Doctor123.org

Ankylosing Spondylitis, Emergency Medicine

Doctor123.org

Ankylosing Spondylitis, Emergency Medicine

Basics

Description

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. пїЅ

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

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

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. пїЅ

Consider if NSAIDs or acetaminophen are ineffective at appropriate doses: пїЅ

http://www.spondylitis.org пїЅ

  • 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."пїЅ
  • Onset 15-35 yr of age
  • Male to female ratio is between 2:1 and 3:1.
  • SI joints 100%
  • Cervical spine 75%
  • Thoracic spine 70%
  • LS spine 50%
  • Hip joints 30%
  • Shoulder joints 30%
  • Ossification (syndesmophyte formation) may lead to complete fusion, ankylosis, of the vertebrae.
  • Extensive spinal involvement causes the radiographic appearance of the brittle "bamboo spine."пїЅ

Risk Factors

Etiology

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 CADIncreased risk for valvular incompetence with prolonged course of AS.Pulmonary:Progressive restrictive lung disease due to limited expansion and fibrosisGI: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
  • Inflammatory back pain, improving with movement and exercise.
  • Higher risk for serious injury from milder traumatic mechanisms.
  • Ocular (the most common):Uveitis (25-40% occurrence). Usually acute and unilateral in onset. Can alternate eyes.
  • Cardiac:Slight increased risk of CADIncreased 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
  • Uveitis (25-40% occurrence). Usually acute and unilateral in onset. Can alternate eyes.
  • Slight increased risk of CAD
  • Increased risk for valvular incompetence with prolonged course of AS.
  • Progressive restrictive lung disease due to limited expansion and fibrosis
  • 5-10% of patients with inflammatory bowel disease.
  • Risk for IgA nephropathy or amyloidosis. Also increased risk for NSAID nephropathy from anti-inflammatory use.
  • Often Achilles tendonitis or plantar fasciitis
  • 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 sleepWomen 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.
  • 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.
  • 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 extremitiesEnthesitis of the ankle, knee, or tarsal bones. Plantar fasciitis and Achilles tendonitis are often common findings.
  • 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

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

Differential Diagnosis

  • JAS:Onset before age 20More 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 colitisPrimarily involves knee, elbow, ankle, or wrist, and usually exacerbated by flares of the bowel disease
  • Psoriatic arthritis:Psoriasis rashMuch 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.
  • Onset before age 20
  • More enthesitis and extraspinal joint involvement.
  • Arthritis, urethritis, and conjunctivitis beginning about 1 mo after an episode of urethritis or enteritis.
  • Crohns disease or ulcerative colitis
  • Primarily involves knee, elbow, ankle, or wrist, and usually exacerbated by flares of the bowel disease
  • Psoriasis rash
  • Much greater predilection for the hands and feet with higher incidence of dactylitis.
  • Exclude with arthrocentesis if clinically suspected in single joint involvement.
  • Improved with rest and exacerbated by exercise without signs of systemic inflammatory process.
  • More constant, unremitting, and typically associated with fever and history of IVDA or immunosuppression.
  • 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 difficultyCervical 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 difficultyChest wall restriction from deformity and pulmonary fibrosis
  • CPR may carry a higher likelihood of rib fractures
  • Cushion stabilization and scoop board in position of comfort may be a better approach than cervical collar and/or backboard.
  • 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).
  • Chest wall restriction from deformity and pulmonary fibrosis

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 effectsNaproxen: 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-О± inhibitors:Adalimumab (adult: 40mg SubQ q2wk)Etanercept (adult: 50mg SubQ qwk)
  • 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)
  • Not well tolerated, especially at higher doses because of GI and CNS effects
  • Celecoxib (adult: 100 mg-200 mg PO BID)
  • Adalimumab (adult: 40mg SubQ q2wk)
  • Etanercept (adult: 50mg SubQ qwk)
  • NSAIDs should be avoided in pregnancy.Acetaminophen is 1st lineOpioids are 2nd line
  • Acetaminophen is 1st line
  • Opioids are 2nd line
  • NSAIDs:GI bleeding risksElderly, 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.
  • GI bleeding risksElderly, 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.
  • 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.
  • Opioid analgesics, muscle relaxants, or low-dose steroids.

Follow-Up

Disposition

  • Acute neurologic impairment
  • Intractable pain
  • Sepsis or septic joint cannot be excluded.
  • No serious injuries or neurologic deficit
  • Pain is manageable to the patient
  • 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 jointsOrthoses for enthesitis (such as heel cushion inserts to rest Achilles tendon attachment)
  • Patients with evidence of a new diagnosis of AS should be considered for early referral to a specialist in rheumatology for immunomodulative therapy.
  • 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.
  • 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 injuryConsider 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.
  • Consider airway adjuncts (such as LMA) until a definitive airway (usually fiberoptic) can be safely assured.
  • Consider cushion/tape stabilization in position of comfort rather than standard cervical collar and backboard

Additional Reading

  • Baraliakos пїЅX, van den Berg пїЅR, Braun пїЅJ, 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 пїЅSD, Paget пїЅSA. Ankylosing spondylitis: Pathogenesis, diagnosis, and therapy. Rheumatology. 2012;40:39-43.
  • Sieper пїЅJ, Braun пїЅJ. Ankylosing Spondylitis: In Clinical Practice. Britain: Springer, 2010.

See Also (Topic, Algorithm, Electronic Media Element)

Codes

ICD9

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)