Comparison of systemic lupus international collaborating clinics 2012 classification criteria and European league against rheumatism/American college of rheumatology 2019 classification criteria for early detection of childhood onset systemic lupus erythematosus (multi-center study).

OBJECTIVE: To assess the performance of the new EULAR/ACR criteria, particularly for early detection of cSLE, in comparison to the SLICC criteria among the pediatric population in multiple centers in Saudi Arabia. METHODS: We conducted a retrospective study that enrolled pediatric patients up to the age of 14 years who've been diagnosed with SLE and followed in pediatric rheumatology clinics at 9 multi-tertiary hospitals in Saudi Arabia from 2010 to 2021 as a case group and were compared to a similar group of pediatric patients who've had defined rheumatological diseases other than SLE with a positive ANA titer (≥1:80) as controls. In total, 245 patients were included and distributed as 129 cases (diagnosed by expert pediatric rheumatologists) versus 116 patients in the control group. All relevant clinical information, including history, physical examination findings, and laboratory tests, was documented at the initial presentations. Then, the two sets of SLE classification criteria were applied to both groups to define who's going to meet both or either one of them. The exclusion criteria included those who had insufficient data or had overlapping or undifferentiated diseases. The sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), receiver operating curve (ROC), and accuracy were calculated for SLICC 2012 and EULAR/ACR 2019 criteria (total scores≥ 10 and ≥ 13). We performed a Chi-squared test to compare sensitivity and specificity of SLICC 2012 and EULAR/ACR 2019. RESULTS: For SLICC (cut-off ≥4 criteria), the sensitivity was found to be 96.9% (95% CI 92.6%-99.4%) and the specificity was 94.8% (95% CI 89.6%-98.32%), with PPV and NPV of 95.4% and 96.5%, respectively. The ROC for it was 0.96 (95% CI 0.93-0.99), and this criterion had an accuracy of 95%. Regarding EULAR/ACR (total score ≥ 10), the performance measure showed a sensitivity of 99.2% and a specificity of 86.2%. Similarly, PPV was 88.9%; while NPV was a little higher (99.0%) than SLICC. The ROC for EULAR/ACR (total score ≥ 10) was 0.93 (95% CI 0.89-0.96), and this criterion had an accuracy of 93%. However, there was no statistically significant difference between the sensitivity and specificity of either using SLICC or EULAR/ACR (total score ≥ 10), as reflected by a p-value of 0.86 using the Chi-squared test. Although applying the EULAR/ACR with a total score of ≥ 13 revealed lower sensitivity (93.8%) than both the SLICC and the EULAR/ACR (total score ≥ 10), the specificity for it was found to increase up to 91.4% (85.7-96.2%) compared to the (86.2%) specificity of the EULAR/ACR (total score ≥ 10). CONCLUSION: In this cohort among the Saudi population with childhood-onset SLE, the new EULAR/ACR 2019 criteria efficiently enable early detection of SLE, although a more frequent rate of false positives was observed with them. Escalating the total score from ≥ 10 to ≥ 13 in the cSLE population improved the specificity close to that of SLICC 2012. Further prospective studies in pediatrics need to be done for the validation of a cut- off score of ≥ 13 in cSLE rather than the traditional score of ≥ 10 in aSLE.

The impact of the COVID-19 pandemic on parents' behavior toward scheduled pediatric vaccinations in Saudi Arabia.

INTRODUCTION: Coronavirus disease (COVID-19) is caused by a newly discovered coronavirus and has resulted in a global pandemic. The World Health Organization recommended avoiding any delay or disruption of immunization services, as this could result in increases in outbreak-prone vaccine-preventable diseases. This study aimed to determine the impact of the COVID-19 pandemic on parents' behaviour towards their children's scheduled vaccinations. METHODOLOGY: This web-based cross-sectional study recruited 1,143 parents/guardians of children below six years of age living in Saudi Arabia between May 1 and May 30, 2020 via social media platforms. A self-developed online questionnaire consisting of eight items was used. Simple and multiple binary logistic regression was used to determine the factors associated with vaccine delay during the COVID-19 pandemic. RESULTS: The parents/guardians were aged 20-60 years; 82% were aged between 20 and 39 years. It was found that 26% of parents did not vaccinate their children on time according to the national immunization schedule in regular situations, and 38% of parents reported delaying vaccination due to the COVID-19 pandemic. The multiple logistic regression analysis found that having two or more children, living in Riyadh or the Western region or not vaccinating children during regular situations were associated with an increased risk of vaccine delay during the COVID-19 pandemic. CONCLUSIONS: Delaying children's vaccinations during the COVID-19 pandemic was influenced most by living in regions with high COVID-19 prevalence and having two or more children.

