Abstract

ObjectiveWe wished to determine the prevalence of fever as one of the first symptoms of the enthesitis-related arthritis (ERA) subtype of juvenile idiopathic arthritis. Also, we wished to ascertain if ERA patients with fever at disease onset differed from those without fever.MethodsConsecutive cases of ERA were diagnosed and followed in a retrospective observational study from 1998 to 2013. Information about clinical/laboratory data, medications, magnetic resonance imaging (MRI), and disease activity during the study period was also recorded.ResultsA total of 146 consecutive ERA patients were assessed. Among them, 52 patients (35.6%) had fever as one of the first symptoms at disease onset. Compared with ERA patients without fever at disease onset, patients with fever had significantly more painful joints (3.5 vs. 2.8), more swollen joints (1.1 vs. 0.8), and more enthesitis (1.0 vs. 0.4) (p<0.05 for all comparisons). Patients with fever had significantly higher mean values of erythrocyte sedimentation rate, C-reactive protein, platelet count, and child health assessment questionnaire (CHAQ) scores (40.8 vs. 26.4 mm/h; 20.7 vs. 9.7 mg/dL; 353.2×109/L vs. 275.6×109/L; 1.0 vs. 0.8, respectively; all p<0.05). During two-year follow-up, CHAQ score, number of flares, as well as the number of patients treated with oral non-steroidal anti-inflammatory drugs, corticosteroids and combination therapy with disease-modifying anti-rheumatic drugs, were significantly higher in ERA patients with fever.ConclusionsFever was a frequent manifestation of ERA. ERA patients with fever had more active disease at disease onset and poorer outcomes than ERA patients without fever.

Highlights

  • Enthesitis-related arthritis (ERA) is a chronic, inflammatory disease

  • During two-year follow-up, Childhood Health Assessment Questionnaire (CHAQ) score, number of flares, as well as the number of patients treated with oral non-steroidal anti-inflammatory drugs, corticosteroids and combination therapy with disease-modifying anti-rheumatic drugs, were significantly higher in ERA patients with fever

  • Fever was a frequent manifestation of ERA

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Summary

Introduction

Enthesitis-related arthritis (ERA) is a chronic, inflammatory disease. Studies from India and China have shown ERA to be the most prevalent category of JIA [1]. Patients with ERA have a clinical picture similar to that of individuals meeting the traditional definitions of juvenile spondyloarthropathies, including juvenile ankylosing spondylitis, seronegative enthesopathy, arthropathy syndrome, and undifferentiated juvenile spondyloarthropathy (SpA) [3]. ERA is an HLA-B27-associated type of pediatric inflammatory arthritis characterized by involvement of entheses, the axial skeleton and peripheral joints, and is associated with a poorer prognosis compared with other categories of JIA [2, 4, 5]. Data from studies carried out in various populations and clinical settings suggest an increase in the prevalence and incidence of juvenile-onset SpA in recent years [6]

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