Diving deep into lupus: Gastrointestinal involvement insights from the Oman lupus study.

OBJECTIVES: This multicenter longitudinal study investigated the prevalence of gastrointestinal (GI) manifestations in lupus patients and determined the risk factors associated with mortality. METHODS: This study is part of the Oman Lupus Study, which included 1160 patients who met the classification criteria for systemic lupus erythematosus (SLE) from January 2006 to February 2020. All patients were screened for GI symptoms and involvement. RESULTS: We identified 91 patients with GI manifestations, with a prevalence rate of 8.53% in the pediatric group and 7.75% in the adult group, and this difference was not statistically significant (p = .755). Ischemic colitis was significantly associated with longer disease duration (p < .001) and positivity for B2-glycoprotein I (B2GPI) autoantibodies (p < .0001). Moreover, a significant correlation was found between ischemic colitis and hematologic manifestations (p = .001), lupus nephritis (p = .007), pulmonary complications (p = .000-.039), and some cardiac complications (p = .012-.269). Mortality rates were greater in patients with GI involvement (24.37%), including those with ischemic colitis (p = .005), chronic peritonitis (p < .001), and spleen/liver infarction (p = .001). Sepsis, thrombocytopenia, and different internal organ involvement rates were significantly associated with increased mortality. CONCLUSION: This research provides significant insights into GI manifestations in lupus patients. A higher mortality rate was found to be associated with organ involvement, disease duration, autoantibody profile, and specific complications. Considering this fact, it is vital to prioritize management strategies to improve clinical outcomes in this group of patients.

Age of Transition Readiness of Adolescents and Young Adults With Chronic Diseases in Oman: Need an Urgent Revisit.

BACKGROUND: The Ministry of Health in Oman and some of Gulf regions set the cut-off age of "transfer" from child health care to adult health care at 13 years of age. Within the existing health system in this part of the world, there is paucity of evidence on the appropriate age for health care "transfer" of adolescents and young adults to adult health care. Similarly, there is lack of a structured health care "transition" program. The objective of the study is to indirectly determine the appropriateness of present cut-off age of transfer by studying readiness for transition among Omani patients suffering from chronic hematological conditions. METHODS: One hundred fifty adolescents and young adults with chronic hematological conditions were recruited from pediatric and adults clinics at Sultan Qaboos University Hospital. Participants were interviewed by a trained research assistant using the Arabic version of UNC TRxANSITION Scale to assess self-management skills and health related knowledge for transition. The score range is 0 to 10; the transition readiness of the patients is assessed as low (0 to 4), moderate (4 to 6), and high (6 to 10) respectively. The continuous variables were analyzed by parametric or nonparametric methods as appropriate. χ2 analysis was done to determine association of age groups within each sexes. RESULTS: The study recruited 150 subjects (52.7% males) with 50 patients in each of the 3 age groups of 10 to 13 years (lower), 14 to 17 years (middle), and 18 to 21years (higher). The mean UNC TRxANSITION Scale scores of 5.14 (SD=1.27) in males in the total sample were significantly lower as compared with that of 5.67 (SD=1.50) in females (P=0.022). There is a steady increase in the overall median score with increase in age group, with median score of 4.42 in the lower, 5.26 in the middle and 6.81 in the higher age group (P<0.001). In section wise analysis, except for Adherence and Nutrition sections of the scale, all sections have statistically significant difference in the median scores across various age categories with lowest scores in the 10 to 13 age group and highest scores in the 18 to 21 years group. In the section related to reproduction, females had significantly higher mean ranks (31.52) and compared with 17.19 in males (P=0.001). The overall median transition score when analyzed separately for males and females across age groups showed that in the higher age group, 67% of males (P=0.008) and 90% females (P<0.001) have high transition scores compared with the other 2 groups. CONCLUSIONS: Higher age was a significant predictor for transition readiness with median score being "moderate" in the lower and middle age groups, while the higher age groups scoring "high" on transition readiness. However, in the higher age group, the females (90%) showed better transition readiness than males (67%). The current age of transfer of 13 years is just at "moderate" levels. We recommend the need for establishing transition preparation program in Oman; increasing health transfer age in Oman to a cut-off age of 18 years and taking sex differences into consideration when providing interventions.