Measurement of Patient Satisfaction With the Trend of Virtual Clinics During the COVID-19 Pandemic.

Background The consequences of the coronavirus 2019 (COVID-19) pandemic on healthcare systems worldwide are undeniably disrupting regularly provided care for non-COVID-19 patients. Since the start of the pandemic, medical services in Saudi Arabia have adapted to the situation by providing medical care through virtual clinics. This article aims to evaluate patient satisfaction with virtual clinics during the COVID-19 pandemic. Material and methods A cross-sectional study was conducted among patients who had experience with the virtual clinics of Unaizah College of Medicine, Qassim University, Saudi Arabia. An online questionnaire was sent to all participants who visited the virtual clinic between May 2020 and July 2020. The questionnaire included demographic data and 16 statements to assess patient satisfaction with the virtual clinic experience.  Results A total of 123 participants completed the questionnaire. Their mean age was 33 ± 12 years; 61% were females and 39% were males. Most of the participants were from the Qassim region (77.2%), while 22.8% were from other regions in Saudi Arabia. Dermatology clinics were the most frequently visited virtual clinics, followed by psychiatry clinics. Most of the participants were satisfied with the virtual clinics, with average scores greater than three for most of the components of the questionnaire. Conclusions The participants showed considerable satisfaction for virtual clinics in the time of the COVID-19 pandemic, even though the service was relatively new to them. Future additional efforts will be needed to support clinically appropriate and acceptable virtual visits combined with in-person visits after the pandemic.

Knowledge, Attitude, and Practice in Management of Childhood Fever Among Saudi Parents.

Fever is considered as a frequent symptom in childhood and is the cause of almost 65% to 70% of pediatric visits. Saudi Arabia has about 10% of child population (under 4 years of age), imposing a high burden of childhood illnesses including fever. A total of 1700 questionnaires were distributed to Saudi parents with children visiting pediatric clinic. Most of the participants were mothers (77.4%). A temperature of 38.0 °C was defined fever by 42% of participants. The majority of parents (80%) believed seizure is the consequence of untreated high fever. A total of 72.5% indicated that 40.7 to 43.20°C is the highest temperature that can be reached if untreated. There was a statistically significant relationship between mothers and fathers for overall knowledge, attitude, and practice scores. This study indicates that numerous misconceptions still persist regarding fever as more than 90% of parents demonstrated undue fear of consequent body damage from fever and also believed antibiotics can reduce high temperature.

Treatment of auricular relapsing polychondritis in a Saudi child using only non-steroidal anti-inflammatory drugs: a case report.

Relapsing polychondritis (RP) is an autoimmune disease that can involve multiple sites within the human body. It is characterized by recurrent bouts of painful cartilage inflammation, and it can cause severe complications if it affects the vital organs. This report describes the case of a five-year-old child with limited auricular RP. The patient's history was obtained from his family, and a physical examination was performed at a pediatric rheumatology clinic. The patient was successfully treated using only a non-steroidal anti-inflammatory drug, and he completely recovered. This treatment and recovery have not been reported in the literature. Therefore, these results are worthy of mention in order to avoid the use of immunosuppressant medications with localized involvement.

Validation of the Cutaneous Lupus Erythematosus Disease Area and Severity Index and pSkindex27 for use in childhood-onset systemic lupus erythematosus.

