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Ankylosing Spondylitis, Emergency Medicine


Basics


Description


  • Chronic inflammatory disease, primarily affects the axial skeleton with predilection toward the spine and sacroiliac (SI) joints:
    • SI joints 100%
    • Cervical spine 75%
    • Thoracic spine 70%
    • LS spine 50%
    • Hip joints 30%
    • Shoulder joints 30%
  • Spondylitis (inflammation of vertebrae) of ankylosing spondylitis (AS) begins at the insertions of the outer fibers of the annulus fibrosus (enthesitis) of the vertebrae:
    • Ossification (syndesmophyte formation) may lead to complete fusion, ankylosis, of the vertebrae.
    • Extensive spinal involvement causes the radiographic appearance of the brittle "bamboo spine."�
  • Onset 15-35 yr of age
  • Male to female ratio is between 2:1 and 3:1.

AS patients are at 4 times the risk for fracture and paralysis compared to the general population. They are 11 times more likely to have spinal cord injuries. �

Risk Factors


Genetics
Strong genetic component. HLA-B27 is present in 80-90% of patients with AS. �

Etiology


Disease is likely triggered by environmental factors such as infection in genetically predisposed individuals. �

Diagnosis


Signs and Symptoms


  • Spinal: Low back pain with sacroiliitis is the most common presentation:
    • Inflammatory back pain, improving with movement and exercise.
    • Higher risk for serious injury from milder traumatic mechanisms.
  • Extraspinal inflammatory conditions (which may precede spinal symptoms):
    • Ocular (the most common):
      • Uveitis (25-40% occurrence). Usually acute and unilateral in onset. Can alternate eyes.
    • Cardiac:
      • Slight increased risk of CAD
      • Increased risk for valvular incompetence with prolonged course of AS.
    • Pulmonary:
      • Progressive restrictive lung disease due to limited expansion and fibrosis
    • GI:
      • 5-10% of patients with inflammatory bowel disease.
    • GU:
      • Risk for IgA nephropathy or amyloidosis. Also increased risk for NSAID nephropathy from anti-inflammatory use.
    • Enthesitis (inflammation at tendon or ligament insertion):
      • Often Achilles tendonitis or plantar fasciitis

History
  • Patients <40 yr of age with insidious onset of low back pain >3 mo, radiating into gluteal areas from SI region, and progressing to involve the entire spinal region:
    • Worse with rest and improved with mild activity. Pain in 2nd half of night waking patient from sleep
    • Women may have more cervical and extraspinal manifestations than men.
  • Possible prior history of uveitis, restrictive pulmonary disease, inflammatory bowel disease, enthesitis, or migrating or polyarthritis.

Physical Exam
  • Tenderness over SI joints elicited with direct pressure over both of patients ASIS simultaneously.
  • Dactylitis or enthesitis.
  • Flattening of the normal lumbar lordosis
  • Exaggeration of thoracic kyphosis
  • Limitation of spinal movement
  • Reduction in chest expansion

  • Patients with juvenile ankylosing spondylitis (JAS) may commonly be misdiagnosed as recurrent sprains
  • Onset of JAS is late childhood or adolescence (between 8 and 12 yr, before age 20); primarily boys.
  • JAS has a much greater predilection for extraspinal joints and entheses of the lower extremities; in addition to SI tenderness, examine for:
    • Asymmetrical pauciarthritis of the joints of the lower extremities
    • Enthesitis of the ankle, knee, or tarsal bones. Plantar fasciitis and Achilles tendonitis are often common findings.

Essential Workup


  • Exclude fracture and neurologic injury in any patient with suspected AS for any new spinal pain (even without trauma).
  • Exclude sepsis or septic joint if clinically indicated.
  • Evaluate for sacroiliitis with pelvic rock test (compression) or Patrick test (downward pressure on the knee of a flexed and externally rotated leg and the contralateral ASIS causing sacral distraction).