Urticarial vasculitis in pediatric systemic lupus erythematosus.

BACKGROUND/OBJECTIVES: To determine the prevalence of urticarial vasculitis (UV) in a pediatric systemic lupus erythematosus (pSLE) cohort of Arab ethnicity from Oman and to describe their demographic, clinical, and laboratory features. METHODS: We conducted a retrospective multicenter study among pediatric rheumatology centers in Oman over a 10-year period from 2008 to 2018. Analyses were performed using univariate statistics. RESULTS: A total of 148 pSLE under the age of 13 years were included of which 30% (n = 44) were men. The overall mean age at SLE diagnosis was 7.6 ± 3.5 years while the mean disease duration was 10.1 ± 5.4 years. UV was diagnosed in 36% (n = 53) of pSLE patients. Patients with pSLE and UV were more likely to be male (57% vs 15%; P < .001), diagnosed at a younger age (5.9 vs 8.5 years; P < .001), have a family history of SLE (53% vs 36%; P = .044), and have associated conjunctivitis, (32% vs 5.3%; P < .001) but less likely to have CNS involvement (7.6% vs 20%; P = .045) or hematological manifestations such as leukopenia (9.4% vs 24%; P = .028) and thrombocytopenia (5.7% vs 18%; P = .045). This subgroup was also more likely to be associated with low C3 complement count (94% vs 66%; P < .001) and positive cytoplasmic ANCA (11% vs 0%; P = .022). CONCLUSION: We report a high occurrence of UV in a pSLE cohort associated with unique demographic, clinical, and laboratory features.

The Omani 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 Omani Arabic language. The reading comprehension of the questionnaire was tested in 10 JIA parents and patients. Each participating centre was asked to collect demographic, 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 57 JIA patients (22.8% systemic, 28.1% oligoarticular, 35.1% RF negative polyarthritis, 14.0% other categories) and 85 healthy children, were enrolled in two centres. Notably, none of the enrolled JIA patients is affected with enthesitis-related arthritis or undifferentiated arthritis. The JAMAR components discriminated well healthy subjects from JIA patients. All JAMAR components revealed satisfactory psychometric performances. In conclusion, the Omani 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 clinical research.

Spectrum of systemic lupus erythematosus in Oman: from childhood to adulthood.

SLE is a disease that mainly affects women of childbearing age, however, a total of 15-20% of cases present in children. Although adult onset SLE (aSLE) and childhood onset SLE (cSLE) share the same diagnostic criteria, differences have been identified. The aim of this study is to compare the similarities and differences in between cSLE and aSLE in an Arab population from Oman. We evaluated 225 SLE patients, 139 adults and 86 children, who fulfilled the criteria for diagnosis. At disease onset, 99% of SLE cohort fulfilled the SLICC criteria; however the ACR 1997 criteria were fulfilled in 66% aSLE and 80% cSLE. The clinical features of SLE in cSLE showed higher frequency of renal (50 vs 19%; p < 0.001), musculoskeletal (67 vs 53%; p = 0.036) and pulmonary involvement (13 vs 2.9%, p = 0.005); while aSLE showed higher frequency of hematological (64 vs 49%; p = 0.25) and mucocutaneous (24 vs 10%; p = 0.13) involvement. The mean disease activity score at disease onset and during disease course was also higher in cSLE (13 vs 8.5; p < 0.0005) (16 vs 11.8; p < 0.0005), respectively. Differences in autoantibody profile were also noted in cSLE with higher positivity of anti-dsDNA and antiphospholipid antibody (94 vs 84%; p = 0.027) (53 vs 37%; p = 0.25), respectively. cSLE patients were more likely than aSLE to be treated with immunosuppressant such as cyclophosphamide (51 vs 22%; p < 0.001) and MMF (70 vs 54%; p = 0.019). Similarities and differences between aSLE and cSLE in a cohort from Oman of Arab ethnicity were identified. It appears that individual races and ethnicities may exhibit differences in disease susceptibility and manifestations.

