Spondyloarthritis in Children

Reviewed by: HU Medical Review Board | Last reviewed: May 2020 | Last updated: October 2020

We might think of spondyloarthritis (SpA) as a condition that occurs in adults, but SpA can also be diagnosed in children. SpA is actually a group of related rheumatic disorders that mostly affect the sacroiliac (SI) joints between the pelvis and the spine, and also joints in the spine.1 Other joints can also be impacted, like the ankles, hips, ribcage, shoulders, and knees. When SpA occurs before the age of 17, it is called juvenile spondyloarthritis (JSpA).1 It can also be called juvenile spondyloarthropathy.

Approximately 20 percent of children with JSpA will go on to be diagnosed with ankylosing spondylitis (AS) as adults.1

Who is affected by JSpA?

Childhood arthritis affects up to 4 in 1,000 kids, and JSpA makes up approximately 20 percent of all types of childhood arthritis.1 It usually has an onset in pre-adolescence or the teen years, usually after puberty starts. It is more common in boys than girls, whereas many other forms of childhood arthritis are usually more common in girls.1

JSpA can include the following conditions, all of which are closely related:1

  • Juvenile ankylosing spondylitis (JAS)
  • Juvenile psoriatic arthritis (JPsA)
  • Juvenile inflammatory bowel disease associated (enteropathic) arthritis (JEnA)
  • Juvenile reactive arthritis (JReA)
  • Juvenile undifferentiated spondyloarthritis (JuSpA)

What causes JSpA?

The exact cause of JSpA is not known. Children who have the HLA-B27 gene are more likely to develop JSpA than those who do not have the gene, but having the gene is not enough to make a diagnosis of JSpA, since many who have the gene will not develop the condition.1 There are also other genes that are thought to contribute to JSpA, so that gene alone cannot be pinpointed as a diagnostic marker. Sometimes symptoms occur after a gastrointestinal or urinary infection.1

How is JSpA diagnosed?

JSpA is characterized by periods of mild to moderate disease flares that alternate with periods of no symptoms.2 The most common presenting symptoms are peripheral or hip arthritis.2 In approximately 20 percent of children with JSpA, the SI joints of the spine are involved at the time of diagnosis.3 In other children, the first symptom can be enthesitis, or pain or tenderness where ligaments and tendons attach to bone.3 This often occurs under the heel, behind the heel, or around the kneecap.3

As with adults with AxSpA, in children with JSpA, a diagnosis is made based on signs and symptoms, looking to see if characteristic signs are present on imaging tests, and asking questions about symptoms.4 Sometimes blood tests or x-rays or MRIs are ordered. A pediatric rheumatologist should be consulted to make an appropriate diagnosis.

Although there is no one diagnostic test for JSpA, sometimes various tests like blood work can be helpful in solidifying a diagnosis or excluding diagnoses. For instance, children with JSpA rarely have antinuclear antibodies (ANA) or rheumatoid factor (RF) on a blood test – but children with other types of juvenile arthritis do.4 In this way, a blood test can help differentiate a diagnosis if there is hesitancy in diagnosing.

How is JSpA treated?

There is no cure for JSpA, but there are treatments available. A pediatric rheumatologist will work with you to develop a treatment plan, and depending on the symptoms, sometimes other specialists will be consulted, like an ophthalmologist, dermatologist, or a gastroenterologist.

Treatment often consists of 4 parts:5

Education and counseling are used to help inform children and their families about JSpA in order to be empowered and make informed decisions. Counseling can be helpful in adjusting to the impact of the condition on one’s life and in dealing with symptoms.

Medications are used for JSpA. The first line of treatment is often a non-steroidal anti-inflammatory drug (NSAID) like naproxen (Naprosyn), ibuprofen (Advil), or tolmetin (Tolectin).5 Indomethacin (Indocin) is especially effective for JSpA but is not used in younger children because of side effects.5 Other medications used include biologics and drugs like methotrexate or sulfasalazine.

Talk with your doctor about the medications available to you, and the side effects associated with each one. Treatment often depends on the symptoms you have and the response to drugs you’ve tried.

Physical and/or occupational therapy are helpful in helping to maintain range of motion of joints, and assist in normal functioning. They can also help reduce the risk of rounding of the shoulders and posture training.

Comfort measures refers to pain relief. Pain medication is usually not necessary, but things like massage and occasional muscle relaxants might help relieve back pain in children with JSpA.

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