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The demographic factors, the socioeconomic status and the ethnicity of populations are important players that determine the incidence, the prevalence and the spectrum of systemic lupus erythematosus (SLE) clinical presentations in different populations. Therefore, the purpose of the present research was to investigate the possible association between the Ikaros family zinc finger 1 gene (IKZF1) rs4132601 and rs11978267 single nucleotide polymorphisms (SNPs) and SLE susceptibility and clinical presentations including lupus nephritis (LN) among Egyptian paediatric patients. After DNA extraction from Ethylenediaminetetraacetic acid (EDTA) blood samples for 104 paediatric SLE (pSLE) patients and 286 healthy controls, the investigated SNPs (IKZF1 rs4132601 and rs11978267) were genotyped using TaqMan‐Real‐time Polymerase chain reaction (PCR). The G allele, GG and GT genotypes of IKZF1 rs4132601 were associated with pSLE (pc<.001, OR 2.97, 3.2 and 2.25, respectively). The GG and GA haplotype were more frequent in pSLE patients than other haplotypes (pc<.001, OR 3.47 and pc =.004, OR = 2.8, respectively). The studied SNPs have no impact on the distinctive features of pSLE. The rs4132601 TG genotype was significantly associated with proliferative LN (pc =.03) The IKZF1 rs4132601 can be considered a risk factor for SLE in the cohort of Egyptian children. The TG genotype of the IKZF1 rs4132601 may predispose to proliferative LN. [ABSTRACT FROM AUTHOR]

Background: Ankylosing spondylitis (AS) is a chronic, inflammatory disease that primarily affects the axial spine. Involvement of the sacroiliac joint is a hallmark of AS. AS is also associated with an increased risk of CVD (cardiovascular disease) which constitutes one of the main causes of death in these patients. AS is a complex immune-mediated condition whose pathogenesis is not fully understood. It is assumed that Reactive oxygen. species is a major event in inflamed tissues leading to AS. Albumin acts as an antioxidant by removing oxidative stress products. Ischemia modified albumin (IMA) had gained wide acceptance as a systemic biomarker of oxidative stress. The aim of study to evaluate the serum level of ischemiamodified albumin in patients with ankylosing spondylitis and to correlate the ischemia-modified albumin serum level with disease activity indices, and carotid artery intima media thickness. Objective: The aim of the study was to evaluate the serum level of IMA in patients with AS and to correlate the IMA serum level with disease activity indices, and Carotid intima media thickness. Subjects and Methods: This cross-sectional study included 40 AS patients and 40 matched healthy controls. In AS patients, the activity parameters (BASDAI score, ASDAS-ESR, ASDAS-CRP, ESR and serum CRP levels) as well as BASFI score and BASMI score and treatment regimen were assessed. In addition, the cIMT and was measured for AS patients and controls. Ischemia-modified albumin (IMA) was also measured. Results: Patients with AS had significantly higher serum IMA level than controls. The ROC curve analysis revealed a strong ability of the IMA to discriminate the AS patients from controls. In AS patients, the serum IMA was significantly correlated with the cIMT and was higher in AS patients with plaques than those without plaques. Conclusion: our study has shown that the serum level of IMA was significantly higher in patients with AS than matched healthy controls. MA serum level was significantly correlated with AS activity and severity. Serum IMA can be used as a marker for subclinical atherosclerosis in AS. [ABSTRACT FROM AUTHOR]

Higher prevalence of fibromyalgia (FM) among chronic hepatitis-C virus (HCV) suspects virally-mediated pathway. To determine the frequency and clinical characteristics of FM in Egyptian patients with chronic HCV and to evaluate its relationship to viral load. 150 Egyptian patients with chronic HCV and 150 controls were included. Liver function tests, thyroid stimulating hormone (TSH), HCV antibodies, HCV-ribonucleic acid (RNA) and abdominal ultrasonography were done. FM was diagnosed according to 2010 American College of Rheumatology (ACR) classification criteria. The mean age of the patients was 54.1 ± 8.4 years and they were 94 females: 56 males; and the mean duration of HCV was 6.9 ± 3.2 years. Fatigue, arthralgia, arthritis, morning stiffness, weakness, sicca-like symptoms, depression, cognitive, sleep disturbances and irritable bowel syndrome (IBS) were significantly more frequent in HCV patients. Twenty six patients with HCV (17.3%) and 4 (2.7%) of the controls had FM. HCV patients with FM had significantly higher widespread pain index, symptom severity score, visual analogue scale (VAS), tender points count and stiffness than controls with FM. HCV patients with FM had significant higher VAS score, fatigue and depression than without FM. Rates of hematological features, autoantibodies, liver enzymes, abdominal US findings, patients with viral load > 800,000 IU/L were comparable. There is increased rate of fibromyalgia in chronic HCV patients and is associated with higher pain intensity, tender point count, symptom severity and fatigue. FM in patients with HCV infection is not related to viral load of HCV. [ABSTRACT FROM AUTHOR]

