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Juvenile Ankylosing Spondylitis

Juvenile Rheumatoid Arthritis

Juvenile ankylosing spondylitis (JAS) is a chronic inflammatory disorder belonging to the group of juvenile-onset spondyloarthritides. It occurs prior to the age of 16 years and primarily leads to enthesopathy, spinal abnormalities, and peripheral joint inflammation.


Presentation

Ankylosing spondylitis (AS) is a chronic inflammatory rheumatic disease that causes pathologic changes both axially and in the regions of ligament and tendon attachments to the skeleton [1]. Juvenile ankylosing spondylitis (JAS) affects children and adolescents younger than 16 years of age and presents considerably differently than adult-onset AS. One of the first mentions of JAS in the scientific literature was by Moll and Wright in 1976 which described a pediatric disease that mainly leads to an inflammation of the entheses and peripheral joints, possibly progressing to axial involvement [2] [3].

The initial manifestations with which JAS patients present are that of monoarthritis or oligoarthritis that usually involves peripheral enthesitis, tarsal disease, and, relatively rarely, sacroiliitis and spinal inflammation [4]. The predominance of lower extremity involvement is irrefutable in JAS and arthritis tends to be asymmetric. Disease progression is also fluctuant. Some patients may experience one occurrence of asymmetric arthritis that responds to treatment and does not recur, whereas others may face a progressive disease that will eventually cause serious sequelae to the affected structures [5]. Even though it is a rare clinical manifestation diagnosed in approximately 1/5 of JAS patients, acute anterior uveitis may coexist and so may additional extra-articular manifestations, including conduction heart disease, valvular disease, and lung disease [3] [6] [7]. Spinal involvement usually appears years after the early symptoms have been observed, mostly after an average period of 6 to 8 years [4]. Nonspecific complaints which may also be reported by the young patients include a kyphotic appearance due to progressed disease that has affected the spine, weight loss, decreased appetite, fatigue, and dyspnea.