Phenotypic characteristics and outcome of juvenile dermatomyositis in Arab children.

This study describes the disease characteristics and outcome of Arab children with juvenile dermatomyositis (JDM) and compares the findings with other ethnicities. We retrospectively reviewed the hospital registries of the participating hospitals for children with JDM seen between 1990 and 2016 in three Arab countries. All patients fulfilled Bohan and Peter criteria for JDM, diagnosed before 14 years of age and were of Arab ethnicity. Clinical and laboratory features as well as the long-term outcomes including accrual disease damage were collected at the last follow-up visit. A total of 92 JDM patients (58 girls) were included. Mean age at the onset was 6 ± 3 years, with a mean follow-up duration of 5 ± 4.4 years. Forty-three patients (46.7%) had polycyclic disease course, 34 (36.9%) had a monocyclic course, while 15 (16.3%) had a continuous progressive course. Forty-five patients (48.9%) had arthritis, 14 (15.2%) patients had an upper airway and dysphagia, and 10 patients (10.9%) had lung involvement. Eight patients (8.7%) were admitted to the intensive care unit (ICU), 4 of them required mechanical ventilation. Methotrexate had been the most frequently used immunosuppressive drug (86%) and rituximab was used in eight patients. Additionally, 31 patients received IVIG. Most of the patients achieved a complete clinical response, but 16 ended up with permanent skin changes and 12 had a residual muscle weakness. Twenty-seven patients developed calcinosis. There were two deaths due to infection during the follow-up period. We report the largest phenotypic data on Arab children with JDM. Our patients have similar characteristics to previously described cohorts. Majority of the patients remained with inactive disease.

Quality indicators for care in juvenile idiopathic arthritis.

Objective: To develop a set of quality indicators (QIs) tailored to improve the care provided to children with juvenile idiopathic arthritis (JIA) in countries across the Asia-Pacific region. Methods: An adaptation of the Research and Development Corporation (RAND)/University of California, Los Angeles (UCLA) Appropriateness Method (RAM) was used. An initial set of 32 QIs was developed after a systematic search of the literature. These were presented to members of a Delphi panel composed of pediatric rheumatologists and other relevant stakeholders from the Asia Pacific League of Associations for Rheumatology Pediatric Special Interest Group (APLAR-Pediatric SIG). After each round, the mean scores for validity and reliability, level of disagreement, and median absolute deviation from the mean were calculated. Results: The panelists were presented with 32 QIs in two rounds of voting, resulting in the formulation of a final set of 22 QIs for JIA. These QIs are categorized within six domains of care, including access to care, clinical assessment, medications and medication monitoring, screening for comorbidities, counseling, and self-efficacy and satisfaction with care. Conclusion: These QIs have been developed to evaluate and improve the quality of care provided to children with JIA, aiming to enhance health outcomes and ensure that healthcare services are tailored to the unique needs of this patient population.

Familial juvenile systemic lupus erythematosus in Arab children.

