One of the more under-recognized forms of juvenile arthritis is spondyloarthropathy. Patients with this condition very often present with recurrent sprains, frequent tendonitis, and low back pain. These children often visit multiple physicians before seeing the rheumatologist, where the pattern of disease involvement is recognized and the diagnosis of spondyloarthropathy is made.
Spondyloarthropathy is, by definition, “arthritis of the back,” and describes a pattern of arthritis that commonly involves the spine, sacroiliac joints, and inflammation of the tendons at their insertion around the joints (enthesitis). In adults, low back pain is often the most significant component; however, in children, the disease may present with peripheral joint involvement. Spondyloarthropathy may occur alone or as part of a larger disease process such as celiac disease, inflammatory bowel disease, psoriasis, or reactive arthritis.
Spondyloarthropathy may occur in association with a genetic marker known as HLA B27. The absence of this marker does not exclude the possibility of a sero-negative spondyloarthropathy. In fact, these patients often have completely normal blood work.
In adults with more aggressive symptoms, this association with HLA B27 is linked to a disease entity known as ankylosing spondylitis. Usually this diagnosis is established in patients in their 3rd or 4th decade and is based on clinical and x-ray findings of joint involvement. Better disease recognition and faster initiation of therapy in young patients with this condition halts progression of severe joint involvement. Early initiation of therapy in young spondyloarthropathy patients also results in fewer radiographic changes and ultimately may lead to fewer patients fulfilling criteria for ankylosing spondylitis.
The medications used to treat spondyloarthropathy range from nonsteroidal anti-inflammatory drugs to traditional disease modifying agents, such as methotrexate, to the now commonly used biologic agents.
Children with spondyloarthropathy are at increased risk of an inflammatory eye disease called iritis, which usually presents with a painful red eye and may be the presenting symptom of disease. Young patients with this condition should be seen regularly by a pediatric ophthalmologist.
Prompt recognition and treatment of juvenile spondyloarthropathy results in better long term disease outcomes. At Hospital for Special Surgery, the Pediatric Rheumatology Department is happy to provide any child displaying symptoms of childhood arthritis with the advice and treatment they need.
Read the Winter 2011 Pediatric Connection.
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Emma Jane MacDermott, MD, MRCPI
Assistant Attending Physician in Pediatrics, Hospital for Special Surgery
Assistant Professor of Pediatrics, Weill Cornell Medical College