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Spondyloarthropathy, Pediatric


Basics


Description


A group of inflammatory arthritides associated with enthesitis (inflammation at the bony insertion of tendons and ligaments) and axial involvement, typically sacroiliitis � �
  • Enthesitis-related arthritis
  • Ankylosing spondylitis (AS)
  • Psoriatic arthritis
  • Inflammatory bowel disease " �associated arthropathy

Epidemiology


  • Spondyloarthritis accounts for 15 " �20% of juvenile arthritis.
  • AS typically affects adolescent boys. Much less common in blacks:
    • HLA-B27 occurs in 70 " �90% of patients and is present in 8% of whites and 6% of blacks in the general population.

Prevalence
� � �1/10,000 white boys � �

Risk Factors


Genetics
  • HLA-B27 associated
  • Usually a family history of a male relative with disease

Pathophysiology


Inflammatory synovitis of joints and inflammation at sites of ligament and tendon attachment (entheses). Progression to ankylosis is a result of calcification of the anterior and posterior longitudinal ligaments of the spine. � �

Etiology


Autoimmune or autoinflammatory arthritis of unknown etiology, microbiome may play a role in disease � �

Diagnosis


  • Inflammatory back pain (better with exercise, not relieved by rest) of insidious onset that has been present for at least 6 weeks
  • Inactivity stiffness resulting in gelling of peripheral joints and back

History


  • Back pain and joint pain/swelling
  • Family history

Physical Exam


  • Sacroiliac (SI) tenderness
    • Indicates site of inflammation
  • Pain on direct palpation at insertion of Achilles tendon and plantar fascia at calcaneal insertion (location of entheses)
    • Indicates site of inflammation
  • Patrick test (FABER)
    • FABER stands for "Flexion, ABduction and External Rotation " �
    • A series of maneuvers to screen for issues with the sacroiliac and hip joints
  • Psoriasis, nail pitting, or dactylitis

Diagnostic Tests & Interpretation


  • Schober test of lumbar spine flexibility
    • Mark 15-cm vertical span at mid-lower back at level of iliac crest while patient is standing.
    • Have patient bend forward at the waist as far as possible without bending knees.
    • Remeasure span.
    • Abnormal if <5 cm increase in span

Lab
CBC, erythrocyte sedimentation rate (ESR), HLA-B27, rheumatoid factor (RF), and antinuclear antibody (ANA) tests � �
  • ESR is occasionally not elevated.
  • RF and ANA are typically negative.

Imaging
Sacroiliac views � �
  • Demonstrate evidence of pseudowidening, erosions, and/or sclerosis, with fusion being a late finding.
  • Because x-ray findings may take years to develop in the presence of disease, MRI is supplanting x-ray as the initial modality to assess SI involvement in some centers.

Differential Diagnosis


  • Caution
    • Overdiagnosis in HLA-B27 " �positive individuals in whom other causes for joint swelling should be considered
  • Infection
    • Reactive arthritis caused by enteric pathogens or Chlamydia species
    • Whipple disease
    • Intestinal bypass " �associated arthritis
    • Discitis
    • Pott disease (vertebral tuberculosis)
  • Tumors
    • Osteoid osteoma
  • Trauma
    • Traumatic injury causing lower back pain/spasm
    • Herniated disc
  • Metabolic
    • Ochronosis
  • Congenital
    • Kyphosis
  • Immunologic
    • Oligoarticular juvenile idiopathic arthritis
  • Psychological
    • Feigning lower back pain/stiffness
  • Miscellaneous
    • Fibromyalgia

Treatment


Medication


  • NSAIDs
    • Naproxen
    • Indomethacin
    • Diclofenac
  • Disease-modifying drugs
    • Sulfasalazine
    • Methotrexate
    • Leflunomide
    • Tumor necrosis factor (TNF) inhibitors

Additional Treatment


General Measures
  • Therapy may need to be lifelong.
  • After initiation of therapy, should see some improvement in stiffness, synovitis, and range of motion over weeks to several months
  • Only TNF inhibitors are effective for axial involvement.

Additional Therapies


Physical therapy � �
  • Physical therapy is an essential component of treatment.
  • Must encourage range-of-motion exercises and avoid prolonged neck flexion

Surgery/Other Procedures


In advanced cases, total hip replacement, C-spine fusion, and/or spinal wedge osteotomy (the latter if posture is severely affected) � �

Ongoing Care


Diet


  • Ensure food intake with NSAIDs.
  • Ensure folate intake with methotrexate.

Patient Education


Activity � �
  • As tolerated. In cases of severe/advanced disease, modify behaviors accordingly in consideration of reduced spine flexibility and subsequent risk of serious injury.