Aim of this study is to analyze the demographic, clinical, and biochemical features, and survival of familial juvenile systemic lupus erythematosus (FJSLE) in Arab children. The medical records of children with FJSLE seen at three pediatric rheumatology clinics in Saudi Arabia and Oman were retrospectively reviewed. All included children have met the following criteria: Arab ethnicity, definite diagnosis of SLE using the revised 1982 American College of Rheumatology classification criteria and family history of more than one affected sibling with SLE. The collected data included: gender, age at diagnosis, clinical and laboratory features at diagnosis. Unusual co-morbidity and mortality associated with the disease were studied. There were 50 children with FJSLE belonging to 18 families; the frequency of FJSLE in our cohort was 20.8%. The mean age at onset of SLE was 86 months (range, 18-168 months), while the mean age at diagnosis was 95 months (range, 24-192 months), and the mean duration of follow-up was 60.9 months (range, 7-132 months). The proportion of girls was predominant (78%). Autosomal recessive mode of inheritance was strongly suggested in number of our families. Mucocutaneous manifestations, arthritis, and nephritis were the most frequent features. Thirty-five patients had renal lesions, 18 of them had class IV nephritis according WHO classification. All patients were treated with different doses of steroid and immunosuppressive drugs; 37 (74%) patients received cyclophosphamide, and 6 patients treated with Rituximab. There were 5 patients required dialysis due to ESRD and 8 deaths related to SLE during the period of follow-up. FJSLE is not uncommon in our society. These findings may be helpful in identifying SLE patients with a stronger genetic predisposition; hopefully, one or more additional risk loci can be identified in multiplex Arab families that are different from what has been reported in other ethnic populations.

All Endocarditis Is Not Infective: Libman-Sacks Endocarditis.

Libman-Sacks endocarditis (LSE) is an uncommon disorder that might be confused with infective endocarditis. It is one of the systemic lupus erythematosus (SLE) manifestations that could present with heart failure. We report a 12-year-old girl who presented with a history of shortness of breath, joint pain for four weeks, and fever for about one week. On examination, she was pale, edematous, and febrile. Her cardiac exam revealed a pan-systolic murmur of mitral regurgitation, harsh, grade 3/6 best heard at the apex. She was diagnosed with systemic lupus erythematosus with lupus nephritis and carditis. Her echocardiography revealed severe mitral regurgitation with nodular thickening of the valve in keeping with a diagnosis of LSE. After appropriate management of her underlying disorder using immunosuppressive, we saw a dramatic clinical improvement and her heart failure symptoms resolved. This case proves that SLE can have significant cardiac involvement and a proper evaluation would help in overall management and prognosis.

Performance of the EULAR/ACR 2019 classification criteria for systemic lupus erythematous in monogenic lupus.

OBJECTIVE: To evaluate the application of the EULAR/ACR-2019 criteria to monogenic lupus patients and compare its performance against the SLICC-2012 criteria. METHODS: In a multicenter retrospective cohort study, consecutive patients with monogenic lupus from three tertiary lupus clinics were enrolled. The diagnosis of monogenic lupus was based on the expert physician's opinion or fulfilling the SLICC-2012 criteria. All enrolled patients had genetic variants. A control group of sporadic childhood SLE (cSLE) and non-SLE patients, were included. A descriptive data analysis was conducted, and the EULAR/ACR-2019 and SLICC-2012 criteria were applied to both groups. RESULTS: Forty-nine patients with monogenic lupus with a median age at diagnosis of 6.0 (IQR 3.0-10.8) years and 104 controls (55 patients with cSLE and 49 non-lupus patients with a median age at diagnosis of 10.0 and 5.0 respectively) were included. Forty-four (89.8%) patients with monogenic lupus fulfilled the EULAR/ACR-2019 with a mean score of 22.3±8.9. The most frequent domains were immunologic (93.9%), musculoskeletal and renal (each 57.1%), and mucocutaneous (55.1%). Fifty-four (98.2%) cSLE patients and six (12.2%) non-lupus patients met the EULAR/ACR-2019 criteria with a mean score of 22.5±9.2 and 8.5±5.2, respectively. The sensitivity of the EULAR/ACR-2019 criteria in monogenic lupus was 89.9% (95% CI: 78.3-90.2), while the specificity was 87.6% (95% CI: 75.2-88.7). CONCLUSION: This is the first and largest cohort of monogenic lupus patients testing the performance of the 2019-EULAR/ACR criteria. It efficiently classifies monogenic lupus patients, irrespective of the underlying genetic variants. Further studies are needed before these criteria are adopted worldwide. Key Points • Typically, patients with monogenic lupus have early onset severe disease, especially with mucocutaneous manifestations and a strong family history of SLE. • Monogenic lupus is a distinctive entity and might differ from the sporadic childhood SLE. • Our study includes a large multinational cohort of monogenic lupus with heterogeneous phenotypic features and underlying genetic variants. • Our study demonstrates that the EULAR/ACR-2019 criteria efficiently classified monogenic lupus patients, irrespective of the diversity of the underlying genetic variants.