Whipple Disease
  • .- ) Type 1 Excludes arthropathy in Whipple's disease ( M14.8 ) Felty's syndrome ( M05.0 ) juvenile dermatomyositis ( M33.0- ) psoriatic juvenile arthropathy ( L40.54 ) Juvenile arthritis ICD-10-CM M08.1 is grouped within Diagnostic Related Group(s) ([icd10data.com]
Increased Susceptibility to Infections
  • ., increased susceptibility to infections, liver disease), children who receive methotrexate therapy should be regularly monitored with kidney, liver function, and blood studies.[rarediseases.org]
Arthritis
  • Reactive arthritis was suspected due to conjunctivitis, arthritis and a previous episode of watery diarrhea. An ophthalmologic examination showed no evidence of uveitis.[ncbi.nlm.nih.gov]
  • METHODS: A cross-sectional study was performed on 32 adult patients with juvenile idiopathic arthritis.[ncbi.nlm.nih.gov]
  • Years ago, there were three types of juvenile rheumatoid arthritis that were recognized. In the United States, juvenile rheumatoid arthritis was also referred to as JRA, or juvenile arthritis.[verywell.com]
  • For more information: Enthesitis-Related Arthritis (ERA): A Guide for Teenagers Exercises for Enthesitis and Arthritis Juvenile Enthesitis-Related Arthritis (ERA) Juvenile Enthesitis-Related Arthritis: Living with ERA Juvenile Enthesitis-Related Arthritis[aboutkidshealth.ca]
  • Psoriatic arthritis-children may have arthritis and psoriasis, or arthritis and a strong family history of psoriasis. This form of arthritis can affect any joint.[jointhealth.org]
Low Back Pain
  • Juvenile ankylosing spondylitis affected youths in their early teens, who presented most commonly with appendicular joint complaints rather than low back pain.[ncbi.nlm.nih.gov]
  • In this paper, a 14-year-old male patient is presented as a typical case of juvenile ankylosing spondylitis and Crohn's disease with low back pain, morning stiffness, limited motion in anterior and lateral flexion and extension, left sacroiliitis, ankylosis[ncbi.nlm.nih.gov]
  • The 1984 Modified New York Criteria for the diagnosis of AS are used to diagnose JAS as well and include: Clinical manifestations: Lumbar spine motion restriction (sagittal and frontal planes) 3 months of low back pain that improves with exercise and[symptoma.com]
  • Low back pain may travel down the back into the butt and thighs. The hips and back may feel stiff, tender, and painful. If the chest (thoracic spine) is affected, AS can make it difficult to take a deep breath.[spineuniverse.com]
Joint Swelling
  • Joint swelling 6wks: Jia/jra - general guide 1.[healthtap.com]
  • Joint swelling is common but is often first noticed in larger joints such as the knee. Stiffness. You might notice that your child appears clumsier than usual, particularly in the morning or after naps. Fever, swollen lymph nodes and rash.[mayoclinic.org]
  • JRA causes joint swelling, pain and stiffness that can last anywhere from a few months to a lifetime, depending on the individual. JRA is classified as an autoimmune disorder, a condition in which the body’s immune system attacks itself.[nynjcmd.com]
  • It causes joint swelling, pain, stiffness, and loss of motion. It can affect any joint, but is more common in the knees, hands, and feet. In some cases it can affect internal organs as well.[medlineplus.gov]
  • The joint problems involve large joints, such as the hips and sacroiliac joints. Swelling of entire toes or fingers, resulting in sausage digits, also occurs.[northshore.org]
Warm Joint
  • The first signs often are joint pain or swelling or warm joints.[kidshealth.org]
Lumbosacral Back Pain
  • ILAR Criteria Patients are said to have enthesitis-related arthritis if they have arthritis or enthesitis and two of the following Sacroiliac joint tenderness [either at time of presentation or in history] Inflammatory lumbosacral back pain Presence of[boneandspine.com]
Denial
  • […] vaccines received, the timing and nature of onset, and, most importantly, differences in the disease susceptibility and presentation sufficiently distinguished the instant case from Koehn , thus a different result was not warranted (than the original denial[mctlawyers.com]

Workup

Diagnosis of both juvenile and adult ankylosing spondylitis, similarly to other spondyloarthritides, is frequently delayed, with up to 10 years passing from the occurrence of the initial symptoms to the definitive diagnosis [8]. JAS is often underdiagnosed or misdiagnosed, and the wider medical community displays a lack of adequate level of information regarding such rheumatic conditions.

The 1984 Modified New York Criteria for the diagnosis of AS are used to diagnose JAS as well and include:

Clinical manifestations:

  • Lumbar spine motion restriction (sagittal and frontal planes)
  • ≥ 3 months of low back pain that improves with exercise and aggravates by rest
  • Restriction of chest expansion compared to the normative population

Radiologic parameters

  • ≥ grade 2 bilateral sacroiliitis
  • grade 3 or 4 unilateral sacroiliitis

The diagnosis of JAS is made when at least one clinical manifestation and at least one radiologic parameter are present.

Sacroiliitis is usually diagnosed radiographically. The disadvantage of this imaging modality is that sacroiliac joint involvement will be depicted on an X-ray late in the course of the disease. Due to this reason, magnetic resonance imaging (MRI) scans has been gaining ground in the depiction of sacroiliitis in pediatric patients with suspected JAS. According to a study by Bollow et al., an MRI scan is considerably more sensitive to diagnose sacroiliac involvement in JAS patients than a plain radiograph [9].