Prognosis


Poor if disease remains active for 10 years or more. � �

Complications


  • Acute anterior uveitis
  • Aortic insufficiency
  • Worsening stiffness
  • Ankylosis with risk of vertebral subluxation, fracture, and nerve damage, including cauda equina syndrome
  • Acute or chronic eye pain
  • Chest pain or shortness of breath

Alert
A red, painful eye in a patient with HLA-B27 " �positive spondyloarthropathy should not be assumed to be infectious conjunctivitis. Slit-lamp exam is required to diagnose acute anterior uveitis. � �

Additional Reading


  • Colbert � �RA. Classification of juvenile spondyloarthritis: enthesitis-related arthritis and beyond. Nat Rev Rheumatol.  2010;6(8):477 " �485. � �[View Abstract]
  • Colbert � �RA. Early axial spondyloarthritis. Curr Opin Rheumatol.  2010;22(5):603 " �607. � �[View Abstract]
  • Homeff � �G, Burgos-Vargas � �R. TNF-alpha antagonists for the treatment of juvenile-onset spondyloarthritides. Clin Exp Rheumatol.  2002;20(6)(Suppl 28):S137 " �S142. � �[View Abstract]
  • Sherry � �DD, Sapp � �LR. Enthesalgia in childhood: site-specific tenderness in healthy subjects and in patients with seronegative enthesopathic arthropathy. J Rheumatol.  2003;30(6):1335 " �1340. � �[View Abstract]
  • Stoll � �ML, Lio � �P, Sundel � �RP, et al. Comparison of Vancouver and International League of Associations for rheumatology classification criteria for juvenile psoriatic arthritis. Arthritis Rheum.  2008;59(1):51 " �58. � �[View Abstract]
  • Tse � �SM, Laxer � �RM. New advances in juvenile spondyloarthritis. Nat Rev Rheumatol.  2012;8(5):269 " �279. � �[View Abstract]
  • Tse � �SM, Laxer � �RM, Babyn � �PS, et al. Radiologic improvement of juvenile idiopathic arthritis-enthesitis-related arthritis following anti-tumor necrosis factor-alpha blockade with etanercept. J Rheumatol.  2006;33(6):1186 " �1188. � �[View Abstract]

Codes


ICD09


  • 721.9 Spondylosis of unspecified site, without mention of myelopathy
  • 720.2 Sacroiliitis, not elsewhere classified
  • 720 Ankylosing spondylitis
  • 720.9 Unspecified inflammatory spondylopathy
  • 720.89 Other inflammatory spondylopathies
  • 720.81 Inflammatory spondylopathies in diseases classified elsewhere
  • 720.1 Spinal enthesopathy

ICD10


  • M12.88 Oth specific arthropathies, NEC, vertebrae
  • M46.1 Sacroiliitis, not elsewhere classified
  • M45.9 Ankylosing spondylitis of unspecified sites in spine
  • M45.0 Ankylosing spondylitis of multiple sites in spine
  • M46.00 Spinal enthesopathy, site unspecified
  • M46.09 Spinal enthesopathy, multiple sites in spine
  • M45.4 Ankylosing spondylitis of thoracic region
  • M46.05 Spinal enthesopathy, thoracolumbar region
  • M46.07 Spinal enthesopathy, lumbosacral region
  • M45.3 Ankylosing spondylitis of cervicothoracic region
  • M46.01 Spinal enthesopathy, occipito-atlanto-axial region
  • M45.1 Ankylosing spondylitis of occipito-atlanto-axial region
  • M46.02 Spinal enthesopathy, cervical region
  • M46.06 Spinal enthesopathy, lumbar region
  • M46.04 Spinal enthesopathy, thoracic region
  • M45.7 Ankylosing spondylitis of lumbosacral region
  • M45.2 Ankylosing spondylitis of cervical region
  • M45.6 Ankylosing spondylitis lumbar region
  • M46.08 Spinal enthesopathy, sacral and sacrococcygeal region
  • M45.5 Ankylosing spondylitis of thoracolumbar region
  • M45.8 Ankylosing spondylitis sacral and sacrococcygeal region

SNOMED


  • 372109003 Disorder of joint of spine (disorder)
  • 55146009 Sacroiliac joint inflamed (disorder)
  • 9631008 Ankylosing spondylitis (disorder)
  • 371082009 Arthritis of spine (disorder)

FAQ


  • Q: Should HLA-B27 be checked routinely in boys with back pain?
  • A: Inflammatory back, joint, or entheseal pain; family history; and exam findings should increase your suspicion for HLA-B27 " �positive disease. Detection of HLA-B27 alone should not precipitate an extensive workup because it is so common in the normal healthy population. However, the risk for developing a spondyloarthropathy is 16 times greater than in HLA-B27 " �negative individuals.
  • Q: Can affected individuals play contact sports?
  • A: This is probably not a good idea in patients with ankylosis because as the spine fuses, the risk for fracture of the spine (especially the cervical spine) increases. However, children with milder forms of disease, such as enthesitis-related arthritis, should not be discouraged.
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