OBJECTIVE: To determine the measurement properties of the Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI) and the paediatric adaptation of the Skindex29 (pSkindex27) when used in childhood-onset SLE (cSLE). METHODS: Patients with mucocutaneous involvement of cSLE were evaluated at the study entry and 6 months later. Besides the CLASI and pSkindex27, the Pediatric Quality of Life Inventory Generic Core scale (PedsQL-GC), its Rheumatology Module (PedsQL-RM), the SLE Disease Activity Index (SLEDAI) and the SLE Damage Index (SDI) were completed. RESULTS: The CLASI and pSkindex27 had high internal consistency (both Cronbach α >0.82). Children were able to complete the pSkindex27, with self-report and caregiver proxy-reports showing excellent agreement (intraclass correlation coefficient=0.97). The CLASI Activity Score (CLASI-A) was strongly correlated with the mucocutaneous domain score of the SLEDAI as was the CLASI Damage Score (CLASI-D) with that of the SDI (both: Spearman correlation coefficients (rs) >0.68). pSkindex27 summary scores were moderately correlated with those of the PedsQL-GC and PedsQL-RM (all: rs >|0.51|), the CLASI-A and CLASI-D (both: rs > 0.64), respectively. Patients who experienced a >50% improvement of the CLASI-A between study visits had significantly higher PedsQL-GC and pSkindex27 scores than those without improvement of mucocutaneous features. CONCLUSION: Both CLASI and pSkindex27 are useful assessment tools in cSLE, active and chronic mucocutaneous lesions and their changes over time can be measured using the CLASI and the pSkindex27 can capture the impact of mucocutaneous involvement on patient health-related quality of life.

The Arabic version of the Juvenile Arthritis Multidimensional Assessment Report (JAMAR).

The Juvenile Arthritis Multidimensional Assessment Report (JAMAR) is a new parent/patient reported outcome measure that enables a thorough assessment of the disease status in children with juvenile idiopathic arthritis (JIA). We report the results of the cross-cultural adaptation and validation of the parent and patient versions of the JAMAR in the Arabic language. The reading comprehension of the questionnaire was tested in 10 JIA parents and patients. Each participating centre was asked to collect demographic and clinical data and the JAMAR in 100 consecutive JIA patients or all consecutive patients seen in a 6-month period and to administer the JAMAR to 100 healthy children and their parents. The statistical validation phase explored descriptive statistics and the psychometric issues of the JAMAR: the 3 Likert assumptions, floor/ceiling effects, internal consistency, Cronbach's alpha, interscale correlations, test-retest reliability, and construct validity (convergent and discriminant validity). A total of 100 JIA patients (27.0% systemic JIA, 23.0% oligoarticular, 25.0% RF negative polyarthritis, and 25.0% other categories) and 100 healthy children, were enrolled in one paediatric rheumatology centre. The JAMAR components discriminated well healthy subjects from JIA patients. All JAMAR components revealed satisfactory psychometric performances. In conclusion, the Arabic version of the JAMAR is a valid tool for the assessment of children with JIA and is suitable for use both in routine clinical practice and in clinical research.

Vitamin D status in children with systemic lupus erythematosus and its association with clinical and laboratory parameters.

To assess serum 25-hydroxyvitamin D (25-OH vitamin D) status in Saudi children with systemic lupus erythematosus (SLE) and determined its association with clinical, laboratory variables and disease activity. This cross-sectional study comprised children with SLE who are followed at Pediatric Lupus Clinic. All patients reviewed for demographic data, age of first disease manifestations, and disease duration. All included patients evaluated for disease activity, which is completed by using the SLE Disease Activity Index (SLEDAI) and laboratory parameters included a vitamin D profile, bone markers at enrollment and 3 months later. All patients treated with Cholecalciferol (vitamin D3 2000 IU daily) and calcium supplement (Caltrate 600 mg twice daily). Twenty-eight patients (26 female) with mean age of 9.7 years completed the evaluation. Fifteen patients had more than one major organ involvement. Most of the patients are on daily vitamin D3 supplement (800 IU) prior enrollment. The baseline assessment revealed 24 patients had low levels of serum 25-OH vitamin D levels, with a mean of 51.1 ± 33.6 nmol/L; 25 patients had high autoantibodies; and 18 patients had high protein/creatinine ratio, with a mean of 0.9 ± 1.7. Bone density was subnormal with a mean of 0.9 ± 1. The mean disease activity was 6 ± 5.6. Levels of 25-OH vitamin D correlated inversely with autoantibodies and SLEDAI and positively with bone density but not statistically significant. After 3 months, treatment of vitamin D3 (2000 IU daily) and Caltrate (600 mg twice daily), 17 patients had improvement in SLEDAI score and autoimmune markers. Disease activity of childhood SLE is probably linked with low serum 25-OH vitamin D levels. Accordingly, high daily vitamin D3 supplement could potentially impact disease activity of childhood SLE. Further follow up and more patients needed to confirm this finding.

