Presentation of enthesitis-related arthritis and juvenile-onset spondyloarthritis: a cross-sectional study in a pediatric and adult clinic

Lanças, Sean Hideo Shirata; Furlan, Matheus Zanata Brufatto; Fernandes, Taciana de Albuquerque Pedrosa; Drumond, Sula Glaucia Lage; Magalhães, Claudia Saad

doi:10.1186/s42358-024-00378-8

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Sumário

Research • Adv. rheumatol. 64 • 2024 • https://doi.org/10.1186/s42358-024-00378-8 copy

Presentation of enthesitis-related arthritis and juvenile-onset spondyloarthritis: a cross-sectional study in a pediatric and adult clinic

Authorship SCIMAGO INSTITUTIONS RANKINGS

Abstract

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 a single visit and retrospective case-notes 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 were included, being 23 (69.7%) with ERA. 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 radiographic progression to spinal involvement occurred in 43.5% of ERA patients. The median time for spinal progression and age at onset was 2.2 and 12 y for ERA, and 4 and 16.5 y for 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 the late diagnoses of SpA, and the hip and spine involvement, with higher frequency of biologic treatment in juvenile-onset SpA compared to ERA.

Keywords
Juvenile idiopathic arthritis; Enthesitis-related arthritis; Spondyloarthritis; Spondyloarthropathy; Classification; Outcome

Variables	ERA (n = 23)	SpA (n = 10)	p value
Age, median (IQR)	13 (11-14.5)	26.5 [19-36]	0.01
Male, n (%)	12 (52.2)	6 (60)	0.7
White, n (%)	18 (78.2)	9 (90)	0.5
Family history of SpA, n (%)	6 [26]	4 [40]	0.7
Positive HLA B-27 ‡. n (%)	5 (31.2)	3 (33.3)	0.62
Uveitis, n (%)	3 [13]	2 [20]	0.6
Comorbidities, n (%)	10 (43.5)	5 (50)	1.0

Onset variables	ERA (n = 23)	SpA (n = 10)	p value
Age at onset (years), median (IQR)	9 (6.5–10.7)	12.5 (10-13.7)	0.01*
Disease duration (years), median (IQR)	3 (2.7–6.2)	16 (12.2–18.7)	0.01*
Time to diagnosis (years), median (IQR)	1.5 (0.5–2.7)	5.5 (2.5-6)	0.01*
N of affected joints at onset, median (IQR)	1 (1–2)	1 (1–1)	0.2
N with upper limbs synovitis, n (%)	0 (0)	0 (0)	1.0
N with lower limbs synovitis, n (%)	21 (91.3)	10 (100)	1.0
Low back pain, n (%)	5 (21.7)	3 [30]	0.7
Enthesitis, n (%)	18 (78.3)	8 (80)	1.0
Symmetric synovitis, n (%)	5 (21.7)	0 (0)	0.2
Systemic signs/symptoms, n (%)	5 (21.7)	4 [40]	0.4

Variable	ERA (n = 23)	SpA (n = 10)	p value
Disease progression, median (IQR)	9 (2.7–15)	18 [12-33]	0.2
(months)
Upper limbs, n (%)	5 (21.7)	5 (50)	0.2
Lower limbs, n (%)	20 (86.9)	10 (100)	0.5
Symmetry, n (%)	8 (34.8)	2 [20]	0.4
Low back pain, n (%)	16 (69.5)	9 (90)	0.4
Inflammatory low back pain, n (%)	11 (47.8)	7 (70)	0.4
SpA shift, n (%)	10 (43.5)	10 (100)	0.01*
Age at Sacroilitis onset, median (IQR) (years)	12 (8.7–14)	16.5(13.6–18)	0.03*
Time to SpA median (IQR) (years)	2.2 (1-3.7)	4 (1.7–6.7)	0.3
Sacroiliitis on X-ray, n (%)	3 [13]	7 (70)	0.01*
Sacroiliitis on MRI, n (%)	11 (47.8)	5 (50)	1.0

Current status variables	ERA (n = 23)	SpA (n = 10)	p value
N of painful joints, median (IQR)	1 (0–2)	1 (0–2)	0.9
N of swollen joints, median (IQR)	1 (0-1.5)	0 (0–0)	0.2
Frequency of Enthesitis, n (%)	16 (69.5%)	5 (50%)	0.4
Clinical sacroiliitis, n (%)	5 (21.7%)	3 (30%)	0.7
JADAS, median (IQR)	8 (4-13.5)	10.5 (3.1–14.7)	0.7
JsPADA, median (IQR)	2.5 (1.7–3.7)	2.75 (1.7–4.7)	0.6
JADI-A, median (IQR)	1 (0–1)	1.5 (0-3.7)	0.2
JADI-E, median (IQR)	0 (0–0)	0 (0–0)	0.9
VAS spine pain (0–10), median (IQR)	5.5 (1.5-8)	2 (0.2-5)	0.8
VAS night pain (0–10), median (IQR)	1 (0-4.2)	4 (0.2–7.5)	0.07
VAS joint/entheses pain (0–10), median (IQR)	4 (0.7-8)	4.5 (2.2–8.7)	0.4
VAS patient (global) (0–10), median (IQR)	4.5 (1.5–7.5)	6 (2.2–7.5)	0.8
VAS physician (global) (0–10), median (IQR)	2.5 [1-4]	2.5 (0.5-4)	0.8
Schöber test (cm)	14 (13-14.5)	14.4 (14-14.9)	0.5
MASES (0–13), median (IQR)	1.5 (0–3)	1 (0–5)	0.9
LEI (0–6), median (IQR)	0 (0–2)	0 (0–1)	0.4
SPARCC (0–16), median (IQR)	0.5 (0-2.5)	1 (0–1)	0.7

Sociedade Brasileira de Reumatologia Av. Brigadeiro Luís Antônio, 2466, Jardim Paulista, 01402-000 - São Paulo, SP, Tel.: +551132897165 - São Paulo - SP - Brazil
E-mail: rbreumatol@terra.com.br

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[1] *Correspondence: Sean Hideo Shirata Lanças sean.lancas@unesp.br