Transition readiness in adolescents and young adults with chronic rheumatic disease in Oman: today's needs and future challenges.

INTRODUCTION: In Oman, the ""transition" of health care of adolescents to adult care occurs at a young age, like many other GCC countries for cultural reasons. In order to address this concern, this study was conducted to determine the transition readiness skills of adolescents and young adults with childhood onset rheumatic diseases using a cross-cultural adaptation of the UNC TRxANSITION scale. METHODS: We used a professionally translated/back translated, provider-administered UNC TRxANSITION Scale. This 32-question scale measures HCT in 10 domains including knowledge about diagnosis or treatment, diet, reproductive health, school/work, insurance, ability to self-manage and identification of new health providers. The maximum transitional score of 10, was categorized as low (1-4), moderate (4 - 7) and high (7 -10) transitional readiness scores. RESULTS: We enrolled 81 Omani adolescents and young adults (AYA) with chronic childhood onset rheumatic diseases. The cohort consisted of 79% females, with mean age of 15.8 years (± 3.53) and mean disease duration of 6.95 years (± 4.83). Our cohort's overall mean score is low 5.22 (±1.68). Only 14.8% of the cohort achieved a high transition score (≥7). Significant direct relationship was observed between age and the mean transition readiness score (r = .533, P < .001). The mean transition readiness score in the younger age group (10-13 years) was 4.07 (±1.29), the middle age group (14-18 years) was 5.43 (±1.27), while the older age group (19-21 year), was 6.12 (±1.81). Mean transition score of youngest age group was found to be significantly lower than the other two age groups (p = .003). CONCLUSION: Overall, the transition readiness of AYA in Oman is low compared to other western countries indicating the need to initiate a health care transition preparation program for patients with chronic diseases across the country. In addition, we need to establish regional guidelines to address the transfer and transition policies to be in line to international recommendations. As transition continues after transfer, and is preferably guided by adolescent developmental status rather than chronological age, it would be preferable to refer to the transition and transfer policies 9rather than transitional age policy) to be in line to international recommendations.

COVID-19 and the Clinical Phase of the Medical Doctorate Curriculum in Oman: Challenges and the way forward.

COVID-19 has gripped the world with lightning speed. Since the onset of the pandemic, activity throughout the world came to a grinding halt. However, business had to continue and people have to learn to live with the virus while the pandemic continues to rage. Medical education is no exception and may even deserve special mention, as it prepares frontline workers against the endemics of tomorrow. We discuss here the journey of medical education at the College of Medicine and Health Sciences at Sultan Qaboos University, Muscat, Oman, as the pandemic struck the world and Oman. This work suggests a roadmap for changes, discusses challenges and proposes measures to mitigate the effects of COVID-19 on medical schools.

Evaluating the performance of ACR, SLICC and EULAR/ACR classification criteria in childhood onset systemic lupus erythematosus.

