 0.04), and lower FMDA (0.16 ± 0.15 mm vs. 0.29 ± 0.20 mm; respectively, p = 0.03). Conclusions Children and adolescents with cerebral palsy may exhibit impairments in vascular structure and function which represent an increased risk of premature atherosclerosis compared to children without cerebral palsy. Additional research to identify risk factors specific to children with cerebral palsy that would support the development of effective screening processes for early identification would enable clinicians to implement targeted preventive strategies.

During the coronavirus disease-2019 (COVID-19) pandemic there were several barriers to treatment access and medication adherence in rheumatoid arthritis (RA) patients. There is no information regarding the RA patient health status in Egypt during the COVID-19. Thus,the aim of this work was to study the impact of the pandemic on RA patients through a patient-reported questionnaire and to determine the influence of gender, geographic regions. This multi-centre study initiated by the Egyptian College of Rheumatology (ECR) was conducted on 1037 RA patients attending rheumatology clinics from 10 governorates. The questionnaire provided covered socio-demographic data, health/disease status, information/knowledge about COVID-19 and medical/family history of the infection. Patients mean age was 44.2 ± 12.3 years;855 females and 182 males; 539(52%) from rural and 497(48%) from urban areas. 41.8% reported a striking difficulty to obtain hydroxychloroquine during the pandemic. The majority (70%) considered maintaining a regular visit to the rheumatologist in addition to remote contact mainly by phone (44.4%) or via WhatsApp (33.1%), in particular among male and urban patients. Urban patients were more likely to be infected by COVID-19 (12.9% vs 6.2%; p

Study Objectives Machine learning (ML) may provide insights into the underlying sleep stages of accelerometer-assessed sleep duration. We examined associations between ML-sleep patterns and behavior problems among preschool children. Methods Children from the CHILD Cohort Edmonton site with actigraphy and behavior data at 3-years (n = 330) and 5-years (n = 304) were included. Parent-reported behavior problems were assessed by the Child Behavior Checklist. The Hidden Markov Model (HMM) classification method was used for ML analysis of the accelerometer sleep period. The average time each participant spent in each HMM-derived sleep state was expressed in hours per day. We analyzed associations between sleep and behavior problems stratified by children with and without sleep-disordered breathing (SDB). Results Four hidden sleep states were identified at 3 years and six hidden sleep states at 5 years using HMM. The first sleep state identified for both ages (HMM-0) had zero counts (no movement). The remaining hidden states were merged together (HMM-mov). Children spent an average of 8.2 ± 1.2 h/day in HMM-0 and 2.6 ± 0.8 h/day in HMM-mov at 3 years. At age 5, children spent an average of 8.2 ± 0.9 h/day in HMM-0 and 1.9 ± 0.7 h/day in HMM-mov. Among SDB children, each hour in HMM-0 was associated with 0.79-point reduced externalizing behavior problems (95% CI −1.4, −0.12; p < 0.05), and a 1.27-point lower internalizing behavior problems (95% CI −2.02, −0.53; p < 0.01). Conclusions ML-sleep states were not associated with behavior problems in the general population of children. Children with SDB who had greater sleep duration without movement had lower behavioral problems. The ML-sleep states require validation with polysomnography.

Introduction: Eosinophilic fasciitis (EF) is a rare scleroderma-like disease, characterized by acute onset of symmetrical swelling, induration and thickness of the skin. Laboratory findings include peripheral eosinophilia, elevated inflammatory markers and increased gammaglobulemia. Although, the diagnosis is mainly clinically, a full thickness skin/fascia/muscle biopsy remains the gold standard for the definite diagnosis. Unlike systemic sclerosis, visceral involvement, such as pulmonary affection, is rare in EF. Although, few cases of systemic involvement in adult EF have been presented, we report a rare case of juvenile EF associated with pulmonary involvement. Case report: A 12-years old boy presented with symmetrical skin thickening of bilateral upper extremity, back, trunk, neck, face, and scalp of 2-weeks duration. The laboratory tests revealed marked peripheral eosinophilia 61.9% (normal 1–3%) and elevation of the acute phase reactants (erythrocyte sedimentation rate 40 mm/1st hour and C-reactive protein 15 mg/dL). Lung imaging study showed bilateral extensive pulmonary nodules. A full thickness skin/fascia/muscle biopsy revealed an inflammatory infiltration, fibrosis in the fascial and muscle tissues. These findings were concordant with EF. An initial treatment of intravenous (IV) methylprednisolone 30 mg/kg/day for 3 successive days was started followed by oral steroid (2 mg/kg/day) plus methotrexate (20 mg/week). Follow up revealed complete improvement in the skin thickening, pulmonary affection and systemic inflammation. Conclusion: To the best of our knowledge this is the first reported co-existence of pediatric eosinophilic fasciitis with pulmonary affection. Systemic involvement should be screened in EF cases, as it may have consequences in the management and outcome. Keywords: Eosinophilic fasciitis, Eosinophilia, Skin thickness, Pulmonary affection