Diagnosis Tests & Interpretation


Lab
  • CBC may show mild leukocytosis with slight to moderate anemia and thrombocytosis.
  • BUN, creatinine, and electrolytes may be useful to assess renal involvement.
  • Erythrocyte sedimentation rate (ESR) or C-reactive protein (CRP) may be elevated, but are of limited use in the ED.
  • HLA-B27 testing can be performed by a specialist. A negative result does not rule out AS.

Imaging
  • Pelvic radiograph: Should be done in any adult patient suspected of undiagnosed ankylosing spondylitis:
    • Sacroiliitis is essential to the diagnosis of AS; this is seen initially as subchondral bony erosions on the iliac side of the SI joint, which later manifest as bony proliferation and sclerosis.
    • If plain films are negative for sacroilitis, MRI should be considered.
  • Lumbar, thoracic, and cervical spine radiographs to exclude fracture for complaint of new pain to these areas with or without trauma
  • CT should be performed to further evaluate possible fractures on plain radiographs.
  • MRI should be performed emergently on any patient with neurologic deficit.
  • Chest radiograph may show patchy inflammatory infiltrates or apical fibrosis.

Diagnostic Procedures/Surgery
  • Electrocardiogram indications:
    • Symptoms of acute coronary syndrome (slightly increased risk compared to general population for CAD)
    • Symptomatic arrhythmia:
      • AV block
  • Echocardiogram indications:
    • New murmur: Increased predilection for aortic insufficiency with AS
    • Evidence of new heart failure.

Differential Diagnosis


  • JAS:
    • Onset before age 20
    • More enthesitis and extraspinal joint involvement.
  • Reactive arthritis (formerly Reiter syndrome):
    • Arthritis, urethritis, and conjunctivitis beginning about 1 mo after an episode of urethritis or enteritis.
  • Enteropathic arthritis:
    • Crohns disease or ulcerative colitis
    • Primarily involves knee, elbow, ankle, or wrist, and usually exacerbated by flares of the bowel disease
  • Psoriatic arthritis:
    • Psoriasis rash
    • Much greater predilection for the hands and feet with higher incidence of dactylitis.
  • Septic arthritis:
    • Exclude with arthrocentesis if clinically suspected in single joint involvement.
  • Mechanical low back pain:
    • Improved with rest and exacerbated by exercise without signs of systemic inflammatory process.
  • Spinal epidural abscess:
    • More constant, unremitting, and typically associated with fever and history of IVDA or immunosuppression.
  • Neoplastic low back pain:
    • Typically in patients older than 40, more constant and unremitting, and more characteristically at night.

Treatment


Pre-Hospital


  • High risk of spinal injury from minor trauma.
  • Spinal immobilization must avoid creating further injury:
    • Cushion stabilization and scoop board in position of comfort may be a better approach than cervical collar and/or backboard.
  • Intubation difficulty
    • Cervical and TMJ restriction may limit success in all but fiberoptic techniques.
    • Consider alternative airway approaches such as LMA or bag valve mask with oral airway until definitive airway can be achieved safely (usually fiberoptic).
  • Ventilation difficulty
    • Chest wall restriction from deformity and pulmonary fibrosis
  • CPR may carry a higher likelihood of rib fractures

Ed Treatment/Procedures


  • Exclude cord compression if clinically suspected (MRI is the study of choice).
  • Exclude spinal fracture for any new spinal pain (CT may be necessary).
  • Exclude infection if clinically suspected with laboratory analysis and arthrocentesis.
  • Control pain and inflammation with NSAIDs

Medication


  • Nonselective NSAIDs:
    • Ibuprofen: 35 mg/kg/d divided QID, max. 50 mg/kg/d (adult: 300-800 mg PO TID or QID)
    • Indomethacin: 1-2 mg/kg/d divided BID or QID, max. 4 mg/kg/d (adult: 25 mg PO BID or TID)
      • Not well tolerated, especially at higher doses because of GI and CNS effects
    • Naproxen: 10 mg/kg/d divided BID, max. 1,000 mg/d (adult: 250-500 mg PO BID)
  • COX 2 inhibitors:
    • Celecoxib (adult: 100 mg-200 mg PO BID)
  • TNF-α inhibitors:
    • Adalimumab (adult: 40mg SubQ q2wk)
    • Etanercept (adult: 50mg SubQ qwk)