Background: Behçet'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. [ABSTRACT FROM AUTHOR]

Background: The prevalence of SLE and the spectrum of clinical manifestations vary widely in different races and geographical populations. Objective: To investigate the possible role of ARID5B rs10821936 and rs10994982 polymorphism as a risk factor for the development of SLE in children (jSLE) and to evaluate their role in relation to clinical manifestations especially lupus nephritis (LN). Methods: DNA extraction and Real-time PCR genotyping of ARID5B rs10821936 and rs10994982 were done for 104 jSLE and 282 healthy controls. Results: The C allele and C containing genotypes (CC, CT and CC+CT) of ARID5B rs10821936 were higher in children with SLE (p = 0.009, OR = 1.56, 0.037, OR = 2.35, 0.016, OR = 1.81 and 0.008 OR = 1.88 respectively). ARID5B rs10994982 alleles, genotypes and haplotypes are not associated with jSLE (p > 0.05). The ARID5B rs10821936 and rs10994982 genotypes showed non-significant associations with LN, proliferative versus non proliferative and biopsy grades (p > 0.05). Conclusion: ARID5B rs10821936 SNP may be a susceptibility risk factor for juvenile SLE in the studied cohort of Egyptian children. [ABSTRACT FROM AUTHOR]

Introduction Intermittent exotropia 'X(T)' is a disorder of binocular eye movement control, in which one eye intermittently moves outward. It is the commonest type of exodeviation and is usually detected by the parents in early childhood. Patients with X(T) tend to manifest their deviation when they are tired, stressed, or have cold. Purpose To manifest the prevalence of different types of X(T) in exotropic children aged 6–12 years attending Ain Shams University ophthalmology outpatient clinic. Patients and methods This is a cross-sectional study including 139 exotropic children aged 6–12 years attending Ain Shams University ophthalmology outpatient clinic. All patients underwent full medical and ophthalmic history. The ophthalmic examinations included visual acuity, ocular movements, and the angles of deviation. Further ocular examinations included slit-lamp examination for anterior segment, fundus examination, and Worth 4-dot test. Results A total of 139 exotropic children (6–12 years old) attending the ophthalmology outpatient clinic of Ain Shams University were included. We found that X(T) is the commonest type of exodeviation, in 86.3%. The 'basic type' was the commonest subtype of X(T), in 84.2%. The true divergence excess was the second most common type, in 7.5%, pseudodivergence was seen in 5.8%, and the convergence insufficiency was the least common type seen in 2.5%. Conclusions Intermittent exotropia was the commonest type of exodeviation among children aged 6–12 years. The basic type was the commonest subtype of X(T). [ABSTRACT FROM AUTHOR]

To assess serum level of CXCL12 in systemic lupus erythematosus (SLE) patients and to study its relation to clinical features, disease activity and damage. Forty SLE patients and 40 controls were included. SLE disease activity index (SLEDAI) and the damage index were assessed. Serum CXCL12 level was measured using ELISA and renal biopsy done. The mean age of the patients was 34.5 ± 10.4 years, disease duration 5 ± 5.2 years and were 38 females and 2 males (F:M 19:1). Renal biopsy was performed in 16 patients; 6 had inactive and10 active lupus nephritis (LN); 24 without signs suggestive of LN. Serum level of CXCL12 was significantly higher in patients (30.8 ± 16.9 ng/ml) than controls (20.2 ± 15.3 ng/ml) (p = 0.004). CXCL12 in patients with active LN (53.2 ± 25.3 ng/ml) was significantly elevated than those without LN (27 ± 12.5 ng/ml)(p < 0.001); and tended to be higher than those with inactive LN (34.2 ± 8.3 ng/ml)(p = 0.31). Levels were comparable between those with inactive LN and those without LN (p = 0.34). A significant correlation was found between serum CXCL12 and each of platelet count (p = 0.02), ANA titer (p = 0.007) and serum creatinine (p = 0.014). No significant correlations was found between CXCL12 and either SLEDAI (p = 0.59) or the damage index (p = 0.48). Alopecia was inversely associated with CXCL12 (p = 0.02). CXCL12 is a potential key-player for SLE development. Adding this test to ANA, serum creatinine, platelet count and renal biopsy findings may enhance their diagnostic capacity for lupus nephritis and can help in early management and prediction of its prognosis. [ABSTRACT FROM AUTHOR]

