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.

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.

Growth Pattern in Children with Systemic Lupus Erythematosus.

OBJECTIVES: Children with childhood-onset systemic lupus erythematosus (cSLE) enter adulthood with considerable morbidity. Of the recognized morbidities, growth failure is unique to cSLE. The aim of this study was to evaluate the growth pattern in children with cSLE longitudinally and identify possible risk factors. METHODS: Serial anthropometric measurements of cSLE patients were obtained over two years and expressed as z-scores. Parental heights were obtained to calculate target height. Parent-adjusted height z-score was calculated as the difference between height z-score and target height. Growth failure was defined as parent-adjusted height z-score < -1.50. Risk factors that might have contributed to growth failure were evaluated including the presence of growth failure at baseline, disease activity, disease duration, and cumulative steroid doses. RESULTS: Twenty-five patients were included in the study. Growth failure was observed in eight patients with an overall incidence of 32.0% (95% confidence interval (CI): 14-50%). When comparing the cohort with and without growth failure, the factors that determined growth failure was the pre-existence of growth failure at the time of diagnosis (z-score < -1.95 vs. 0.35; p < 0.001); higher cumulative steroid dose (15.8 vs. 9.1 g ; p = 0.061); and tendency for longer disease duration (5.4 vs. 3.7 years; p = 0.240). However, the severity of disease activity at the time of diagnosis was not a significant contributing factor (12 vs. 14; p = 0.529). CONCLUSIONS: Children with cSLE are at risk of having a negative effect on height including patients with pre-existing growth failure, high cumulative steroid dose, and longer disease duration. However, longitudinal prospective studies are needed to examine damage over time to improve health-related quality of life.

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.