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[Juvenile-onset ankylosing spondylitis].

C J Menkes, C Job-Deslandre, J L Feldmann

    La Semaine Des Hopitaux : Organe Fonde Par L'Association D'Enseignement Medical Des Hopitaux De Paris
    |February 16, 1984
    PubMed
    Summary
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    Juvenile onset ankylosing spondylitis (AS) affects many young boys, often starting in lower limb joints. While many experience peripheral and axial disease, the functional prognosis for these patients is generally good.

    Area of Science:

    • Rheumatology
    • Pediatric Rheumatology
    • Immunogenetics

    Background:

    • Ankylosing spondylitis (AS) with juvenile onset (under 17 years) is a significant clinical entity.
    • Previous studies have highlighted the prevalence and characteristics of juvenile AS, but further characterization is needed.

    Purpose of the Study:

    • To analyze the clinical features, diagnostic criteria, and long-term outcomes of juvenile onset ankylosing spondylitis.
    • To evaluate the diagnostic utility of HLA B27 and radiologic findings in this population.

    Main Methods:

    • Retrospective study of 36 patients diagnosed with juvenile onset ankylosing spondylitis between 1977 and 1981.
    • Diagnostic criteria included radiologic sacroiliitis, presence of HLA B27, and/or clinical manifestations.
    • Data collected on age of onset, clinical presentation, disease progression, radiologic findings, laboratory markers (ESR), HLA B27 status, and functional prognosis.

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    Main Results:

    • The study included 36 patients (85% male) with a mean age of onset of 12.3 years.
    • Common onset sites were lower limb joints (knee, hip, ankle, heel).
    • During follow-up, 81% tested positive for HLA B27; radiologic sacroiliitis was present in 31 patients, often with a delay in diagnosis.
    • Peripheral joint disease (35 patients) and axial involvement (25 patients) were common. Hip involvement was frequent, with 6 cases of complete destruction.
    • Erosion and ossification of the calcaneum were observed in 15 cases. ESR was elevated in 72% of patients.
    • Functional prognosis was good, with over 50% leading almost normal lives.

    Conclusions:

    • Juvenile onset ankylosing spondylitis predominantly affects young males and often presents with peripheral joint involvement.
    • Early diagnosis is crucial, as radiologic sacroiliitis may be delayed.
    • Despite significant radiologic findings and disease progression, the functional prognosis for juvenile AS is generally favorable.