Rheumatoid arthritis (RA) is an inflammatory autoimmune polyarthritis with progressive destruction of the synovial joints associated with systemic manifestations. RA is characterized by infiltration of the synovial joints with inflammatory immune cells with premature immunosenescence. Shorter telomere length in the peripheral blood cells and increase in the oxidative stress have been detected in patients with RA. The aim of the present study was to study the association of markers of telomere shortening and oxidative stress with RA disease activity. Sixty-one RA patients and 15 healthy controls were enrolled in the study. Demographic data, clinical examination, and disease activity status were evaluated for the RA patients. Serum levels of chitinase and NAG (telomere markers) were determined by biochemical reactions using colloidal chitin and NAG as substrates, respectively. Nitric oxide and superoxide dismutase (oxidative stress markers) were determined colometrically and spectrophotometrically, respectively, in the sera of RA patients and controls. Results were correlated with disease activity. Indices of telomere shortening and oxidative markers were significantly higher in RA patients compared to controls. These indices were correlated with signs of disease activity (including number of swollen and tender joints, DAS-28, and inflammatory markers). Rheumatoid arthritis is a disease in which markers of telomere shortening and elevated oxidant stress correlate with disease activity.

Background: Red blood cell distribution width (RDW) is a biomarker for cardiovascular disease(CVD). RDW is associated with sedentary behavior (SB) and physical activity (PA) in adults.To date, no study has evaluated this association in children. The purpose of this study was to evaluate the association between RDW and SB and PA levels of children and adolescents. Methods: This observational study included data from participants aged 12-20 years in the 2003–2006 National Health and Nutrition Examination Survey (NHANES). SB and PA were measured using accelerometers. Activity levels were classified into intensity categories. Sex specific multivariable regression analyses (adjusted for covariates) were used to explore the associations between SB, PA and RDW. Results: The study included 2143 children and adolescents (1080 boys and 1063 girls). In the fully adjusted regression model for boys, SB was positively associated with RDW (β =0.116,P=0.004) while moderate PA was negatively associated with RDW (β =-0.082, P=0.048). In girls, there were no significant associations between activity levels and RDW. Conclusion: This study provides preliminary evidence of the association between SB, moderate intensity PA and RDW in boys, but not in girls. Further research to determine the mechanisms associated with this relationship and underlying sex differences is warranted.

Background Attempts are ongoing to unveil unresolved queries about anti-double-stranded deoxyribonucleic acid (anti-dsDNA), their precise pathogenic effects and to what extent blocking them would be a useful therapeutic goal. Objectives The aim of the present study was to determine the anti-dsDNA antibodies titre in systemic lupus erythematosus (SLE) patients and investigate their relation to the disease characteristics, activity, damage and antiphospholipid autoantibodies (aPL). Methods Seventy female SLE patients and 35 age- and sex-matched controls were included. The anti-dsDNA level and aPL were measured. Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) and Systemic Lupus International Collaborative Clinics/American College of Rheumatology Damage Index (SLICC/ACR-DI) were assessed. Results The mean age of the patients was 27.5 ± 5.1 years, disease duration 7.7 ± 5.4 years, and SLEDAI and SLICC/ACR-DI scores were 6.8 ± 8.04 and 1.2 ± 1.3, respectively. Anti-dsDNA was positive in 61.4% of the patients and the titre (133.2 ± 100.5 IU/ml) was significantly higher compared to controls (22.03 ± 17.2 IU/ml) ( p Conclusion Anti-dsDNA is remarkably increased in SLE patients especially those with musculoskeletal manifestations and aPL. A protective role seems likely in those with neuropsychiatric manifestations and those receiving CYC and may form a shield against disease tissue damage.

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 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.

