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Spondyloarthropathy, Pediatric

Basics

Description

- Caution - Overdiagnosis in HLA-B27 " пїЅpositive 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 " пїЅassociated arthritis - Discitis - Pott disease (vertebral tuberculosis)

- Tumors - Trauma - Traumatic injury causing lower back pain/spasm - Herniated disc

- Metabolic - Congenital - Immunologic - Oligoarticular juvenile idiopathic arthritis

- Psychological - Feigning lower back pain/stiffness

- NSAIDs - Naproxen - Indomethacin - Diclofenac

- Disease-modifying drugs - Sulfasalazine - Methotrexate - Leflunomide - Tumor necrosis factor (TNF) inhibitors

A group of inflammatory arthritides associated with enthesitis (inflammation at the bony insertion of tendons and ligaments) and axial involvement, typically sacroiliitis пїЅ пїЅ

  • Enthesitis-related arthritis
  • Ankylosing spondylitis (AS)
  • Psoriatic arthritis
  • Inflammatory bowel disease " пїЅassociated arthropathy

Epidemiology

  • Spondyloarthritis accounts for 15 " пїЅ20% of juvenile arthritis.
  • AS typically affects adolescent boys. Much less common in blacks:HLA-B27 occurs in 70 " пїЅ90% of patients and is present in 8% of whites and 6% of blacks in the general population.

Prevalence

пїЅ пїЅ пїЅ1/10,000 white boys пїЅ пїЅ

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. пїЅ пїЅ

Etiology

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

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) tendernessIndicates 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 " пїЅA 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 flexibilityMark 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 пїЅ пїЅ

  • ESR is occasionally not elevated.
  • RF and ANA are typically negative.

Imaging

Sacroiliac views пїЅ пїЅ

  • 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

  • CautionOverdiagnosis in HLA-B27 " пїЅpositive individuals in whom other causes for joint swelling should be considered
  • InfectionReactive arthritis caused by enteric pathogens or Chlamydia speciesWhipple diseaseIntestinal bypass " пїЅassociated arthritisDiscitisPott disease (vertebral tuberculosis)
  • Tumors
  • TraumaTraumatic injury causing lower back pain/spasmHerniated disc
  • Metabolic
  • Congenital
  • ImmunologicOligoarticular juvenile idiopathic arthritis
  • PsychologicalFeigning lower back pain/stiffness
  • Miscellaneous

Treatment

Medication

  • NSAIDsNaproxenIndomethacinDiclofenac
  • Disease-modifying drugsSulfasalazineMethotrexateLeflunomideTumor 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 пїЅ пїЅ

  • 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) пїЅ пїЅ

Ongoing Care

Diet

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

Patient Education

Activity пїЅ пїЅ

  • 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. пїЅ пїЅ

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 " пїЅpositive spondyloarthropathy should not be assumed to be infectious conjunctivitis. Slit-lamp exam is required to diagnose acute anterior uveitis. пїЅ пїЅ

Additional Reading

  • Colbert пїЅ пїЅRA. Classification of juvenile spondyloarthritis: enthesitis-related arthritis and beyond. Nat Rev Rheumatol. 2010;6(8):477 " пїЅ485. пїЅ пїЅ[View Abstract]
  • Colbert пїЅ пїЅRA. Early axial spondyloarthritis. Curr Opin Rheumatol. 2010;22(5):603 " пїЅ607. пїЅ пїЅ[View Abstract]
  • Homeff пїЅ пїЅG, Burgos-Vargas пїЅ пїЅR. TNF-alpha antagonists for the treatment of juvenile-onset spondyloarthritides. Clin Exp Rheumatol. 2002;20(6)(Suppl 28):S137 " пїЅS142. пїЅ пїЅ[View Abstract]
  • Sherry пїЅ пїЅDD, Sapp пїЅ пїЅLR. Enthesalgia in childhood: site-specific tenderness in healthy subjects and in patients with seronegative enthesopathic arthropathy. J Rheumatol. 2003;30(6):1335 " пїЅ1340. пїЅ пїЅ[View Abstract]
  • Stoll пїЅ пїЅML, Lio пїЅ пїЅP, Sundel пїЅ пїЅRP, et al. Comparison of Vancouver and International League of Associations for rheumatology classification criteria for juvenile psoriatic arthritis. Arthritis Rheum. 2008;59(1):51 " пїЅ58. пїЅ пїЅ[View Abstract]
  • Tse пїЅ пїЅSM, Laxer пїЅ пїЅRM. New advances in juvenile spondyloarthritis. Nat Rev Rheumatol. 2012;8(5):269 " пїЅ279. пїЅ пїЅ[View Abstract]
  • Tse пїЅ пїЅSM, Laxer пїЅ пїЅRM, Babyn пїЅ пїЅPS, 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 " пїЅ1188. пїЅ пїЅ[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 " пїЅpositive 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 " пїЅnegative 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.