Discover everything you need to know about juvenile ankylosing spondylitis - from its causes and symptoms to treatment options and prognosis.

Juvenile Ankylosing Spondylitis

Humza Khizer

Overview

Juvenile ankylosing spondylitis is a rare type of inflammatory arthritis that mostly affects the spine, but may also affect entheses, i.e., the place where tendons, ligaments, and muscle attach to the bone. It is a systemic disease, which means it can affect other organs or body parts with time. 
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Juvenile ankylosing spondylitis (JAS) is one of the four disorders that are classified as spondyloarthropathies; the other three are inflammatory bowel disease, psoriatic arthritis, and Reiter&rsquo;s syndrome. 

According to the American Academy of Pediatrics, ankylosing spondylitis affects about 1% of the population in the United States, and about 10-20% of people with this condition are diagnosed in childhood<a href="#">[7]</a>. 
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Signs and Symptoms

Symptoms of JAS are typically different in children than in adults<a href="#accordion">[2]</a>. They are more severe. Often, pain starts in the peripheral joints (joint pain in wrists, hips, knees, and ankles) and then progresses to the back and spine over a period of 5-10 years<a href="#accordion">[2]</a>. 
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The following are common symptoms of juvenile ankylosing spondylitis:

<ul>
	<li>Back pain, severe at night especially</li>
	<li>Stiffness in the early morning</li>
	<li>Stiffness upon waking, after sitting, or after being inactive for long periods</li>
	<li>Fatigue</li>
	<li>Low-grade fever</li>
	<li>Appetite loss</li>
	<li>Weight loss</li>
	<li>Stooped posture</li>
	<li>Shortness of breath</li>
	<li><a href="https://merisehat.pk/disease/anemia">Anemia </a></li>
	<li>Joint pain in the legs that worsens with activity</li>
	<li>Difficulty standing or bending to touch the toes</li>
	<li>Swelling and warmth in the neck, upper spine, rib cage, shoulders, buttocks, toes, ankles, and heels</li>
	<li>Pain at the site of attachment of ligaments, muscles, and/or tendons to bone</li>
	<li>Chronic inflammation can lead to inflammatory bowel disease, causing diarrhea and abdominal pain </li>
	<li>Chronic eye inflammation (iritis or uveitis) causes redness and light sensitivity, leading to vision problems</li>
	<li>Organ damage, such as eyes, heart, and lungs 
	&nbsp;</li>
</ul>

Causes

There is no known reason for JAS, but genetic and environmental factors are thought to play a role<a href="#accordion">[3]</a>. 
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<ul>
	<li>Genetic Factors</li>
</ul>

According to the Arthritis Foundation, the HLA-B27 gene is present in 80-90% of children with JAS. 

According to a 2015 study, HLA-B27 causes a decrease in protective bacteria, without which the gut becomes prone to inflammation, leading to disease progression. 
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<ul>
	<li>Environmental Factors</li>
</ul>

It is believed that exposure to viruses, bacteria, or other environmental factors can trigger this disease. 
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Children who are known to be at risk of developing JAS can be screened for the condition through blood tests to look for HLA-B27 as well as other disease markers such as C-reactive protein (CRP), erythrocyte sedimentation rate (ESR or sed rate), and a comprehensive metabolic panel. 
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NOTE: A lot of people who have the HLA-B27 gene don&#39;t have ankylosing spondylitis, while some people have the disease without having the gene. Contact a doctor to get a proper diagnosis. 
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Diagnosis

A pediatrician begins by asking about the patient&rsquo;s history, i.e., when did they first experience the symptoms, other medical conditions, and any medication that they are currently taking. They will also ask about the family history to confirm whether anyone in the family has arthritis. 

Next, the doctor examines the joints for signs of tenderness, swelling, and pain, including the spine, wrists, knees, and feet. They may also order imaging tests, including MRI, CT scan, X-ray, and ultrasound to diagnose juvenile ankylosing spondylitis.

In case JAS is suspected, the case will be referred to a pediatric rheumatologist who is an expert in inflammatory arthritis. 
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Treatment

There is no cure; treatment is to help manage symptoms and slow disease progression. The earlier a child with JAS receives treatment, the better the overall outcome and quality of life will be. 
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<h5>Medications</h5>

Medications are commonly used to reduce inflammation and pain. These include<a href="#accordion">[8]</a>:

<ul>
	<li>Biologics such as tumor necrosis factor (TNF) inhibitors</li>
	<li>Nonsteroidal anti-inflammatory drugs (NSAIDs), including over-the-counter drugs like ibuprofen and naproxen, or prescription NSAIDs such as indomethacin, celecoxib, or diclofenac </li>
	<li>Disease-modifying antirheumatic drugs (DMARDs) like methotrexate and sulfasalazine</li>
	<li>Short-term and the lowest possible dose of corticosteroids such as prednisone due to potential side effects 
	&nbsp;</li>
</ul>

A study suggests that doctors are still learning how to treat JAS. They often start by giving patients NSAIDs or csDMARDs. If those don&#39;t work well enough, doctors may try TNFi drugs<a href="#accordion">[6]</a>.

