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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 < 0.0001) than rural. Northern cities had more patients with suspected COVID-19 (13.9% vs 6.1%; p < 0.0001); was significantly associated with more disease flares (30.8% vs 5.8%) with subsequent change in the RA treatment (20.9% vs 6.4%; p < 0.0001). Patients with RA faced remarkable difficulty to obtain their medications with subsequent change in their disease status. The challenges of the pandemic have hastened changes in the way we deliver health care.

ObjectivesThe aim of the present work was to explore the perspectives of Egyptian Rheumatology staff members as regards the coronavirus disease-19 (COVID-19) vaccine.MethodsThe survey is composed of 25 questions. Some questions were adapted from the global rheumatology alliance COVID-19 survey for patients.Results187 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%).ConclusionsThe 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.

Primary Sjögren's Syndrome (pSS) is a systemic autoimmune disease that predominantly impacts the exocrine glands. It is characterized by a diverse clinical manifestation and the existence of various autoantibodies. There is a lack of studies assessing the primary pSS phenome driven by anti-Sjögren syndrome autoantibodies in Africa, particularly in Egypt. This study aims to evaluate the clinical implications of antinuclear antibodies (ANA) and anti-Ro/anti-La autoantibodies in an Egyptian national cohort of pSS patients. We conducted a cross-sectional analysis of pSS patients, comparing clinical manifestations and disease severity based on serological profiles. A total of 301 pSS patients (mean age: 45.6±10.2 years; F:M ratio 7.4:1) were included. Patients with positive ANA (59.5%) had a higher prevalence of anti-Ro (p=0.001) and anti-La (p=0.0001) antibodies, along with lower rates of dry eyes (p=0.04) and enlarged parotid glands (p=0.001). Corticosteroid and azathioprine use was more frequent in ANA-positive patients (p=0.017, p=0.003). Double-positive anti-Ro/anti-La patients exhibited higher rates of dry mouth (p=0.045), articular manifestations (p<0.0001), fibromyalgia (p=0.001), RF positivity (p<0.001), and C4 consumption (p<0.001). Patients with pSS exhibit distinct clinical and laboratory profiles based on their autoantibody status, emphasizing the importance of immunological assessment for disease management.

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.

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.

Purpose Juvenile systemic lupus erythematosus (J-SLE) is a complex, heterogeneous disease affecting multiple organs. However, the classification of its subgroups is still debated. Therefore, we investigated the aggregated clinical features in patients with J-SLE using cluster analysis. Methods: Patients (≤ 16 years) diagnosed using the Systemic Lupus International Collaborating Clinics (SLICC) classification criteria were identified from the clinical database of the Egyptian College of Rheumatology (ECR) SLE study group. Demographic data, clinical characteristics, laboratory features, and current therapies were selected. A cluster analysis was performed to identify different clinical phenotypes. Results: Overall, 404 patients, of whom 355 (87.9%) were female, had a mean age at diagnosis of 11.2 years and a mean disease duration of 2.3 years. We identified four distinct subsets of patients. Patients in cluster 1 ( n  = 103, 25.5%) were characterized predominantly by mucocutaneous and neurologic manifestations. Patients in cluster 2 ( n  = 101, 25%) were more likely to have arthritis and pulmonary manifestations. Cluster 3 ( n  = 71, 17.6%) had the lowest prevalence of arthritis and lupus nephritis (LN), indicative of mild disease intensity. Patients in cluster 4 ( n  = 129, 31.9%) have the highest frequency of arthritis, vasculitis, and LN. Cluster 1 and 4 patients had the highest disease activity index score and were less likely to use low-dose aspirin (LDA). The SLE damage index was comparable across clusters. Conclusions: Four identified J-SLE clusters express distinct clinical phenotypes. Attention should be paid to including LDA in the therapeutic regimen for J-SLE. Further work is needed to replicate and clarify the phenotype patterns in J-SLE.

