Juvenile ankylosing spondylitis

Abstract

Fifteen patients with juvenile ankylosing spondylitis were reviewed. Eight presented with extraspinal arthritis. Certain clinical features were of value early in the disease in distinguishing these patients from those with juvenile rheumatoid arthritis: male sex, remittent oligoarthritis, late onset in childhood, relative sparing of hands and wrists, infrequent cervical spine involvement, and negative tests for rheumatoid factors and antinuclear antibodies. Roentgenographic demonstration of sacroiliac arthritis with reactive sclerosis makes the diagnosis virtually certain.

The early clinical recognition of juvenile‐onset ankylosing spondylitis and its differentiation from juvenile rheumatoid arthritis

“…Hip disease, which in other studies (3,11,17,48) was shown to influence functional outcome in patients with juvenile-onset AS, became prominent in our group some years after onset. Although we confirmed the predominance of pauciarthritis as reported by others (3)(4)(5)(6)11,(13)(14)(15)(16)(17)(18)(19), its value as a discriminator between juvenile-onset AS and JRA was only marginal at 6 months. Furthermore, the number of patients with juvenile-onset AS extending to polyarthritis at the end of the first year of disease increased steadily thereafter.…”

“…Such symptoms, however, did not occur early in the course of the disease. Fewer than 15% of our patients had pain, stiffness, or reduced mobility of the lumbosacral spine and/or sacroiliac joints in the initial year, and in contrast to some previou:s reports (13)(14)(15)(16)(17)19), none presented with solely axial symptoms. Scarcity of axial disease at onset has been attributed (though this has not been reasonably dlocumented) to the lack of sensitive methods of assessment.…”

“…Scarcity of axial disease at onset has been attributed (though this has not been reasonably dlocumented) to the lack of sensitive methods of assessment. It is of interest to note, however, that this study and most of those carried out retrospectively have found axial disease within 10 years from onset and less frequently many years later (3,11,(13)(14)(15)(16)(17)(18)(19)20,24). Some of our own investigations indicate that regardless of age or duration of the disease, only patients who report persistent pain and stiffness and have spinal or sacroiliac tenderness show reduced mobility of the spine, radiographic sacroiliitis, or low values for chest expansion (20,26,52).…”

“…Such intervals were chosen because 1 ) current criteria (22,30) state that the presence of symptoms for 6 months is appropriate for both diagnosis and classification of JRA, 2) our previous observations (20,26) suggest that most clinical differences between juvenile-onset AS and JRA, except for axial involvement, occur in the first year of symptoms and do not change in a significant way for several years, and 3) by 10 years after onset most of such differences, including axial symptoms and definite diagnosis, and JRA patterns are well established (3,6,8,10,(13)(14)(15)(16)(17)(18)(19)(20)(21)29,30,(32)(33)(34)(35). …”

“…Juvenile-onset AS has become the most important example of this problem. The disease is named, defined, and diagnosed according to clinical and radiographic signs of spinal, sacroiliac, costosternal, and costovertebral joint disease, but, in contrast to adultonset AS (1 1,12), the juvenile-onset form is characterized by persistent or recisrrent bouts of peripheral arthritis and enthesitis in the initial years and definite symptoms of axial disease and radiographic sacroiliitis 5-10 years after onset, or sometimes much later (3)(4)(5)(6)(7)(13)(14)(15)(16)(17)(18)(19)(20)(21). In the meantime, imost patients with juvenileonset AS might meet the diagnostic criteria for juvenile rheumatoid arthritis (JRA) (22) or juvenile chronic arthritis (23) and therefore might be classified into any of the 3 main subgroups of Ihose diseases, creating not only confusion and misinterpretation, but also lack of recognition of children at risk, and even sometimes of adolescents in whom the dlisease is well established.…”