Smoking is an established risk factor for the development and severity of rheumatoid arthritis (RA) with prominent production of cytokines. The aim of the work was to study the possible effect of secondhand exposure on disease activity in non-smoking female RA patients. This cross-sectional study include 100 women with RA attending the rheumatology outpatient clinic and were grouped according to the non-smoking status into those not exposed to smoking and those considered secondhand smokers (SHS). Disease activity score in 28 joints (DAS28) was calculated and the patients' global assessment (PGA) score were assessed. The mean age of the patients was 45.2 ± 12.1 years and disease duration was 8.3 ± 6 years. Their DAS28 score was 4.3 ± 0.93 with a PGA score of 1.47 ± 1.36. Forty-seven of the patients were SHS and 53 were non-exposed. The secondhand smokers were significantly younger (41.6 ± 11.7 years) than the non-smokers (48.3 ± 11.6 years) (p = 0.005), and the DAS28 was significantly higher (4.6 ± 0.84 versus 4.1 ± 0.97; p = 0.02) compared to non-smokers. The disease duration and medications received were comparable. There is evidence pointing to the important role of secondhand smoking on disease activity in RA female patients. Studying the effect of secondhand smoking in view of the cytokine milieu could help confirm the relation to the disease pathogenesis. Taking into consideration the risk of cardiovascular disease and interplay with other potential factors should be well thought of. It is essential to draw patients' attention to the expected hazardous effect of passive smoking.

Context: Subjects with spinal cord injury (SCI) may experience a low quality of life due to struggling with sensory and motor impairments and various underlying illnesses. Objectives: The present study was conducted to investigate the health-related quality of life (HRQoL) of Iranian patients with SCI. Methods: In the present study, a search was done in domestic and international databases including MagIran, Iran Medex, SID, Google Scholar, Web of Science, PubMed, and Scopus using specific keywords (“Quality of Life” OR “Health-Related Quality of Life” OR “Life Style” OR “QoL” OR “HRQoL”) AND (“Shortform questionnaire 36” OR “SF-36”) AND (“spinal cord injury” OR “SCI”) AND “Iran”, without time limitation. Observational studies evaluating HRQoL in patients with SCI were included. The HRQOL was assessed using the 36-item Short Form questionnaire (SF-36). The SF-36 has two main domains of Physical Component summary (PCS), and Mental Component summary (MCS). Based on the heterogeneity of the data, the data were analyzed by fixed and random effects models. Stata 14 was used to analyze the collected data. Results: Eight studies with a sample size of 848 SCI patients were included in the analysis. The mean scores of PCS and MCS of the subjects with SCI were 55.47% (95% confidence interval [CI]: 42.50 - 68.43) and 67.91% (95%CI: 59.18 - 76.64), respectively. The findings showed that the highest and the lowest scores of the subscales of HRQoL in both paraplegia and tetraplegia groups were related to physical functioning and social functioning, respectively. The mean score of eight subscales of HRQoL (except for social functioning) was higher in paraplegic patients than in those with tetraplegia. Conclusions: Individuals with SCI had half of the total score of the physical dimension of HRQoL. Providing strategies for improving the HRQoL of Iranian people with SCI, especially in the physical aspect, seems necessary.

Objective: Individuals with rheumatoid arthritis (RA) have increased risk of cardiovascular disease (CVD). Lifestyle factors such as prolonged sedentary behavior (SB) and reduced physical activity ...

Objective The aim of this study was to present the epidemiology, clinical manifestations and treatment pattern of systemic lupus erythematosus (SLE) in Egyptian patients over the country and compare the findings to large cohorts worldwide. Objectives were extended to focus on the age at onset and gender driven influence on the disease characteristics. Patients and method This population-based, multicenter, cross-sectional study included 3661 adult SLE patients from Egyptian rheumatology departments across the nation. Demographic, clinical, and therapeutic data were assessed for all patients. Results The study included 3661 patients; 3296 females and 365 males (9.03:1) and the median age was 30 years (17–79 years), disease duration 4 years (0–75 years) while the median age at disease onset was 25 years (4–75 years). The overall estimated prevalence of adult SLE in Egypt was 6.1/100,000 population (1.2/100,000 males and 11.3/100,000 females).There were 316 (8.6%) juvenile-onset (Jo-SLE) and 3345 adult-onset (Ao-SLE). Age at onset was highest in South and lowest in Cairo (p Conclusion SLE in Egypt had a wide variety of clinical and immunological manifestations, with some similarities with that in other nations and differences within the same country. The clinical characteristics, autoantibodies and comorbidities are comparable between Ao-SLE and Jo-SLE. The frequency of various clinical and immunological manifestations varied between gender. Additional studies are needed to determine the underlying factors contributing to gender and age of onset differences.