Objective The aim of the work was to delineate the computed tomography pulmonary angiography (CTPA) findings in Behçet’s disease (BD) patients with and without chest manifestations. Patients and methods The study included 122 BD adults recruited from 5 Teaching University Hospitals in Egypt of those who agreed to perform a CTPA. The Arabic version of BD current activity form (BDCAF) and BD damage index (BDI) were assessed. Detailed pulmonary manifestations, examination, plain radiology chest, and CTPA findings were recorded. Results The mean age of patients was 36.9 ± 11.3 years, male:female was 1.8:1, disease duration 9.6 ± 8.2 years, and age at onset 28.3 ± 8.6 years. Their mean BDCAF was 4.4 ± 2.2 and BDI 3.4 ± 1.8. There were chest manifestations in 51 (41.8%) and plain chest x-ray findings in 13 (10.7%) and CTPA findings in 31 (25.4%) in the form of pulmonary thromboembolism in 15 (12.3%), pulmonary aneurysms in 7 (5.7%), pneumonia in 5 (4.1%), interstitial lung disease in 4 (3.3%) and pleural effusion in 3 (2.5%). Patients with chest manifestations had significantly higher frequency of cardiac manifestations (15.7%) compared to those without (2.8%; p  = 0.023); chest x-ray findings tended to be higher (17.6% vs 5.6%; p  = 0.05) while CTPA findings were significantly detected (51% vs 7%; p  < 0.0001). Higher frequency of CTPA findings were in females ( p  < 0.0001). Yet the rate of serious pulmonary embolisms, aneurysms, and thrombosis was exclusive in males. Conclusion Meticulous investigation of the chest manifestations is warranted in BD patients to undermine the actual magnitude of pulmonary impact. CTPA provides a realistic estimate of the extent of involvement even in asymptomatic cases. Key Points • Meticulous chest assessment is warranted in Behçet’s disease patients to undermine the actual magnitude of pulmonary impact • CTPA provides a realistic estimate of the extent of involvement even in asymptomatic cases

Aim of the work: This study investigates whether serum and urinary interleukin-6 (IL-6) represent an early marker of kidney involvement and assesses the difference between them and renal biopsy in lupus nephritis (LN). Patients and methods: A total of 60 systemic lupus erythematosus (SLE) patients were compared to 20 healthy controls. Urinary and serum IL-6 were measured in both patients and controls. In addition, renal biopsy was done prior or shortly after urine and blood sampling; the results were classified according to the International Society of Nephrology/Renal Pathology Society classification of LN by recording the activity score and chronicity score for each sample. Results: There was a significant higher level of urinary IL-6 in the SLE patients with biopsy-proven LN than in those without LN and those of the control group. However, no significant difference was reported between the three groups as regards serum IL-6. A strong positive correlation was found between urinary IL-6 and renal disease activity based on the renal SLE disease activity index (SLEDAI) score with no significant correlation regarding the extra renal SLEDAI. Urinary IL-6 was positively correlated with renal biopsy results and with its activity scores but weakly correlated with the chronicity scores. Conclusion: Urinary IL-6 may provide a simple noninvasive potential marker of disease activity of renal involvement in adult patients with SLE.

BackgroundBehçet’s disease (BD), commonly seen in the Silk road countries, is a variable vessel vasculitis with no specific investigation that reflects disease activity. The Behçet’s Disease Current Activity Form (BDCAF) is the most famous and acceptable clinical activity score.PurposeTo 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.Patients and methodsThe 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.ResultsPatients 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).ConclusionThe 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. Key Points• The Arabic version of the BDCAF can be extended to other Arab countries for development of a Pan-Arab score.• This is the first study to provide a reliable and valid Arabic version of the BDCAF-Egyptian dialect for measuring current disease activity in BD patients.

ObjectivesThe 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 methodThe 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.ResultsThe 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.ConclusionsThis 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.