Doctors are still figuring out if other drugs like IL-17 blockers can help treat JAS<a href="#accordion">[6]</a>.&nbsp; 
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<h5>Surgery</h5>

Some children may require surgery such as hip replacements or spinal corrective surgery to help with pain due to joint fusion. However, most children with JAS do not need surgery. 
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<h5>Non-Drug Treatment</h5>

Non-drug treatments are also available. Physical therapists, for example, can design customized exercise programs for children to help them manage pain. Walking, biking, yoga, and swimming are some options. Assistive devices are recommended, too. 
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<ul>
	<li>Lifestyle Changes </li>
</ul>

Staying active can help with muscle stiffness and mobility. Additionally, some experts advise following an anti-inflammatory diet consisting of whole foods, fruits and vegetables, and fewer processed foods, but there is less evidence to support this recommendation.

<ul>
	<li>Alternative Therapy</li>
</ul>

Some people may choose alternative treatments in addition to conventional medicine. This includes acupuncture to release the body&rsquo;s natural pain-relieving hormones. Massage therapy and transcutaneous electrical nerve stimulation can also provide relaxation to some patients. 
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If JAS is not treated, it can lead to the following complications: 
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<ol>
	<li>Spinal Fusion</li>
</ol>

JAS-induced inflammation can form new bone tissue, which can fuse the vertebrae in the spine together over time. This may lead to back stiffness and limited mobility, making it difficult for the individual to bend or twist. 
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<ol start="2">
	<li>Eye Inflammation</li>
</ol>

JAS can also cause inflammation of the eyes, a condition known as uveitis. Uveitis can result in vision loss if left untreated. 
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<ol start="3">
	<li>Lung Problems</li>
</ol>

In rare cases, JAS may result in lung inflammation, leading to scarring and difficulty breathing. 
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<ol start="4">
	<li>Heart Problems</li>
</ol>

Some research shows that JAS may increase the risk of heart disease in children and young adults. 
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<ol start="5">
	<li>Psychological Impact</li>
</ol>

Living with a chronic condition like JAS can be difficult for children and adolescents, leading to feelings of anxiety, isolation, and depression. 
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NOTE: If your child is experiencing symptoms of JAS, such as joint pain or stiffness, it is important to seek medical attention as soon as possible. Early detection and treatment can help prevent complications.

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Anyone can get juvenile ankylosing spondylitis, but some people are more susceptible than others<a href="#accordion">[7]</a>: 
&nbsp;

<ul>
	<li>Tests positive for HLA-B27 </li>
	<li>Boys are 2-3 times more likely to be diagnosed; however, research indicates that this is because females are underdiagnosed</li>
	<li>Children over the age of 10 </li>
	<li>European ethnicity</li>
	<li>Someone in the immediate family has JAS 
	&nbsp;</li>
</ul>

There is currently no known way to prevent JAS as its exact cause is not yet fully understood. However, some steps may help reduce the risk of developing the condition or manage its symptoms, including: 
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Regular Exercise

Maintaining a regular exercise routine can help keep joints and muscles healthy and reduce inflammation. Low-impact exercises, such as swimming, cycling, and walking can particularly benefit people with JAS. 
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Good Posture

Poor posture puts extra strain on the spine and other joints, potentially exacerbating symptoms of JAS. Practicing good posture habits, such as sitting up straight and avoiding slouching, may help reduce the risk of developing the condition or manage its symptoms. 
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Healthy Lifestyle Habits

Maintaining a healthy weight, eating a balanced diet, and avoiding smoking and excessive alcohol consumption can all help reduce the risk of developing JAS or manage its symptoms.

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Key Points

<ul>
	<li>JAS is a type of arthritis that primarily affects children and adolescents under the age of 16.</li>
	<li>The condition causes inflammation, pain, and stiffness in the joints, particularly in the spine and pelvis.</li>
	<li>The exact cause of JAS is not fully understood, but it is believed that genetic and environmental factors cause it.</li>
	<li>There is no known cure for JAS, but early diagnosis and treatment can help manage symptoms and prevent complications.</li>
	<li>Treatment may include medication, physical therapy, and lifestyle changes such as maintaining a healthy weight and engaging in regular exercise.</li>
	<li>Complications of JAS can include spinal fusion, eye inflammation, lung problems, heart problems, and psychological impacts. 
	&nbsp;</li>
</ul>

[1] Harjacek M, Joos R, Vargas RB. Juvenile Spondyloarthritis: From Basic Science to Clinical Translation. Front Med. 2022;9:378.

[2] Horneff G, Fitter S, Foeldvari I, Minden K, Kuemmerle-Deschner J, Tzaribacev N, et al. Double-blind, placebo-controlled randomized trial with adalimumab for treatment of juvenile onset ankylosing spondylitis (JoAS): significant short term improvement. Arthritis Res Ther [Internet]. 2012;14(5):R230.

[3] Gmuca S, Weiss PF. Juvenile spondyloarthritis. Curr Opin Rheumatol. 2015 Jul;27(4):364–72.

[4] Onel KB, Horton DB, Lovell DJ, Shenoi S, Cuello CA, Angeles-Han ST, et al. 2021 American College of Rheumatology guideline for the treatment of juvenile idiopathic arthritis: therapeutic approaches for oligoarthritis, temporomandibular joint arthritis, and systemic juvenile idiopathic arthritis. Arthritis \& Rheumatol. 2022;74(4):553–69.

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