Background:Osteoarthritis (OA) is the most common joint disease that results in patient’s morbidity and disabilities. There is strong evidence that OA is a significant risk factor for cardiovascular disease (CVD). Red cell distribution width (RDW) blood test is a measure of the variation in red blood cell volume and size. Elevated RDW has recently been found to correlate with CVD risk in patients with and without heart disease and autoimmune diseases. RDW may be a marker for factors driving CVS risk.Objectives:: To investigate whether RDW can serve as a potential parameter for indicating cardiovascular risk in OA patients.Methods:A subsample of 819 OA patients was extracted from 2003-2006 National Health & Nutrition Examination Survey in a cross-sectional study. 63.7% of them were females. Their mean age was 66.4 ± 14.1 yrs. Demographic, medical data, inflammatory markers & lipid panel were obtained. Only patients with Haemoglobin>12 mg/dl were included. Functional limitations were assessed using a physical function questionnaire.Results:Elevated levels of RDW were associated with CVD risk factors in OA patients. 532 (65.8%) OA patients had functional limitations, while 78 (9.5%) and 63 (7.6%) known to have heart attacks or stroke ever. Mean RDW was 12.9±1.1fL. There was a positive significant correlation between RDW & CVD risk factors including body mass index (r=0.17, pConclusion:In addition to known CVD risk in OA patients, elevated RDW levels should prompt physicians to aggressively screen and treat their patients for modifiable CVS risk factors, in addition to OA.Disclosure of Interests:None declared

Objectives. This study aims to correlate subclinical echocardiographic features with the clinical, laboratory, and therapeutic profiles of the patients to characterize risks for systemic lupus erythematosus (SLE) cardiac diseases. Methods. The study included 59 SLE patients. Demographic data, disease characteristics, and current therapies were recorded, and the anthropometric measurements and routine laboratory tests were performed. The disease activity by the SLE Disease Activity Index-2K (SLEDAI2K) and the presence of metabolic syndrome (MetS) were assessed. Two-dimensional echocardiography was performed. Results. The mean age of the patients was 31.3 ± 10.5 years, and the disease duration was 5.18 ± 4.1 years. 86.4% of the patients were females. Cardiac presentations by echocardiography were mainly mitral regurgitation (33.9%), tricuspid regurgitation (32.2%), mitral thickening (18.6%), aortic thickening (13.6%), pericardial effusion (13.6%), and pulmonary hypertension (8.5%) in order of frequency. The frequency of different echocardiographic findings with respect to other clinical phenotypes showed peaks with renal disease, MetS, and leukopenia. Components of MetS (triglycerides, high systolic blood pressure) and avascular necrosis were significant predictors for pericardial diseases (OR=1.011 CI 95% 1-1.022, p=0.046, OR=1.157 CI 95% 1.025-1.307, p=0.018, and OR=74.78 CI 95% 2.52-2215.76, p=0.013, respectively), and it is likely that hydroxychloroquine was protective against them. Age of the patients was a significant predictor for tricuspid regurgitation (OR=1.063 CI 95% 1.004-1.126, p=0.036). Mucosal ulcers were negative predictors for mitral thickening and regurgitation (OR=0.2 CI 95% 0.059-0.673, p=0.009). The use of corticosteroids appeared to protect against a number of valve lesions especially tricuspid regurgitation (OR=0.299 CI 95% 0.088-1.019, p=0.054). Conclusion. This study highlighted different echocardiographic features and identified clinical predictors of different cardiac pathologies aiming to determine patients at risk and improve the prognosis of SLE cardiac diseases.