Enlargement of the Liver
  • In some cases, active joint disease may be associated with low-grade fever; mild anemia, and slight enlargement of the liver, spleen, and lymph nodes.[rarediseases.org]
HLA-B27
  • Nine of the 10 patients exhibited the genetic marker HLA B27, but only one patient was homozygous for HLA B27. This prevalence of homozygosity among HLA B27 positive persons could be expected to occur by chance alone.[ncbi.nlm.nih.gov]
  • All three patients and four family members (26%) had positive HLA-B27 and ankylosing spondylitis (AS). Five (33%) of these three family members had positive HLA-B27 but were asymptomatic; six members(40%) were HLA-B27 negative and symptom-free.[ncbi.nlm.nih.gov]
  • The HLA B27 marker makes it possible to identify those patients at increased risk for development of ankylosing spondylitis.[ncbi.nlm.nih.gov]
  • HLA-B27 was found in 4 out of 6 cases. This frequency is almost equal to that of adult ankylosing spondylitis patients. HLA typing was very important in the early diagnosis.[ncbi.nlm.nih.gov]
  • If your child has HLA-B27, she may have a genetic susceptibility (increased chance) of developing JAS. But it's important to remember that while most people with JAS do have HLA-B27, only a few people with HLA-B27 ever actually have JAS.[childrenshospital.org]

Treatment

  • In both patients, treatment with infliximab at a dosage of 5 mg/kg body weight already led to considerable improvement with loss of joint pain the day after it was given.[ncbi.nlm.nih.gov]
  • Treatment for juvenile idiopathic arthritis While there is no cure for juvenile idiopathic arthritis, treatment advances are allowing more children to live normal lives.[jointhealth.org]
  • Inactive phase of the disease during treatment with etanercept was recorded after 4 weeks, remission - at 7th month of treatment.[pharmateca.ru]
  • The good response to synovectomy suggests this is the preferred treatment for tamale foot.[ncbi.nlm.nih.gov]
  • The goals of treatment for JAS are to: Reduce pain and stiffness Prevent deformities Help your child be as active as possible Treatment will depend on your child’s symptoms, age, and general health.[elcaminohospital.org]

Prognosis

  • […] patients with worse prognosis to receive intensive therapy earlier in their disease course."[rheumatologynews.com]
  • Current therapies, including the use of biologic medications, have improved the prognosis of this condition significantly.[pedsinreview.aappublications.org]
  • […] cell-mediated immune system are involved T-cells release tumor necrosis factor-α (TNF-α), interleukin-6 (IL-6), and IL-1 the humoral immune system is involved by increasing antinuclear antibody (ANA) production, immunoglobulins, and circulating immune complexes Prognosis[medbullets.com]

Etiology

  • The etiology is not completely understood but is known to be multifactorial, with both genetic and environmental factors playing key roles.[pedsinreview.aappublications.org]
  • It is a chronic inflammatory rheumatic disease with unknown etiology. AS is associated with the HLA-B27 antigen and also with other chronic inflammatory diseases.[physio-pedia.com]
  • A bacterial etiology, in the absence of immune dysfunction, is unlikely in polyarticular disease. Chronic recurrent multifocal osteomyelitis, a sterile bone inflammation of unknown etiology, is also to be considered.[rheumatologyadvisor.com]

Epidemiology

  • There was evidence of an alternate trigger aside from the vaccine, and epidemiologic evidence that JAS is not associated with Gardasil. Further, it was not shown that Gardasil can trigger JAS.[mctlawyers.com]
  • […] pain and inflammation 1 or more joints are involved for at least 6 weeks in patients 16 years of age subtypes polyarticular juvenile idiopathic arthritis (JIA) 5 joints involved pauciarticular JIA 1-4 joints involved systemic JIA visceral involvement Epidemiology[medbullets.com]
  • Epidemiology /Etiology AS Affects 0.1 to 0.2% of the population and [8] is predominantly seen in males in a 3:1 ratio. The onset of symptoms generally occurs in late adolescent years to early adulthood.[physio-pedia.com]
Sex distribution
Age distribution