To assess health-related quality of life (HRQoL) and associated variables in Egyptian ankylosing spondylitis (AS) patients. 50 male patients with AS and 50 age-matched controls were enrolled. Clinical and laboratory data were examined including history for peripheral arthritis. Visual analog scale (VAS), Bath AS disease activity index (BASDAI), AS disease activity score (ASDAS)-C-reactive protein (CRP) and ASDAS-erythrocyte sedimentation rate (ESR) , Bath AS functional index (BASFI), Bath AS metrology index (BASMI) and AS quality of life (ASQoL). Spinal radiographs were graded by the modified Stoke AS spinal score (mSASSS). The mean age of the patients was 33.1 ± 7.4 years and disease duration of 6.8 ± 3.1 years. The mean ASQoL score in patients was significantly higher (9.4 ± 3.8; 3–15) compared to the control. 26 (52%) patients had peripheral arthritis. Of these, 17 (65.4%) had hip involvement, 6 (23.1%) had knee, 5 (19.2%) had shoulder affection and 3 (11.5%) had ankle joint involvement. Patients with peripheral joint involvement had significantly higher ASQoL score than those without (11.4 ± 3.1 and 7.3 ± 3.4 respectively, p < 0.001). ASQoL significantly correlated with BASMI (p = 0.048), BASFI (p = 0.02), BASDAI (p = 0.01), ASDAS.CRP (p = 0.02) and ASDAS.ESR (p = 0.01). The multiple regression analysis to identify the independent variables associated with the ASQoL showed a significant association with peripheral joint involvement, BASDAI, ASDAS.ESR, ASDAS.CRP and BASFI scores. AS is a chronic inflammatory disease that affects HRQoL especially with higher disease activity, functional disability, more peripheral joint involvement and lesser spinal mobility in Egyptian patients. [ABSTRACT FROM AUTHOR]

The aim of this work is to trace how rheumatologists all over Egypt are approaching the COVID-19 pandemic and what changes it has brought about in the patients' care with special attention to its effect on vulnerable rheumatic disease (RD) patients. This survey further aims to help inform the rheumatology community about the changes in practice during the COVID-19 pandemic. The survey included 26 questions distributed to University staff members across Egypt members of the Egyptian College of Rheumatology (ECR). It takes 5–10 min to fill out. The practice setting of participating rheumatologists included University Teaching Hospitals that are the main rheumatology and clinical immunology service providers for adults and children RD patients. There was an overall agreement across the country in the responses to the survey that took a median time of 7 min to fill in. Potential changes in rheumatology outpatient practice by staff members evolved since the COVID-19 pandemic. None of the university rheumatology staff members has prescribed chloroquine or HCQ to prevent or treat COVID-19 in a non-hospitalized patient who was not previously on it. Twenty-three recommended decrease/avoid NSAIDs if the RD patient had confirmed COVID-19 or symptoms. There is an agreement to the key emerging frontline role of rheumatologists in treating COVID-19. During the pandemic, RD cases requiring admission were dealt with by several modified strategies. The overall agreement among the different university rheumatology departments during such critical situation has provoked the ECR to consider providing provisional guidelines for dealing with RD patients during this global catastrophe. [ABSTRACT FROM AUTHOR]

Objectives: The present work was conducted to estimate the prevalence of adult Behçet'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. Patients and method: 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. Results: 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. Conclusions: 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. Key Points: • The prevalence and phenotype of Behçet'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. [ABSTRACT FROM AUTHOR]