Sleep disorders are significant problems in patients with rheumatoid arthritis (RA) and are associated with poor quality of life. Irisin is myokine which may have anti-inflammatory and energy regulatory roles. This study assessed the association of serum irisin levels with the quality of sleep and disease activity in RA patients.In sum, 58 RA patients and 30 matched healthy controls were included. Disease activity score in 28 joints (DAS28-ESR) and the patients' global score were calculated. RA patients were grouped according to the Pittsburgh Sleep Quality Index score (PSQI) into good-sleepers (group 1) defined as a PQSI score≤5 and poor sleepers (group 2) with a PSQI 5. Serum irisin levels were measured for both patients and controls by commercially available enzyme-linked immunosorbent assay kits.Poor sleep quality was found in 26 (45%) of the RA patients. Irisin levels were significantly lower in RA patients with poor sleep compared to those with good sleep and healthy controls (p 0.001). Serum irisin levels correlated inversely with disease duration, morning stiffness duration, DAS28-ESR, global score, and total PSQI score (r = -0.722 to -0.263p values≤0.001-0.04) indicating a possible anti-inflammatory role of irisin in RA patients. The analysis employed Student's t-test, ANOVA, and Pearson correlation.Irisin levels were decreased in RA patients with poor sleep quality compared to RA patients with good sleep quality and healthy controls, indicating a possible association of decreased serum irisin with sleep impairment in RA patients.

Background Sympathetic skin response (SSR) is a technique to assess the sympathetic cholinergic pathways. Sympathetic dysfunction may participate in the development of pain, which is the major complaint in patients with systemic sclerosis (SSc) and rheumatoid arthritis (RA). Objectives In this study, we aimed to assess the autonomic dysfunction in patients with (SSc) and (RA) using SSR as a simple neurophysiologic test. Methods Palmar SSR to median nerve electrical stimulation was recorded in 21 patients with SSc, 39 patients with RA, and in 60 healthy age and sex-matched control subjects. Results Palmar SSR to median nerve stimulation (of SSc patients and RA patients) shows significantly delayed latency and reduced amplitude in comparison to the control group. SSR of SSc patients has significantly delayed latency and reduced amplitude when compared to RA patients. Moreover, six SSc patients have delayed SSR in spite of the absence of manifestations of polyneuropathy. Conclusions Patients with SSc and RA have features of autonomic dysfunction with more affection of SSc patients.

Background Juvenile idiopathic arthritis (JIA) affects wrist and hand joints leading to decrease hand function and patients’ daily living activities. The assessment of hand grip strength (HGS) in children and adolescents with JIA is of major importance, and the association of HGS with JIA disease activity, disability and quality of life has not been explored. The primary objective of this study was to evaluate hand grip strength (HGS) in children and adolescents with Juvenile Idiopathic Arthritis (JIA) compared to matched healthy peers. The secondary objective was to explore the relationship between HGS and JIA disease activity, disability, and quality of life. Methods This study involved 23 patients with JIA and 46 age and sex matched healthy controls. Hand held dynamometer was used to evaluate HGS for all study participants. Anthropometric parameters for all study participants were measured. Disease activity, physical function, and quality of life were assessed for the JIA group using juvenile arthritis disease activity score (JADAS-27), juvenile arthritis functionality scale (JAFS), and pediatric quality of life inventory (PedsQL) respectively. Laboratory marker of inflammation, erythrocyte sedimentation rate (ESR), and plain radiography of hands were performed for all patients. Results Hand grip strength of children and adolescents with JIA was significantly weaker compared to matched controls (p

The study aims to evaluate the presence of metabolic syndrome (MetS) among Egyptian systemic lupus erythematosus (SLE) patients. Additionally, we intended to determine the association of MetS with disease activity, clinical and laboratory features. This cross-sectional study included adult SLE patients diagnosed and followed in rheumatology outpatient clinics. The demographic data, clinical and laboratory parameters were assessed for all patients. Disease activity was measured using SLE Disease Activity Index (SLEDAI-2k). Patients were diagnosed to have MetS according to The National Cholesterol Education Program Adult Treatment Panel III (NCEP). The characteristics of SLE patients and their disease status were compared between those with and without MetS. A total of 74 SLE patients were included; mean age was 32.1 ± 10.9 years and 87.8% were females. Twenty-five (33.8%) SLE patients had neuropsychiatric lupus (NPSLE), with headache the most common manifestation. MetS was diagnosed in 45.9% of the SLE group. Patients with MetS had significantly older age, delayed age of diagnosis and higher SLEDAI-2k scores (p = 0.015, p = 0.014 and p = 0.019, respectively) compared to those without MetS. NPSLE was significantly correlated with MetS (r = 0.32, p = 0.006) and in particular with higher central obesity (r = 0.43, p

The author wishes to correct the record and clarify that in the original version of this article in the Discussion section under "Prevalence over the country governorates" inadvertently presented incorrect data cited in the reference [30].

