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.

• Anemia

  This test allows for assessment of anemia, or low blood counts. Anemia can be secondary to chronic inflammation from JAS or slow blood loss from the gut if there is accompanying intestinal inflammation. [chop.edu]

  Back pain and sometimes stooped posture Early morning stiffness Inability to take deep breath Appetite loss and weight loss Fatigue Fever Anemia Joint pain, particularly in the legs Pain at the site of muscles, ligaments, and or tendons to bone Organ [ortho.wustl.edu]

  […] night during rest early morning stiffness stooped posture in response to back pain (bending forward tends to relieve the pain) inability to take a deep breath, if the joints between the ribs and spine are affected appetite loss weight loss fatigue fever anemia [childrenshospital.org]

• Asymptomatic

  Five (33%) of these three family members had positive HLA-B27 but were asymptomatic; six members(40%) were HLA-B27 negative and symptom-free. A high positive rate of HLA-B27 was found among the patients (100%) and the family members (60%). [ncbi.nlm.nih.gov]

  Five subjects (31%) were asymptomatic and had a normal physical exam despite ongoing active sacroiliitis. [ped-rheum.biomedcentral.com]

  Neural complications secondary to fractures of a fused spine Asymptomatic inflammation of the proximal colon and terminal ileum has been observed in about 60% Osteopenia and osteoporosis Clinical Presentation of Ankylosing Spondylitis Ankylosing spondylitis [boneandspine.com]

• Constitutional Symptom

  Malignancy (osteosarcoma, rhabdo-myosarcoma, leukemia, lymphoma) Painful joints, bone pain, constitutional symptoms; no age predominance because clinical presentations vary Ill appearance, malignant effusions, joint swelling CBC abnormal (any cell line [aafp.org]

  Constitutional symptoms of malaise, fever and weight loss are extremely common. Diagnosis is made with demonstration of inflammatory markers on the laboratory investigation, and demonstration of bowel lesions on barium Xray studies. [arthritis.co.za]

• Hepatomegaly

  […] of children with JIA) Children with systemic arthritis have arthritis symptoms, with fever lasting two weeks or more, spiking for three days or more, along with one of the following symptoms: Enlargement of lymph nodes Enlargement of liver or spleen (hepatomegaly [verywell.com]

• Eye Pain

  […] up straight or touching toes Abdominal symptoms (pain, diarrhea, weight loss) Fatigue Eye pain, redness, and sensitivity to light Many symptoms of JAS are also common in other diseases and medical conditions. [chop.edu]

  Eye pain and redness, significant sensitivity to light, or blurred vision—in addition to or instead of back pain. [arthritis-health.com]

  […] loss Tiredness (fatigue) Fever Eye pain, redness, and sensitivity to light The symptoms of juvenile ankylosing spondylitis can be like other health conditions. [elcaminohospital.org]

• Blurred Vision

  Eye pain and redness, significant sensitivity to light, or blurred vision—in addition to or instead of back pain. [arthritis-health.com]

  Extraarticular manifestations These depend on the organ involved Uveitis usually presents as an acute red painful red eye with photophobia, increased lacrimation, and blurred vision. [boneandspine.com]

  Extra-articular features Uniocular anterior uveitis occurs in about 40% of patients and typically presents as acute painful red eye, with blurred vision and photophobia. [racgp.org.au]

  Acute anterior uveitis presents with ocular pain, redness and blurred vision for several weeks. It usually affects one eye at a time but it may have a recurrent pattern. Immediate control by an ophthalmologist (an eye doctor) is necessary. [printo.it]

• Skin Disease

  NIH: National Institute of Arthritis and Musculoskeletal and Skin Diseases [medlineplus.gov]

  […] the PSOAS cohort L S Gensler 1, M M Ward 2, J D Reveille 3, T J Learch 4, M H Weisman 5, J C Davis Jr 1 1 University of California, San Francisco, California, USA 2 National Institute of Health, National Institute of Arthritis and Musculoskeletal and Skin [ard.bmj.com]

  Exercise can also help. nih: national institute of arthritis and musculoskeletal and skin disease Chronic inflammation of the spine and the low back bone joint Codes M45 Ankylosing spondylitis M45.0 Ankylosing spondylitis of multiple sites in spine M45.1 [icd10data.com]

• Arthritis

  METHODS: A cross-sectional study was performed on 32 adult patients with juvenile idiopathic arthritis. [ncbi.nlm.nih.gov]

  Medical condition: Subjects with Moderate to Severe Rheumatoid Arthritis despite Methotrexate Therapy. The intended use of SB4 is Rheumatoid Arthritis (RA), Juvenile Idiopathic Arthritis (JIA), Psoriatic Arthritis (P... [clinicaltrialsregister.eu]

  knee M08.061 Unspecified juvenile rheumatoid arthritis, right knee M08.062 Unspecified juvenile rheumatoid arthritis, left knee M08.069 Unspecified juvenile rheumatoid arthritis, unspecified knee M08.07 Unspecified juvenile rheumatoid arthritis, ankle [icd10data.com]

• Fracture

  fractures is difficult due to osteopenia and deformity, and it is important to specifically search for disc space widening and discontinuity of the ossified paraspinal ligaments also known as " chalk stick " or "carrot stick fractures" 19 Andersson lesion [radiopaedia.org]

  A CT scan shows a nondisplaced extension-type fracture of the lower cervical spine and no evidence of epidural hematoma. He is treated with a cervical orthosis and admitted for pain management. [orthobullets.com]