BACKGROUND: The ACR 1997, SLICC 2012 and EULAR/ACR 2019 classification criteria were validated based on adult patients. To date, there are no classification criteria specific for children with SLE. The aim of the study is to compare the performance characteristics among the three SLE classification criteria (ACR-1997, SLICC-2012 and EULAR/ACR-2019) in childhood onset SLE (cSLE) cohort of Arab ethnicity from Oman. METHODS: We conducted a retrospective multicenter study in Oman of cSLE patients as cases and patients with other rheumatic disease with a positive ANA titer as controls. The cSLE cases recruited were children diagnosed with SLE before 13 years of age. Data was retrospectively collected to establish the ACR-1997, SLICC-2012 and EULAR/ACR-2019 criteria fulfilled at first visit, first year follow up and last follow up. RESULTS: Study population included 113 cSLE cases (mean age at diagnosis of 7.3 ± 3.4 years with disease duration of 6.1 ± 4.6 years) and 51 controls (mean age at diagnosis 5.0 ± 3.4 with disease duration 5.7 ± 3.9). The cSLE cases had higher frequency of familial SLE than controls (38% vs 7.8%; p < 0.001). The performance measures demonstrated that EULAR/ACR-2019 criteria had the highest sensitivity (81, 88, 89%) compared to ACR 1997 (49, 57, 66%) and SLICC 2012 (76, 84,86%); while the ACR 1997 had the highest specificity (96%) compared to SLICC 2012 (94%) and EULAR/ACR 2019 (90%) at first visit, first year and last assessment. When we increased the threshold score to ≥13 rather than the traditional score ≥ 10 for ACR/EULAR 2019, there was increased specificity (96%) at the expense of lower sensitivity (76, 83, and 84%) at first visit, first year and last assessment. CONCLUSION: In this cSLE population, EULAR/ACR 2019 scored better at initial presentation, first year and last assessment follow up. Further multinational studies are needed to validate the appropriate cut off score for the newly proposed ACR/EULAR 2019 classification criteria in cSLE to increase early sensitivity and specificity for cSLE classification.

Familial aggregation of juvenile idiopathic arthritis with other autoimmune diseases: Impact on clinical characteristics, disease activity status and disease damage.

OBJECTIVES: To evaluate the impact of family history of autoimmune diseases (FHADs) on the clinical characteristics and outcome of juvenile idiopathic arthritis (JIA). METHODS: We retrospectively reviewed children with JIA seen in 7 pediatric rheumatology clinics from 6 Arab countries. All included patients met the International League of Associations for Rheumatology classification criteria for JIA and had a disease duration greater than 1 year. Data were collected at the last follow-up visit and comprised clinical findings, including FHADs. Disease activity and disease damage were assessed by Juvenile Arthritis Multidimensional Assessment Report, and juvenile arthritis damage index (JADI) respectively. Disease activity was categorized as remission off treatment, remission on treatment, or active disease. RESULTS: A total of 349 (224 females) JIA patients with a disease duration of 5 (interquartile range 2.9-7.5) years were included. The most frequent JIA categories were polyarticular JIA and oligoarticular JIA, followed by systemic JIA. There were 189 patients with FHADs and 160 patients without FHADs. The most frequent FHADs were diabetes mellitus (21.2%), JIA (18.5%), rheumatoid arthritis (12.7%). Among patients with FHADs, 140/189 (74.1%) achieved clinical remission, while 131/160 (81.9%) patients without FHDs had clinical remission (odds ratio [OR] = 1.2, 95% CI 0.97-1.5). Rate of consanguinity, enthesitis-related arthritis (ERA) and psoriatic arthritis were higher in patients with FHADs (OR = 0.6, 95% CI 0.4-0.9 and OR = 1.2, 95% CI 1.1-1.4). Also, articular JADI correlated significantly with presence of FHADs (OR = 1.1, 95% CI 1.0-1.1). CONCLUSION: This study shows that autoimmune diseases cluster within families of patients with JIA with a high proportion of ERA and psoriatic arthritis. JIA patients with FHADs are likely to have more disease damage.

Consensus clinical approach for a newly diagnosed systemic juvenile idiopathic arthritis among members of the pediatric rheumatology Arab group.