Background: Osteoporosis (OP) is defined by low bone mass and microstructural deterioration. It is an escalating public health problem due to increase life expectancy and the resulting bone fractures represent a significant burden for both the individual and the society in terms of morbidity, mortality and cost. Osteoporosis, a multifactorial disease, results from the interaction between genetic and environmental risk factors. Currently, the data available regarding OP epidemiology and predisposing risk factors differ greatly between regions and within population ethnicities. Proper estimation of the epidemiology of OP and its health related outcomes can help identity those at risk and permit prophylactic treatment before its occurrence. The main barrier towards disease prevention strategies is the impaired awareness of the disease and its risk. Enhanced understanding of the OP disease may influence personal behaviors and reduce its prevalence. Objectives: This review was undertaken to wrap-up and throw-light on the published literatures related to the epidemiology of osteoporosis in the Middle East and North Africa (MENA) region, and expose the extent of awareness in the corresponding populations. Describing and discussing key points on the current state of knowledge on these hot issues are well thought-out. Conclusion: Osteoporosis prevalence is variable among MENA populations. Limited reports regarding the established prevalence of osteoporotic fractures among those populations and therefore, lack of guidelines for prevention and management were noticed. In order to improve bone health, preventive measures against OP should be considered. Increase OP awareness and preventive practices in the societies as part of the prophylactic strategy need to be initiated.

The objective of this study is to assess toll-like receptor-9 (TLR9) expression in CD3-positive T lymphocytes and CD19-positive B lymphocytes in systemic sclerosis (SSc) patients and to study their relation to the extent of skin fibrosis, disease characteristics, and severity as well as the functional status. Fifty-five female SSc patients and 30 matched controls were included. Skin thickness was scored according to the modified Rodnan skin score (mRss). The severity of major organ involvement was assessed using the Medsger severity score (MSS). Scleroderma health assessment questionnaire (SHAQ) was measured to evaluate patients’ functional status. Expression of TLR9 in CD3-positive T lymphocytes and CD19-positive B lymphocytes was studied using flow cytometry. The mean age of the patients was 40.5 ± 9.1 years, and their disease duration was 6.7 ± 3.3 years. There were 21 (38.2%) with diffuse (dcSSc) and 34 (61.8%) with limited cutaneous (lcSSc) subtypes. There was a significant increase in the expression of TLR9/CD3 and TLR9/CD19 in the SSc patients (44.9 ± 18.1 and 24.1 ± 9.6) compared to that in the control (1.4 ± 0.97 and 1.3 ± 0.94; p

Background Systemic lupus erythematosus (SLE) is chronic autoimmune inflammatory disease, affecting women in their reproductive period [1]. SLE patients are at increased risk for multiple comorbidities including cardiovascular diseases, obesity and metabolic syndrome (MetS) [2]. These comorbidities may be driven by related disease factors or medications exposure. Metabolic syndrome is characterized by presence of hypertension, hyperglycemia, dyslipidaemia, and abdominal obesity [3]. Objectives The aim of this study was to detect the prevalence of MetS and obesity in SLE patients. Also, to evaluate the association of demographic factors, disease activity, medications used and patients9 body mass index (BMI) with presence of MetS. Methods A total of 85 patients with SLE meeting four or more of the American College of Rheumatology (ACR) classification criteria for SLE were enrolled in this cross-sectional study. Patients9 clinical and laboratory data and therapeutic exposures were collected. Disease activity was assessed using SLE Disease Activity Index (SLEDAI). Presence of MetS (according to the National Cholesterol Education Program Adult Treatment Panel III) was considered when patients met 3 or more of the definition criteria [4]. Anthropometric measurements and BMI were calculated. Obesity defined as BMI more than 30 kg/m 2 . Results Overall, 42.4% of our SLE patients had MetS. Patients with MetS were older age ( P =0.011), had significant delayed age at diagnosis ( P =0.008). Significant differences were noted between patients with and without MetS regarding the MetS parameters ( P ≤0.002). 29.4% of our SLE patients were obese. BMI was significantly difference between patients with and without MetS ( P =0.001). No significant differences were observed regarding SLE disease duration, therapeutic variables, or mean SLEDAI score. However, in SLEDAI score >10 there was significant association with MetS towards higher disease activity ( P =0.050). Regarding laboratory features, only serum creatinine showed significant elevation ( P =0.005) in patients with MetS. Conclusions Metabolic syndrome is frequent among our SLE patients. Metabolic syndrome is associated with delayed age at diagnosis, obesity and serum creatinine. Within SLE patients with high disease activity, there is correlation between MetS and disease activity. Targeted metabolic syndrome management can alter disease staus in SLE. References Hochberg, M.C., Systemic lupus erythematosus. Rheumatic diseases clinics of North America, 1990. 16(3): p. 617-639. Manzi, S., et al., Age-specific incidence rates of myocardial infarction and angina in women with systemic lupus erythematosus: comparison with the Framingham Study. American journal of epidemiology, 1997. 145(5): p. 408-415. Alberti, K.G., P. Zimmet, and J. Shaw, The metabolic syndrome – a new worldwide definition. Lancet, 2005. 366(9491): p. 1059-62. Alberti, K., P. Zimmet, and J. Shaw, Metabolic syndrome – a new world-wide definition. A consensus statement from the international diabetes federation. Diabetic Medicine, 2006. 23(5): p. 469-480. Disclosure of Interest None declared