Abstract Background There is risk of premature atherosclerosis in juvenile idiopathic arthritis (JIA) patients which predisposes to cardiovascular disease (CVD) in adulthood. This can be assessed by flow mediated dilatation (FMD) and carotid intima media thickness (IMT) of the arterial wall and by soluble vascular cell adhesion molecule (sVCAM-1). Aim of the work To assess endothelial dysfunction in JIA children and to correlate sVCAM with FMD of brachial artery and carotid IMT. Patients and methods The study was conducted on 55 JIA patients. The following was assessed: body mass index (BMI), blood pressure, juvenile arthritis disease activity score (JADAS27). Childhood Health Assessment Questionnaire (C-HAQ), physical activity questionnaire (PAQ), fatigue assessment using The Pediatric Quality of Life (PedsQL) inventory, full blood count, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), rheumatoid factor (RF), serum creatinine and lipid profile, sVCAM-1, FMD and IMT. Results The patients' age was 10.9 ± 3.9 years and were 28 (50.9%) females. JADAS-27 and CRP was higher in systemic JIA, but fatigue scores were significantly lower. CHAQ was significantly lower in patients with polyarticular disease. Patients with high disease activity had significantly younger age of onset, lower BMI, shorter disease duration, lower fatigue scale and physical activity scores and higher CHAQ. sVCAM-1 significantly correlated with CHAQ, low-density lipoprotein, CRP and ESR while FMD significantly correlated with PedsQL and PAQ. Conclusion JIA patients had impaired endothelial function and increased cIMT with increased sVCAM-1, impaired lipid profile, decreased physical activity and increased fatigue with a potentially higher cardiovascular risk in this pediatric population. [ABSTRACT FROM AUTHOR]

Abstract Background The diagnosis of early rheumatoid arthritis (RA) is challenging. B-cell chemokine (CXCL13) plays a critical role in the disease pathogenesis. Aim of the work To assess the diagnostic value of serum CXCL13 in early RA and compare it with rheumatoid factor (RF) and anti-cyclic citrullinated peptide (anti-CCP) antibodies. Patients and methods The study included 60 RA patients; 30 early, 30 established RA and 30 healthy controls. The modified health assessment questionnaire (MHAQ), modified Sharp-van der Heijde score (MSS) and disease activity score (DAS28) were assessed in RA patients. RF, anti-CCP and serum level of CXCL13 were measured. Results Patients had a mean age of 39 ± 7.4 years and disease duration of 4.4 ± 5.7 years; they were 46 females and 12 males (F:M 3.8:1). Serum CXCL13 was significantly higher in early (191.7 ± 74.4 pg/ml) compared to established (136.4 ± 79 pg/ml) RA (p = 0.007) which were not observed with RF and anti-CCP; both were higher than in control (30.4 ± 13.5 pg/ml) (p < 0.001). In early RA, the frequencies of CXCL13, RF and anti-CCP positivity were 90%, 73.3% and 56.7% while in the established cases the frequencies were 36.7%, 66.7% and 63.3% respectively. CXCL13 significantly correlated with DAS28 (early: 0.49, p = 0.006; established: r = 0.38, p = 0.04) but not with MHAQ or MSS. The CXCL13 significantly correlated with both the RF and anti-CCP in both early and established cases (p < 0.001). Conclusion CXCL13 is an important for the diagnosis of early RA with a superior diagnostic performance compared to RF and anti-CCP. It may also be considered a potential biomarker of disease activity. [ABSTRACT FROM AUTHOR]

Background: Ankylosing spondylitis (AS) is a chronic inflammatory disorder that is characterized by inflammatory backache, enthesitis, sacroiliitis, peripheral arthritis, and anterior uveitis. Currently, a significant number of studies have been performed to seek markers to monitor disease activity in AS, and no novel markers satisfy the characteristics of use in the clinical setting. Objective: To assess serum Dickkopf -1 (DKK-1) level among AS patients and control individuals and evaluate their possible correlation with disease activity. Patients and Methods: This was a case-control study which comprised 40 AS cases and 40 gender matched healthy controls. ELISA was used in measuring DKK-1 concentration in the serum of studied subjects. Results: Mean ASDAS was 2.4±0.7, ranged from 0.8-4.1, mean BASFI was 3±1.9, ranged from 0.8-6.8, mean BASDI was 3.6±1.3, ranged from 0.9-6.8. AS patients demonstrated significantly greater DKK-1 level in comparison with controls (mean =197.7 vs. 87.1, p<0.001). There were positive significant association between DKK-1 and AS disease activity measures. There were significant positive correlations between ESR, CRP, MS, ASDAS CRP, BASFI, BASDI and DKK-1 values. Conclusion: serum DKK-1 concentration is elevated in cases with AS, and it is significantly accompanied by disease activity and functional impairment. [ABSTRACT FROM AUTHOR]

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]. [ABSTRACT FROM AUTHOR]