Pathophysiology

  • Objectives After completing this article, readers should be able to: Understand the pathophysiology of juvenile idiopathic arthritis (JIA). Recognize the clinical features of the different types of JIA. Be aware of the complications of JIA.[pedsinreview.aappublications.org]
  • […] spondylitis symptoms ankylosing spondylitis sy ptoms of ankylosing spondylitis ankylosing spondylitis and neck pain how common is ankylosing spondylitis anglo spondylitis symptoms ankylosing spondylisisankylosing spondylitis iritis ankylosing spondylitis pathophysiology[youtube.com]
  • Arthritis Res Ther. 2012, 14 (6): R253- PubMed Central View Article PubMed Google Scholar Ronneberger M, Schett G: Pathophysiology of spondyloarthritis. Curr Rheumatol Rep. 2011, 13 (5): 416-420.[ped-rheum.biomedcentral.com]

Prevention

  • Thorough clinical, radiographic, and laboratory examination should prevent confusion with juvenile rheumatoid arthritis.[ncbi.nlm.nih.gov]
  • PHARMACOECONOMIC ANALYSIS OF GENETICALLY ENGINEERED BIOLOGICAL PRODUCTS USED IN THE TREATMENT OF PSORIATIC ARTHRITIS AND ANKYLOSING SPONDYLITIS PROSPECTS FOR ANTI-B-CELL THERAPY OF SYSTEMIC LUPUS ERYTHEMATOSUS MODERN APPROACHES TO TREATMENT OF JUVENILE ARTHRITIS Prevention[pharmateca.ru]
  • Treatment for juvenile ankylosing spondylitis The goal of treatment for JAS is to reduce pain and stiffness, prevent deformities, and help your child maintain as normal and active a lifestyle as possible.[stanfordchildrens.org]
  • Treatment focuses on controlling pain and inflammation, improving function, and preventing joint damage. Symptoms The most common signs and symptoms of juvenile idiopathic arthritis are: Pain.[mayoclinic.org]

References

Article

  1. Burgos-Vargas R.The assessment of the spondyloarthritis international society concept and criteria for the classification of axial spondyloarthritis and peripheral spondyloarthritis: A critical appraisal for the pediatric rheumatologist. Pediatr Rheumatol Online J. 2012;10:14.
  2. Wright and Moll. Seronegative Polyarthritis. Amsterdam. Elsevier/North Holland Publishing.1976.
  3. Burgos-Vargas R, Pacheco-Tena C, Vazquez-Mellado J. The juvenile-onset spondyloarthritides: rationale for clinical evaluation. Best Pract Res Clin Rheumatol.2002;16:551-572.
  4. Burgos-Vargas R, Vasquez-Mellado J. The early clinical recognition of juvenile-onset ankylosing spondylitis and its differentiation from juvenile rheumatoid arthritis. Arthritis and Rheumatism.1995;38(6):835-844.
  5. Weisman M, Reveille M, van der Heijde D. Ankylosing spondylitis and the spondyloarthropathies. In: Burgos-Vargas R, editor. A companion to rheumatology. 3rd ed. Mosby: St Louis.2006;94-106.
  6. Huppertz H, Voight I, Muller-Scholden J, et al. Cardiac manifestations in patients with HLA B-27-associated juvenile arthritis. Pediatr Cardiol.2000;21:141-147.
  7. Camiciottoli G, Trapani S, Ermini M, et al. Pulmonary function in children affected by juvenile spondyloarthropathy. J Rheumatol. 1999;26:1382-1386.
  8. Feldtkeller E, Khan MA, van der Heijde D, et al. Age at disease onset and diagnosis delay in HLA-B27 negative vs positive patients with Ankylosing Spondylitis. Rheumatol Int.2003; 23:61-66.
  9. Bollow M, Braun J, Hamm B, et al. Use of contrast-enhanced MR imaging to detect sacroiliitis in children. Skeletal Radiol.1998; 27:606-616.

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Last updated: 2019-07-11 20:08