  • NSAIDs should be avoided in pregnancy.
    • Acetaminophen is 1st line
    • Opioids are 2nd line

NSAID use may increase risk in the elderly for cardiovascular disease, GI bleeding, renal function, and hypertension. Although effective in select patients, close follow-up is prudent. �
  • NSAIDs:
    • GI bleeding risks
      • Elderly, history of PUD, concurrent use of glucocorticoids, anticoagulants, aspirin, smoking, alcohol.
      • Consider celecoxib or adding an H2 blocker or PPI if patient is at higher risk for GI bleeding.

Second Line
Consider if NSAIDs or acetaminophen are ineffective at appropriate doses: �
  • Opioid analgesics, muscle relaxants, or low-dose steroids.

Follow-Up


Disposition


Admission Criteria
  • Acute neurologic impairment
  • Intractable pain
  • Sepsis or septic joint cannot be excluded.

Discharge Criteria
  • No serious injuries or neurologic deficit
  • Pain is manageable to the patient

Issues for Referral
  • The patient should be encouraged to obtain a medical alert bracelet.
  • Rheumatology:
    • Patients with evidence of a new diagnosis of AS should be considered for early referral to a specialist in rheumatology for immunomodulative therapy.
  • Physical medicine and rehabilitation:
    • Resting splints for inflamed joints
    • Orthoses for enthesitis (such as heel cushion inserts to rest Achilles tendon attachment)

Follow-Up Recommendations


  • Routine primary care re-evaluation within 1-2 wk to assess response to treatment.
  • Referral to a rheumatologist for immunomodulating medications.
  • Earlier follow-up in any patient with higher risk for adverse response to NSAIDs:
    • Elderly, hypertensive patients, and patients with higher GI bleeding risks.

Pearls and Pitfalls


  • Intubation is likely to be difficult and should avoid neck repositioning due to risk of C1 subluxation.
    • Consider airway adjuncts (such as LMA) until a definitive airway (usually fiberoptic) can be safely assured.
  • Immobilization must avoid creating additional injury
    • Consider cushion/tape stabilization in position of comfort rather than standard cervical collar and backboard
  • Minor traumatic injuries in AS can result in spinal fracture and possible cord injury. Maintain a high clinical suspicion.

Additional Reading


  • Baraliakos �X, van den Berg �R, Braun �J, et al. Update of the literature review on treatment with biologics as a basis for the first update of the ASAS/EULAR management recommendations of ankylosing spondylitis. Rheumatology.  2012;51(8):1378-1387.
  • Chakravarty �SD, Paget �SA. Ankylosing spondylitis: Pathogenesis, diagnosis, and therapy. Rheumatology.  2012;40:39-43.
  • Sieper �J, Braun �J. Ankylosing Spondylitis: In Clinical Practice. Britain: Springer, 2010.

See Also (Topic, Algorithm, Electronic Media Element)


http://www.spondylitis.org �

Codes


ICD9


720.0 Ankylosing spondylitis �

ICD10


  • M45.2 Ankylosing spondylitis of cervical region
  • M45.8 Ankylosing spondylitis sacral and sacrococcygeal region
  • M45.9 Ankylosing spondylitis of unspecified sites in spine
  • M45.4 Ankylosing spondylitis of thoracic region
  • M45.0 Ankylosing spondylitis of multiple sites in spine
  • M45.1 Ankylosing spondylitis of occipito-atlanto-axial region
  • M45.3 Ankylosing spondylitis of cervicothoracic region
  • M45.5 Ankylosing spondylitis of thoracolumbar region
  • M45.6 Ankylosing spondylitis lumbar region
  • M45.7 Ankylosing spondylitis of lumbosacral region
  • M45 Ankylosing spondylitis

SNOMED


  • 9631008 Ankylosing spondylitis (disorder)
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