  § Instructor in orthopedic and fracture surgery, Boston University School of Medicine; lecturer in orthopedic surgery, Tufts University School of Medicine; visiting orthopedic surgeon, Robert Breck Brigham and Massachusetts Memorial hospitals. [nejm.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 axial spondyloarthritis (AxSpA), the presence of inflammatory low back pain is a key symptom for its diagnosis. [helvia.uco.es]

  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]

  Although spondyloarthropathies all result in joint pain, each type also has specific symptoms. Ankylosing spondylitis causes stiffness and low back pain. Over time, the pain usually moves from the lower back into the upper back. [northshore.org]

• Oligoarthritis

  Girls are more likely to be diagnosed with oligoarthritis and to experience eye inflammation, a condition called uveitis. Oligoarthritis typically develops by age 6. [kidsgetarthritistoo.org]

  CONCLUSION: In this group of subjects, juvenile AS had onset more often with oligoarthritis and enthesitis than with spinal disease. Hip and knee joint involvement was more common in JAS than AAS. [ncbi.nlm.nih.gov]

  Oligoarthritis (affects 30-60 percent of children with JIA) Children with oligoarthritis have arthritis affecting one to four joints within the first six months of disease. More than four joints may be involved after the first six months. [verywell.com]

  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. [symptoma.com]

  The two types of oligoarthritis, persistent and extended, are determined by how many joints are ultimately involved. Polyarticular arthritis, rheumatoid factor negative. [kidshealth.org]

• Joint Swelling

  Joint swelling >6wks: Jia/jra - general guide 1. [healthtap.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]

  While your child might not complain of joint pain, you may notice that he or she limps — especially first thing in the morning or after a nap. Swelling. Joint swelling is common but is often first noticed in larger joints such as the knee. [mayoclinic.org]

  Ankylosing spondylitis (AS) is an inflammatory disease of the spine that begins with painful swelling of the joints in the spine and pelvis that can eventually lead to fusion of the bones (vertebrae) in the spine. [nynjcmd.com]

  […] number of affected joints: Pauciarticular: Limp with nonpainful joint swelling involving four or fewer joints; age of onset usually younger than eight years Polyarticular: Joint swelling involving five or more joints, diminished use; bimodal age distribution [aafp.org]

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].

• Pericardial Effusion

  effusion Imaging Radiography indication performed if only 1 joint is involved in order to evaluate for other causes ( e.g., osteomyelitis) Studies Labs ↑ c-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) typically in systemic and polyarticular [medbullets.com]

• Chlamydia

  Chlamydia, Yersinia and Salmonella, can infect epithelial cells and macrophages. The big question is - Is it persistent antigen from destroyed organism, or persistent infection of viable organism. [arthritis.co.za]

  The micro-organisms implicated more frequently in children with ReA include Salmonella spp, and Yersinia enterocolitica, but rarely Shigella flexneri, Chlamydia pneumoniae, and Chlamydia trachomatis. 26, 51 Gut In both children and adults, gut inflammation [ard.bmj.com]

  Antibiotics such as ciprofloxacin, given over a three-month course soon after disease onset, may have a beneficial effect on the prognosis of reactive arthritis, especially when triggered by Chlamydia trachomatis, but not in other types of spondyloarthritis [www2.courses.vcu.edu]

• HLA-B27

  The patients and their parents were examined clinically and were HLA-typed. Nine of the 10 patients exhibited the genetic marker HLA B27, but only one patient was homozygous for HLA B27. [ncbi.nlm.nih.gov]

  HLA-B27 There is a group of genes called HLA antigens that play a major role in whether your child will get a certain disease. The HLA antigen associated with JAS is called B27. [childrenshospital.org]

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]

Inactive phase of the disease during treatment with etanercept was recorded after 4 weeks, remission - at 7th month of treatment. [pharmateca.ru]

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]

Even though there is actually no cure for JAS, there are many treatments available that can significantly help patients. Doctors create an individual treatment plan according to clinical and laboratory findings. [ic.steadyhealth.com]

[…] patients with worse prognosis to receive intensive therapy earlier in their disease course." [rheumatologynews.com]

Hip joint involvement appears to be closely related to poorer prognosis. 50-75% patients with undifferentiated spondyloarthropathy progress to ankylosing spondylitis. [boneandspine.com]

The absence of hip disease in juvenile onset AS confers a good prognosis. [academic.oup.com]

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]

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]

[…] 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]

[…] synovial process disc space involvement inflammation of the annulus lead to bridging osteophyte formation (syndesmophytes) Genetics there is a genetic predisposition, but mode of inheritance is unknown HLA-B27 is located on sixth chromosome, B locus Epidemiology [orthobullets.com]

She completed a master’s degree in epidemiology at the University of Washington School of Public Health and Community Medicine, Seattle, and a fellowship in pediatric rheumatology at Children’s Hospital and Regional Medical Center, University of Washington [aafp.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]

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]

Certain cytokines appear to have a prominent role in the pathophysiology of SpA. [ped-rheum.biomedcentral.com]

Pathophysiology of Ankylosing Spondylitis Ankylosing spondylitis and other spondyloarthropathies are chronic inflammatory diseases affecting the sacroiliac joints, axial skeleton, and, peripheral joints. [boneandspine.com]

Pathophysiology [ edit ] Ankylosing spondylitis (AS) is a systemic rheumatic disease, meaning it affects the entire body. [en.wikipedia.org]

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]

Three out of five (59%) reported fatigue as “the most debilitating symptom of having an AD,” and 68% reported “profound” fatigue that prevented completion of everyday tasks. [healio.com]

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]

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.