BACKGROUND: Systemic juvenile idiopathic arthritis (sJIA) is a diagnosis of exclusion. The complex nature and clinical variety of the disease, as well as the vast clinical variation of disease presentation, may lead to difficulties in disease detection and subsequent delays in treatment. AIM: To provide a consensus guidance on the management of newly diagnosed sJIA patients among pediatric rheumatologists in Arab countries. METHODS: This work was conducted in two phases. The first phase utilized an electronic survey sent through an email invitation to all pediatric rheumatologists in Arab countries. In the second phase, a Task Force of ten expert pediatric rheumatologists from Arab countries met through a series of virtual meetings. Results obtained in phase one were prioritized using a nominal group and Delphi-like techniques in phase two. RESULTS: Seven overarching principles and a set of recommendations were approved by the Task Force to form the final consensus. CONCLUSION: This is the first consensus on a clinical approach for pediatric rheumatic diseases among Arab pediatric rheumatologists. It is presented as a guidance on the clinical approach to sJIA that requires further evidence, and future updates are anticipated.

Cumulative Damage in Juvenile Idiopathic Arthritis: A Multicenter Study From the Pediatric Rheumatology Arab Group.

OBJECTIVE: To report the cumulative articular and extraarticular damage in Arab children with juvenile idiopathic arthritis (JIA) and to identify variables that correlate with disease damage. METHODS: We conducted a multicenter, cross-sectional study among 14 pediatric rheumatology centers from 7 Arab countries. JIA patients who met the International League of Associations for Rheumatology classification criteria and had a disease duration of >1 year were enrolled. Disease activity status was assessed using the Juvenile Arthritis Multidimensional Assessment Report. Disease damage was assessed by the Juvenile Arthritis Damage Index, articular (JADI-A) and extraarticular (JADI-E). RESULTS: A total of 702 (471 female) JIA patients with a median age of 11.3 years (interquartile range [IQR] 8.0-14.0 years) were studied. Median age at disease onset was 5 years (IQR 2.0-9.0 years) and the median disease duration was 4 years (IQR 2.0-7.0 years). The most frequent JIA categories were oligoarticular JIA (34.9%), polyarticular JIA (29.5%), and systemic JIA (24.5%). Clinical remission was achieved in 73.9% of patients. At the last clinic visit, 193 patients experienced joint damage, with a mean ± SD JADI-A score of 1.7 ± 4.5, while 156 patients had extraarticular damage, with a mean ± SD JADI-E score of 0.5 ± 1.1. Patients with enthesitis-related arthritis had the highest JADI-A score. JADI-A correlated significantly with the presence of a family history of JIA. JADI-A and JADI-E had a significant correlation with long disease duration. CONCLUSION: Cumulative damage was common in this Arab JIA cohort, and consanguinity and JIA in a sibling were frequent findings and were associated with a greater cumulative damage.

A multicenter longitudinal study of the prevalence and mortality rate of systemic lupus erythematosus patients in Oman: Oman Lupus Study.

AIM: This study is a longitudinal multicenter study which aims to find the prevalence, the demographic data, survival and mortality rates of patients with systemic lupus erythematosus (SLE) in Oman. METHOD: All Omani patients, pediatrics and adults diagnosed with SLE, who fulfill either the 1997 American College of Rheumatology or Systemic Lupus International Collaborating Clinics classifications criteria for SLE were included from January 2006 till February 2020. RESULTS: In total 1160 patients were included in this cohort. Data analysis showed that patient's ages ranged from 2-82 years with female predominance and female-to-male ratio of 7:1 (87.7% female,12.3% male). The mean prevalence of SLE among different age groups was 38.8 (range 5-63 per 100 000 inhabitants). The mortality rate was found to be 5%. Male patients had significantly higher mortality rate than females (7.6% vs 5.4%, P value = .04). Sepsis was the commonest cause of mortality (34%). The coexistence of systemic sclerosis correlates significantly with death (P = .002). Survival analysis in our data showed 5, 10, 20, 40-year survival rates of 100%, 100%, 99% and 90% respectively for antinuclear antibody (ANA) positive patients and lower survival rate for ANA negative patients with 5,10, 20, 40-year survival rates of 100, 99%, 99% and 75%, respectively. CONCLUSION: This study showed that the mean prevalence of SLE in Oman to be 38.8 (range 5-63) per 100 000 inhabitants. The 40-year survival rate among patients with positive ANA was found to be 90%, while patients with negative ANA had worse survival outcomes.