Abstract
BackgroundObservations among Israeli pediatric rheumatologists reveal that pediatric Juvenile Spondyloarthritis (JSpA) may present differently compared to patients from the United States (US). This study is aimed to compare the demographic and clinical variables of Israeli and US JSpA patients upon presentation.MethodsWe performed a retrospective, cross-sectional, multicenter comparison of JSpA patients among 3 large Israeli pediatric rheumatology centers and a large US pediatric rheumatology center. Patients with diagnosis of Juvenile Ankylosing Spondylitis (JAS) and/or Enthesitis-related Arthritis (ERA) were included. The demographic, clinical and radiologic features were compared.ResultsOverall 87 patients were included (39 Israeli, 48 US patients). Upon presentation, inflammatory back pain, sacroiliac joint tenderness and abnormal modified Schober test, were significantly more prevalent among Israeli patients (59% vs. 35.4, 48.7% vs. 16.7, and 41.2% vs. 21.5%, respectively, all p < 0.05), whereas peripheral arthritis and enthesitis were significantly more prevalent among US patients (43.6% vs. 91.7 and 7.7% vs. 39.6% in Israeli patients vs. US patients, p < 0.05). In addition, 96.7% of the Israeli patients versus 29.7% of the US patients demonstrated sacroiliitis on MRI (p < 0.001, N = 67). Less than one-third of the Israeli patients (32%) were HLA-B27 positive vs. 66.7% of US patients (p = 0.007).ConclusionIsraeli children with JSpA presented almost exclusively with axial disease compared to US patients who were more likely to present with peripheral symptoms. HLA B27 prevalence was significantly lower in the Israeli cohort compared to the US cohort. Further studies are needed to unravel the genetic and possibly environmental factors associated with these findings.
Highlights
Observations among Israeli pediatric rheumatologists reveal that pediatric Juvenile Spondyloarthritis (JSpA) may present differently compared to patients from the United States (US)
The extra-articular manifestations of juvenile SpA may include: (1) Acute anterior uveitis (AAU), which is reported to occur in one quarter of children, to adults [3], (2) Bowel inflammation, which is reported in approximately two thirds of children, to adults [4, 5], (3) Psoriasis, in which prevalence is unclear among children with JSpA and is reported as 10–25% in adult SpA [6], and (4) Cardiovascular manifestations of conduction disturbances and aortic insufficiency, which are documented in adults but are rare among children [6, 7]
All subjects met the following inclusion criteria: (1) Diagnosis of Enthesitis-related arthritis (ERA) according to International League against Rheumatism (ILAR) criteria upon presentation OR diagnosis of unilateral or bilateral sacroiliitis, proved by MRI imaging, who did not meet the ILAR criteria for diagnosis of ERA, (2) Aged < 16 years at symptom onset
Summary
Observations among Israeli pediatric rheumatologists reveal that pediatric Juvenile Spondyloarthritis (JSpA) may present differently compared to patients from the United States (US). Studies of North American and European JSpA patients have demonstrated that JSpA is strongly associated with the expression of class I MHC molecule HLA-B27, especially among those with axial involvement [11], and characterized by a high male to female ratio, and a peak age of onset at early adolescence [7]. Those studies showed low frequency of axial involvement (e.g. sacroiliitis) as a presenting symptom, unlike the adult type [2]. Since clinical observations of Israeli pediatric rheumatologists demonstrated different demographic features and presenting symptoms of Israeli JSpA patients, our aim in this study is to characterize the demographic and clinical features of these patients and compare them to a cohort of patients from the United States (US)
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