Dealing with an AS diagnosis is never easy, and JAS can be especially scary for both children and parents — and it may be more difficult to diagnose because doctors aren’t looking for it in this age group. To make things even more complicated, the first signs of JAS may not include back pain, but rather pain from inflammation in surrounding areas that leads to a different or less specific diagnosis. If you have juvenile ankylosing spondylitis, you may initially be told that you have:
Sacroiliitis (inflammation of your sacroiliac joints)Juvenile idiopathic arthritis (JIA), a broad “umbrella” term that includes all types of arthritis in childrenEnthesitis-related arthritis (ERA or JIA-ERA), a subtype of JIA that includes JAS
While they may be confusing, all of these terms can be correct when describing JAS. Here’s what you should know about navigating juvenile ankylosing spondylitis if you believe you or your child might have it, or you’re dealing with a new diagnosis.
Juvenile Ankylosing Spondylitis Symptoms and Diagnosis
Many people experience a long, uncertain journey in the healthcare system before they’re diagnosed with ankylosing spondylitis — and in children, this process can be even longer and prone to unsatisfying answers. “I think back pain is not well acknowledged in kids or young adults,” says Cuoghi Edens, MD, a pediatric and adult rheumatologist and assistant professor of internal medicine and pediatrics at The University of Chicago Medicine. As with adults, the onset of back pain in JAS “is usually very slow and insidious,” she says, and many doctors initially dismiss it as a mild complaint that doesn’t merit investigation. JAS can cause a range of symptoms, including:
Pain in the back, buttocks, hips, thighs, or shouldersPain in joints anywhere in the body, especially the knees or heelsStiffness in your back or joints when you wake upSwelling in your knees or anklesTrouble touching your toesTrouble standing up straightTrouble breathing deeplyLoss of appetite or weight lossFatigue
JAS Signs and Symptoms Doctors May Miss
Diagnosing JAS can be especially difficult in children who don’t experience back pain in the classic way. “A lot of times, when kids have lower back pain, the pain actually radiates to their sides, and kids say they have hip pain,” says Dr. Edens. “They get a very extensive workup for hip pain, but no one evaluates their back or sacroiliac joints.” What’s more, children with JAS can also experience pain in other joints that may lead doctors to overlook their back. And tests that don’t look at specific areas of the body where inflammation may be happening — such as blood tests for genetic or inflammatory markers — often don’t help much. While a genetic marker called HLA-B27 can predict a higher risk of developing AS, only a small fraction of people who test positive for it will actually end up with the disease. That leaves imaging as the main way doctors identify JAS — but even this isn’t a foolproof way to diagnose the condition. Edens notes that many children with JAS develop a condition called enthesitis — inflammation of the areas where tendons and ligaments connect to bones — before they experience detectable inflammation in their joints or spine. This means that X-rays and even MRIs won’t show signs of JAS. Even once joint inflammation begins, says Edens, X-rays often won’t show anything out of the ordinary. “A lot of times we actually have to do an MRI or ultrasound to see if there’s inflammation in the joints,” she adds. Another common problem, Edens says, is that healthcare providers may only order imaging of the lumbar spine region when a child complains of lower back pain. This means that any inflammation of the sacroiliac joints — below the lumbar region — won’t be detected. “If you don’t request the right image, you’re never going to find the diagnosis,” she notes.
Treatment for Juvenile Ankylosing Spondylitis
Treatment for JAS is similar to treatment for AS in adults, says Edens, and should begin as soon as a diagnosis is confirmed. “I think we should treat our youngest patients the most aggressively, because these are joints we want to preserve for their entire life,” she notes. The first step in treating JAS is typically to prescribe a nonsteroidal anti-inflammatory drug (NSAID) to reduce pain and inflammation. If this treatment isn’t successful, Edens says, the next step is usually to prescribe a biologic drug, from a drug class called TNF inhibitors. These recommendations are based on 2019 guidelines from the American College of Rheumatology.
Medication Delivery Methods May Make a Difference
Biologic drugs for JAS are injected, which presents a practical or emotional barrier to some parents and children. But injections are typically given only every week or two, and “the improvement their child has in their arthritis and back pain makes the shot worth it for a lot of our families,” Edens notes. As an alternative, an infusion of the drug may be given in a healthcare setting every month or two. Your doctor will recommend a drug treatment plan based on your symptoms and the extent of your disease activity. Your doctor may also consider prescribing drugs called DMARDs, which aren’t effective for the spine but may help address inflammation in other areas of the body. These drugs include Azulfidine (sulfasalazine), methotrexate, and Arava (leflunomide). Other potential treatments include exercises to strengthen your back and abdominal muscles, as well as physical therapy. Whatever treatment your doctor recommends, it’s important to try to keep your child’s day-to-day life as normal as possible — and to remember that your child may be able to do more as the treatment takes effect. Edens notes that she’s seen children with pain and inflammation so severe that “they’re in a wheelchair or can’t sit down to go to the bathroom.” When it leads to dramatic improvements, she says, it’s easy to accept giving them a treatment “that may sound scary and traumatic to administer. They’re able to have a normal, functioning life.”
