Similarities and Differences Between Juvenile and Adult Spondyloarthropathies

Fisher, Corinne; Ciurtin, Coziana; Leandro, Maria; Sen, Debajit; Wedderburn, Lucy R.

doi:10.3389/fmed.2021.681621

Cited by 11 publications

(3 citation statements)

References 232 publications

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“…JIA categories are complex, heterogeneous, with different contributions of immune system players and effector cells ( 24 , 25 , 58 – 63 ). Indeed, several studies have demonstrated a predominance of adaptive immunity in the pathogenesis of oJIA, pJIA, ERA, and JPsA ( 14 , 17 , 18 , 24 , 25 , 39 , 57 , 64 – 86 ), whereas innate immune responses are the major contributors to disease development and progression in sJIA ( 29 , 59 , 87 – 98 ). In fact, oJIA, pJIA, ERA, and JPsA are classified as autoimmune diseases, while sJIA has been proposed as an autoinflammatory disorder ( 25 , 58 , 59 ).…”

Section: Immunopathogenesis Of Juvenile Idiopathic Arthritismentioning

confidence: 99%

B Cells on the Stage of Inflammation in Juvenile Idiopathic Arthritis: Leading or Supporting Actors in Disease Pathogenesis?

Moura

Fonseca

2022

Front. Med.

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Juvenile idiopathic arthritis (JIA) is a term that collectively refers to a group of chronic childhood arthritides, which together constitute the most common rheumatic condition in children. The International League of Associations for Rheumatology (ILAR) criteria define seven categories of JIA: oligoarticular, polyarticular rheumatoid factor (RF) negative (RF-), polyarticular RF positive (RF+), systemic, enthesitis-related arthritis, psoriatic arthritis, and undifferentiated arthritis. The ILAR classification includes persistent and extended oligoarthritis as subcategories of oligoarticular JIA, but not as distinct categories. JIA is characterized by a chronic inflammatory process affecting the synovia that begins before the age of 16 and persists at least 6 weeks. If not treated, JIA can cause significant disability and loss of quality of life. Treatment of JIA is adjusted according to the severity of the disease as combinations of non-steroidal anti-inflammatory drugs (NSAIDs), synthetic and/ or biological disease modifying anti-rheumatic drugs (DMARDs). Although the disease etiology is unknown, disturbances in innate and adaptive immune responses have been implicated in JIA development. B cells may have important roles in JIA pathogenesis through autoantibody production, antigen presentation, cytokine release and/ or T cell activation. The study of B cells has not been extensively explored in JIA, but evidence from the literature suggests that B cells might have indeed a relevant role in JIA pathophysiology. The detection of autoantibodies such as antinuclear antibodies (ANA), RF and anti-citrullinated protein antibodies (ACPA) in JIA patients supports a breakdown in B cell tolerance. Furthermore, alterations in B cell subpopulations have been documented in peripheral blood and synovial fluid from JIA patients. In fact, altered B cell homeostasis, B cell differentiation and B cell hyperactivity have been described in JIA. Of note, B cell depletion therapy with rituximab has been shown to be an effective and well-tolerated treatment in children with JIA, which further supports B cell intervention in disease development.

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Section: Immunopathogenesis Of Juvenile Idiopathic Arthritismentioning

confidence: 99%

B Cells on the Stage of Inflammation in Juvenile Idiopathic Arthritis: Leading or Supporting Actors in Disease Pathogenesis?

Moura

Fonseca

2022

Front. Med.

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“…We compared ERA and juvenile-onset SpA patients due to similarities in clinical features (24). SpA comprises a wide spectrum of clinical and muscle-skeletal features, evolving to spinal involvement with back pain; this is less frequent in ERA, where arthritis and enthesis are predominant features and spine involvement is more frequently seen in adolescents and youngsters (25); such differences could be possibly related to muscle-skeletal development stage, immune response, and the gut microbiota, beyond genetic and other environmental factors.…”

Section: Discussionmentioning

confidence: 99%

Presentation of Enthesitis Related Arthritis and Juvenile-onset Spondyloarthritis, a Cross-sectional Study in a Pediatric and Adult Clinic