Systemic lupus erythematosus in Arab children. Differences and similarities with different ethnicities.

OBJECTIVE: To describe systemic lupus erythematosus (SLE) features among Arab children, and compare with cohorts from different ethnicities. METHODS: This retrospective analysis of all published English literature on SLE in Arab children was conducted in March 2013. The percentage and frequencies of the clinical and laboratory features were collected, and compared between different Arab countries as well as Caucasian and South East Asian cohorts. RESULTS: A total of 560 children from 5 Arab cohorts with an average age at diagnosis of 10 years; 7.7% of patients were diagnosed before the age of 5 years. Familial SLE was frequent. Most patients had major organ involvement. Renal involvement was diagnosed in 80%, while neuropsychiatric manifestations were seen in 30%. Immunosuppressive agents were commonly used. Beta cell depletion was recently introduced for refractory cases. The outcome of the disease could not be determined from the available data. However, 145 out of 300 patients had disease damage (52.6% Saudi and 43.9% Egyptian) with mean of 1.3 for Saudi and 0.93 for Egyptian. Forty patients died during the disease course due to infections and severe organ disease. CONCLUSION: Systemic lupus erythematosus is common in Arab children, particularly familial SLE. The manifestations observed in Arabs are comparable with previous reports. However, there is a noticeable difference in the damage accrual and mortality rate between Arab and Canadian studies, which might reflect the disease severity.

Safety and efficacy of combined cyclophosphamide and rituximab treatment in recalcitrant childhood lupus.

To report the safety and efficacy of combined cyclophosphamide and rituximab treatment in Saudi children with systemic lupus erythematosus (SLE). Medical records of all children with SLE treated with cyclophosphamide and rituximab between June 2007 and June 2012 at King Faisal Specialist Hospital and Research Center, Riyadh, were reviewed for demographic characteristics, age at diagnosis, concomitant treatments, indication of using rituximab and adverse events during the treatment period. Clinical and serologic response parameters included SLE Disease Activity Index (SLEDAI), complement, anti-ds DNA antibody and ANA levels, and mean daily corticosteroid dose assessed 3 months before combined cyclophosphamide and rituximab infusion course and at 6-month interval afterward. Sixteen patients (13 girls) with refractory SLE treated with cyclophosphamide and rituximab were included. The mean age at onset of SLE was 7.8 + 3.3 years, while the mean age at diagnosis was 8.1 + 3.4 years; the mean disease duration was 4.7 + 3.2 years. All patients were treated with corticosteroid and immunosuppressive drugs. Nephritis (8 patients) was the most frequent indication; other indications included refractory arthritis, thrombocytopenia, severe mucocutaneous lesions and central nervous system involvement. All patients received 2 doses, but 4 required 4-8 extra doses. All patients showed improvement in response parameters. There was significant reduction in SLEDAI (P < 0.0002) and corticosteroid dose (P < 0.005). A total of 4 adverse events were notified; 2 developed infusion-related reactions. One patient had severe soft tissue fungal infection, and other patient had pancreatitis. Our data showed beneficial therapeutic and steroid-sparing effects of rituximab as adjunctive treatment for children with refractory SLE including both renal and extrarenal manifestations. Although rituximab was well tolerated by the majority of patients, it may associated with various adverse events.