Background/Purpose: Juvenile spondyloarthritis (JSpA) manifests with axial and peripheral involvement, enthesitis and HLAB27+ in 60-90% children. Radiological sacroiliitis may occur within 10 years, representing an important prognostic factor. To determine initial and long term clinical profiles of JSpA patients from a single tertiary university center; HLAB27 prevalence and relationship with disease progression to ankylosing spondylitis (AS), according to ASAS criteria.

Methods: Descriptive cross-sectional study of a cohort of JSpA subjects. Demographic, clinical and radiological data were obtained by chart review and HLA-B27 tested by flow cytometry (Becton Dickinson). Fisher and McNemar´s tests were used for statistical analyses and p<0.05 considered significant.

Results: Fifty patients with JSpA were evaluated, mean age=31.5±11.1yrs (15-60), mean age at onset=12.2±2.73yrs (7-16), mean age at diagnosis=19.8±9.0yrs (7-44), mean disease duration=18.9±11.4yrs (3-44). Most were males (44M:6F,88%) and whites (n=42,84%). Eleven (22%) children had a 1st-degree relative with SpA and 87% (34/39) were HLAB27+. At diagnosis (Table), peripheral manifestations prevailed, particularly asymmetric oligoarthritis while axial involvement was mainly inflammatory back and buttocks pain; 21 (42%) had enthesitis, all at the Achilles insertion; major extra-articular manifestation was anterior uveitis. After a mean follow up period of 12.8±9.13yrs (1-45), 5 patients were lost, axial involvement was predominant, none had uveitis and enthesitis remained in 13/21 (Table). Radiological sacroiliitis developed in 96% (n=48) patients: 41.7% (n=20) ≤5yrs, 16.7% (n=8) within 6-10yrs and 41.7% (n=20) >10yrs of initial symptoms. Remarkably, HLA-B27+ children had earlier sacroiliitis ≤5yrs of diagnosis (p=0.02), high ESR at diagnosis (p=0.04) and developed AS (p=0.02). Sacroiliitis progression was not prevented (p>0.05) despite daily NSAIDs therapy intake by all patients. Sulfasalazine was used by 86% and MTX by 72%. Currently 49% are receiving anti-TNF drugs.

Table: Clinical manifestations of patients with JSpA

Manifestations	At Diagnosis (n=50), n (%)	Currently (n=45), n (%)	p values
Peripheral	35 (70)	13 (28.8)	0.0001
Asymmetry oligoarhritis	24 (68.5)	8 (61.5)	0.19
Symmetry oligoarthritis	1 (2.8)	1 (7.6)	0.002
Polyarthritis	10 (28.5)	5 (38.4)	0.008
Axial	29 (58)	43 (95.5)	0.001
Pain in buttocks	22 (75.8)	0	0.0001
Inflammatory back pain	25 (86.2)	18 (41.8)	0.001
Extra-articular	14 (28)	1 (2.2)	0.03
Anterior uveitis	12 (85.7)	0	0.01
Gastrointestinal	2 (14.3)	1 (100)	0.009
Oral ulcers	1 (7)	0	0.009
Enthesitis	21 (42)	13 (28.8)	0.26
Calcaneus	21 (100)	11 (84.6)	0.02
Hips	0	1 (7.7)	0.12
Spinous process L5	0	2 (15.3)	0.05

Conclusion: Brazilian JSpA patients are typically white males with initial peripheral joint and enthesitic involvement that progress to axial disease. The high prevalence of HLAB27+ in JSpA associated to early sacroiliitis, elevated ESR at diagnosis and development of AS strengthen its role as a genetic marker of disease severity in children.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/positive-hla-b27-in-juvenile-spondyloarthritis-is-associated-to-early-sacroiliitis-and-progression-to-ankylosing-spondylitis/