Lanças,

Brufatto,

Fernandes

et al. 2024

Preprint

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Background Juvenile idiopathic arthritis (JIA) comprises a whole spectrum of chronic arthritis starting before 16 years of age. The study aims to explore the clinical and demographic descriptors, treatment, and disease progression of enthesitis-related arthritis (ERA) in comparison with juvenile-onset spondyloarthritis (SpA). Methods Cross-sectional analysis of consecutive patients in two dedicated clinics, with one visit and retrospective case-note review. Arthritis, enthesitis and sacroiliitis were evaluated by scoring disease activity and damage. Continuous variables were reported by median, interquartile range; categorical variables were reported by the frequency comparison of the two groups. Results Thirty-three cases, 23 (69.7%) of ERA, were included. The median age at diagnosis was 12.5 y (SpA) vs. 9 y (ERA) (p < 0.01); the time from symptom onset to diagnosis was 5.5 y (SpA) vs. 1.5 y (ERA) (p < 0.03). In both groups, the predominant presentation was a single joint or < 5 lower limb joints and asymmetric involvement, with a high frequency of enthesitis. There was a higher frequency of mid-tarsal and ankle synovitis in the ERA group and hip involvement in those with SpA. The comparison of the frequency of spine symptoms at presentation, 30% SpA vs. 21.7% ERA (p = 0.7), was not significant, and progression to spinal involvement occurred in 43.5% of ERA patients. The median time for spinal progression was 2.3 and 12 y for ERA and SpA, respectively. Activity and damage scores were not significantly different between the groups. Treatment comparison resulted in 91.3% of ERA and 100% SpA being treated, predominantly with NSAIDs in both groups, followed by DMARDs and biologics, with a higher frequency of biologics in SpA. Conclusion The main differences were that SpA had late diagnoses and hip spine involvement, with a higher frequency of biologic treatment, than ERA.

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“…It’s a chronic inflammatory disease characterized by enthesitis, peripheral arthritis predominantly in the lower limbs and axial arthritis (sacroiliitis and spondylitis) which can progress to ankylosing spondylitis at an advanced stage, occurring before 18 years [ 1 , 2 ]. It is distinguished by male predominance, a strong association with HLA-B27 and the development of anterior uveitis [ 3 ]. ERA represents a continuum with adult spondyloarthritis (SpA) and children with HLA-B27-positive ERA are associated with a prolonged disease course and higher incidence of anterior uveitis [ 4 , 5 ].…”

Section: Introductionmentioning

confidence: 99%

Profile of HLA-B27-positive enthesitis/spondylitis-related arthritis in Senegal, West Africa

Sabounji,

Ndiaye,

Diallo

2024

Pediatr Rheumatol

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Background Enthesitis/spondylitis-related arthritis (ERA) is a type of juvenile idiopathic arthritis (JIA) frequently associated with HLA-B27. In sub-Saharan Africa, HLA-B27-positive ERA hasn’t been the subject of a specific study. Objectives We aimed to describe the clinical features, disease activity, functional disability and treatment of HLA-B27-positive ERA at diagnosis in Senegal and compare the findings to other populations. Methods We conducted a retrospective study by reviewing the medical records of patients diagnosed with ERA with an age of symptom onset < 18 years according to the 2019 PRINTO provisional criteria for ERA from January 2012 to December 2022. We collected demographic, clinical, paraclinical and therapeutic data. Disease activity score was assessed by Ankylosing Spondylitis Disease Activity Score (ASDAS) and Bath Ankylosing Spondylitis Disease Activity Index (BASDAI). Functional disability was assessed using Bath Ankylosing Spondylitis Functional Index (BASFI). Results A total of 31 patients with HLA-B27-positive ERA were included. Twenty of 31 (64.5%) were males. Twenty-seven (87%) were Fula (ethnicity). The median age at symptom onset and at diagnosis was 12 years and 19 years, respectively. Seven patients had a family history of Spondyloarthritis. Peripheral arthritis and enthesitis were the most common presenting features at disease onset. Peripheral arthritis was present in 29 (93.5%) and located in the lower limbs in 27/29 (93.1%) patients. Heel enthesitis was present in 26 (83.8%) patients. Axial involvement was present in 27 (87%) patients, dominated by low back pain and sacroiliac pain/ buttock pain in 24 (88.8%) and 22 (81.5%) patients, respectively. Seven (22.5%) patients had anterior uveitis. The ESR and CRP were elevated in 65.5% and 57.1% of cases, respectively. On imaging, sacroiliitis was found in 22 patients. The mean BASDAI was 5.5/10 (77.2% of patients had a high active disease; BASDAI ≥ 4/10). The mean ASDAS-ESR/CRP was 3.8. The mean BASFI was 5.4/10 (80% of patients had high functional disability; BASFI ≥ 4/10). Twenty-seven (87%) patients were treated with methotrexate and non-steroidal anti-inflammatory drugs. After 6 months of treatment, mean BASDAI was 3/10 and mean BASFI was 2.5/10. Conclusion In our study, HLA-B27-positive ERA was found in our Senegalese cohort mainly in adolescents of the Fula ethnic group. 22 (70.9%) patients developed ankylosing spondylitis at adulthood. The disease was very active at the time of diagnosis with significant functional disability. Treatment was mainly based on methotrexate and NAISDs.

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Similarities and Differences Between Juvenile and Adult Spondyloarthropathies

Cited by 11 publications

References 232 publications

B Cells on the Stage of Inflammation in Juvenile Idiopathic Arthritis: Leading or Supporting Actors in Disease Pathogenesis?

B Cells on the Stage of Inflammation in Juvenile Idiopathic Arthritis: Leading or Supporting Actors in Disease Pathogenesis?

Presentation of Enthesitis Related Arthritis and Juvenile-onset Spondyloarthritis, a Cross-sectional Study in a Pediatric and Adult Clinic

Profile of HLA-B27-positive enthesitis/spondylitis-related arthritis in Senegal, West Africa

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