The prevalence, epidemiological characteristics and mortality trends of inflammatory myopathies patients in Oman: the Prevision study.

OBJECTIVES: This research aims to investigate the prevalence, epidemiological characteristics, mortality rates, survival rates and the rate of malignancy in patients diagnosed with inflammatory myopathies (IIM) in Oman. METHODS: This is a longitudinal study, that covered a span of 16 years at eight rheumatology centres in Oman. The study included all adults and paediatric patients diagnosed with different types of idiopathic inflammatory myopathies (IIM) and who fulfil either the Bohan classification criteria or the 2017 EULAR/ACR classification criteria. RESULTS: The study included a total of 116 patient with an average age of 38.78 (±17.61 SD) years. The most prevalent form of myositis was found to be dermatomyositis (DM) 48 (41.38%), followed by polymyositis (PM) 36 (31.03%) and juvenile myositis (JDM) 18(15.52%). However, inclusion body myositis and necrotising myopathy were relatively rare conditions. The prevalence rates for DM, PM and JDM were determined as 2.2, 2.2, and 1.14 per 100,000 population respectively. Cardiac complications were observed in 14.66% of cases. Among the individuals studied, a history of malignancy was present in around 1.72% of cases. ANA antibodies were present in 71.55% of the cases, anti-Jo 1 and anti-RNP/SM antibodies were detected in 8.62%, and Anti-Ro antibodies in 24.14%. The overall mortality rate was found to be 6.90% with a rate of 11.1% among JDM cases. The five-year survival rates for PM, DM and JDM were found to be 94.4%, 91.7% and 89.0% respectively. These rates decline over a 10-year period to 67%, 69% and 83.3% respectively. CONCLUSIONS: The study highlights the prevalence, mortality, and survival rates of IIM in Oman. Patients with JDM had a higher mortality rate. This underscores the significance of using novel healthcare strategies to improve clinical outcomes and meet special requirements for this group of patients.

Vogt-Koyanagi-Harada Syndrome (VKHS): First Two Cases Reported in Pediatric Age Group in Oman.

The Vogt-Koyanagi-Harada syndrome (VKHS) is a unique form of granulomatous autoimmune disease that mostly impacts the pigmented tissues of the body. The main feature is bilateral granulomatous panuveitis, which is detected on ophthalmologic examination, along with additional systemic signs such as vitiligo, white hair, neurological involvement, or hearing loss. This study aims to report two cases of Vogt-Koyanagi-Harada syndrome presented in the children age group, which is unusual and very rare, to improve recognition of this disease to avoid complications and delay referral.

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 Mortality in Patients with Rheumatic Diseases: A Real Concern.

BACKGROUND: Coronavirus disease 2019 (COVID 19) is a worldwide pandemic that has devastated the world in a way that has not been witnessed since the Spanish Flu in 1918. In this study, we aim to investigate the outcomes of patients with rheumatic diseases infected with COVID-19 in Oman. METHODS: A multi-center retrospective cohort study included patients with underlying rheumatological conditions and COVID-19 infection. Data were collected through the electronic record system and by interviewing the patients through a standard questionnaire. RESULTS: 113 patients with different rheumatic diseases were included with the following rheumatological diagnoses: rheumatoid arthritis (40.7%), systemic lupus erythematosus (23.1%), psoriatic arthritis (8%), Behcet's disease (7%), ankylosing spondylitis (6.2%), other vasculitides, including Kawasaki disease (4.4%), and other diagnoses (10.6%). The mean (SD) age of patients was 43 (14) years, and 82.3% were female. The diagnosis of COVID-19 was confirmed by PCR test in 84.1% of the patients. The most common symptoms at the time of presentation were fever (86%), cough (81%), headache (65%), and myalgia (60%). Hospitalization due to COVID-19 infection was reported in 24.1% of the patients, and 52.2% of these patients had received some form of treatment. In this cohort, the intake of immunosuppressive and immunomodulating medications was reported in 91.1% of the patients. During the COVID-19 infection, 68% of the patients continued taking their medications. Comorbidities were present in 39.8% of the patients. Pregnancy was reported in 2% of the patients. The 30 days mortality rate was found to be 3.5%. Diabetes, obesity, and interstitial lung diseases (ILD) were the strongest risk factor for mortality (p-value 0.000, 0.000, and 0.001, respectively). Rituximab was given in 3.8% of the patients, and it was significantly associated with increased mortality among the patients (p-value <0.001). CONCLUSION: COVID-19 infection in patients with rheumatic diseases have an increased mortality rate in comparison to the general population, with diabetes, morbid obesity, chronic kidney diseases, interstitial lung disease, cardiovascular disease, obstructive lung disease, and liver diseases as comorbidities being the most severe risk factors associated with death. Greater care should be provided to this population, including the prompt need for vaccination.

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.

SARS-CoV-2-related Multisystem Inflammatory Syndrome in Children: A case series.

On 27 April 2020, the National Health Service England issued an emergency alert for a new condition owing to the observation of an increasing number of cases of a COVID-19-related hyperinflammatory syndrome termed multisystem inflammatory syndrome in children (MIS-C). Some of the presenting symptoms appeared similar to the Kawasaki disease and toxic shock syndrome. We report the cases of six children fitting the criteria of MIS-C, admitted to Royal Hospital and Sohar Hospital, Oman, between the months of June and July in 2020. Four of these patients required admission at the paediatric intensive care unit for inotropic support while two were admitted to the paediatric ward on suspicion of appendicitis. MIS-C has been reported in a small number of individuals below the age of 21 years with a median age of 9-10 years. Five of the current patients were aged less than the median age reported in the existing literature. All of the patients showed complete recovery with supportive management, intravenous immunoglobulin and steroids, with one patient requiring interleukin-6 inhibitor (tocilizumab).