Background Juvenile idiopathic arthritis (JIA) is a chronic inflammatory disease resulting in joints arthritis and deformities in the hands and fingers. This leads to decrease of joint mobility and strength of the hands which can lead to physical and functional hand disability (1) and subsequent reduction in daily living activities (2). Despite the presence of indices to assess the hand function, grip strength assessment remains the strong predictor of both hand and upper limb function (3). Hand grip strength (HGS) can be measured quantitatively using a hand dynamometer (4). Although the role of HGS in evaluating hand function in JIA has been studied (5), the use of HGS as a predictor of disease activity and disability in JIA has not been explored. Objectives The aim of the study was to evaluate the HGS in JIA patients using electronic hand dynamometer and to assess its relationship with JIA disease activity, disability and quality of life. Methods Twenty-three patients with JIA (14 girls and 9 boys) diagnosed according to International League of Associations for Rheumatology (ILAR) classification criteria and 59 healthy children were enrolled in the study. The disease activity was assessed using Juvenile Disease Activity Score (JDAS-27). Juvenile Arthritis Functionality Scale (JAFS) and Pediatric QOL Inventory (PedsQL) were used to assess physical function and quality of life respectively. HGS was tested 3 times in each hand of every patient and control using electronic hand dynamometer and the mean value was considered. Results Patients with JIA showed low HGS in one or both hands compared with healthy children ( p =0.001). Grip strength showed a significant inverse correlation with JDAS-27 ( p =0.003, r value = -0.591) and with JAFS (total and after exclusion of hand component) ( p =0.001 and 0.002, r value = -0.650 and -0.608 respectively). Significant correlations were observed between HGS and PedsQL ( p =0.036). Conclusions Patients with JIA have reduced HGS when compared to healthy children. HGS may be considered as a non invasive predictor of JIA disease activity and disability. Pediatric quality of life is adversely affected with decrease in HGS. HGS testing might be used a simple screening for disease activity in patients with JIA. References 1. Filocamo G, Sztajnbok F, Cespedes-Cruz A, Magni-Manzoni S, Pistorio A, Viola S, Ruperto N, Buoncompagni A, Loy A, Martini A, Ravelli A (2007) Development and validation of a new short and simple measure of physical function for juvenile idiopathic arthritis. Arthritis Rheum 57 (6) Article: 913-20 2. Varni JW, Seid M, Kurtin PS (2001) PedsQL 4.0: reliability and validity of the Pediatric Quality of Life Inventory version 4.0 generic core scales in healthy and patient populations. Med Care 39 (8) Article: 800-12 3. Dunn W (1993) Grip strength of children aged 3 to 7 years using a modified sphygmomanometer: comparison of typical children and children with rheumatic disorders. Am J Occup Ther 47 (5) Article: 421-8 4. Sheehy C, Gaffney K, Mukhtyar C (2013) Standardized grip strength as an outcome measure in early rheumatoid arthritis. Scand J Rheumatol 5. Lindehammar H (2003) Hand strength in juvenile chronic arthritis: a two-year follow-up. Acta Paediatr 92 (11) Article: 1291-6 Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.1858