Determinants of Discordance Between Criteria for Inactive Disease and Low Disease Activity in Juvenile Idiopathic Arthritis.

OBJECTIVE: To assess concordance among criteria for inactive disease (ID) and low disease activity (LDA) in juvenile idiopathic arthritis (JIA) and to seek factors driving discordance. METHODS: The frequency of fulfillment of existing criteria was evaluated in information on 10,186 patients extracted from 3 cross-sectional data sets. Patients were divided up according to the functional phenotypes of oligoarthritis and polyarthritis. Concordance between criteria was examined using weighted Venn diagrams. The role of each individual component in explaining discordance between criteria was assessed by calculating the absolute number and percentage of instances in which the component was responsible for discrepancy between definitions. RESULTS: Criteria for ID were met by 28.6-41.1% of patients with oligoarthritis and by 24.0-33.4% of patients with polyarthritis. Criteria for LDA were met by 44.8-62.4% of patients with oligoarthritis and by 44.6-50.4% of patients with polyarthritis. There was a 57.9-62.3% overlap between criteria for ID and a 67.9-85% overlap between criteria for LDA. Parent and physician global assessments and acute-phase reactants were responsible for the majority of instances of discordance among criteria for ID (8.7-15.5%, 10.0-12.3%, and 10.8-17.3%, respectively). CONCLUSION: We found fair concordance between criteria for ID and LDA in JIA, with the main drivers of discordance for ID being physician and parent global assessments and acute-phase reactants. This observation highlights the need for further studies aimed to evaluate the impact of subjective physician and parent perception of disease remission and of laboratory measures of inflammatory activity on the definition of ID.

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.

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.

Epidemiology of juvenile idiopathic arthritis in Oman.

BACKGROUND: There is a worldwide variation in the prevalence and subtype distribution of juvenile idiopathic arthritis (JIA) which may be affected by ethnicity and genetic factors. The purpose of this study is to determine the prevalence, subtype distribution and characteristic features of JIA among Omani children and to compare our results with other ethnic populations worldwide. METHODS: A population-based, multicenter study among pediatric rheumatology clinics in the Sultanate over a 10 year period between 2004-2013. The diagnosis of JIA and identification of JIA subtypes was based on the ILAR 2004 revised criteria. The hospital charts of these patients were retrospectively reviewed and information was collected. All patients were screened for uveitis by an ophthalmologist using slit lamp examination during regular follow up visits. RESULTS: The study included a total of 107 cases of JIA in Oman over the study period. Among the 107 patients, 71 % (n = 77) were girls with a female:male ratio of 2.5:1. The mean age of disease onset was 6.85 ± 3.86 years (range 1-13years) while the mean disease duration of 4.8 ± 2.9 years (range 1-11 years). The incidence of JIA was estimated at 2/100,000 with a prevalence of JIA of 20/100,000. The prevalence of JIA in girls was 28/100,000 while the prevalence in boys was 12/100,000. According to disease distribution, the most frequent subtype was polyarticular JIA rheumatoid factor negative (39.2 %) followed by oligoarthritis (31.8 %), systemic (17.8 %), polyarticular JIA rheumatoid factor positive (7.5 %). The unique feature of the Omani cohort is the lack of occurrence of uveitis. CONCLUSIONS: This is the first epidemiological JIA study conducted in Oman that highlights unique geographical disease phenotype. Compared to Western counties, there were higher frequency of polyarticular disease and lack of occurrence of uveitis. Further studies are needed to evaluate the implications of genetic, ethnic and environmental differences of disease expression.

Geographical Clustering of Juvenile Onset Systemic Lupus Erythematosus within the Sultanate of Oman.

OBJECTIVE: While SLE is found worldwide, there is diversity in clinical presentation of the disease according to geographical variations. The aim of this study is to describe geographical distributions of childhood onset SLE within Oman to identify geographical clustering and to compare the demographic, clinical, and immunological characteristics of this cluster against the rest of Oman. METHODS: We retrospectively reviewed the hospital charts of 104 consecutive children with childhood onset SLE who were seen in pediatric rheumatology centers in the Sultanate of Oman over a 15-year period between 1995 and 2010. RESULTS: Geographical clustering of childhood onset SLE was identified in Sharqiya region, which constituted 41% (n=43) of all cases in Oman. This cohort of patients had characteristic disease features which consisted of significantly more boys affected with SLE compared to the rest of the country (42% versus 15%; p=0.002). These children also tended to be younger (10.3 versus 16.5 years; p=0.001), diagnosed at an earlier age (6.4 versus 9.4 years; p<0.001) with a stronger family history of SLE (58% versus 33%; p=0.010). These children also had increased incidence of mucocutanous changes (81% versus 62%; p=0.036) and decreased hematological abnormalities (30% versus 51%; p=0.036). CONCLUSION: We identified geographical clustering of childhood onset SLE to Sharqiya region in Oman which is associated with unique demographical and clinical features. Whether increased prevalence of disease in this region is due to geographical, environmental, ethnic or genetic factors is yet to be determined. However, it is likely to be interplay of known and other unrecognized factors.