Author:
Morgan P.Eutermoser
Description
- Most common tick-borne illness in North America
- Endemic in Northeast, Upper Midwest, and northwestern California
Etiology
- Peak April-November; 80-90% in summer months
- Spirochete Borrelia burgdorferi introduced by Ixodes tick:
- Ixodes scapularis(deer tick) most common
- Same tick that transmits anaplasmosis, babesiosis
- Pathogenesiscombination of:
- Organism-induced local inflammation
- Cytokine release
- Autoimmunity
- No person-to-person transmission
Signs and Symptoms
Stage I (early localized)
- Onset few days to a month after tick bite (arthropod transmission)
- 30-50% of patients recall tick bite
- Erythema chronicum migrans (ECM):
- Pathognomonic finding:
- Bull's-eye rash at site of bite 3-32 d after bite (50-75% of infected patients develop rash)
- Maculopapular, irregular expand ing annular lesion:
- Single or multiple
- Central clearing with red outer border
- Diameter >5 cm
- Usually painless and nonpruritic
- Regional adenopathy
- Low-grade intermittent fever
- Headache
- Myalgia
- Arthralgias
- Fatigue
- Malaise
Stage II (secondary, early disseminated)
- Days to weeks after tick bite
- Intermittent and fluctuating symptoms with eventual disappearance
- Triad of aseptic meningitis, cranial neuritis, and radiculoneuritis:
- Facial (Bell's) palsy most common cranial neuritis and can be bilateral
- May present without rash
- Prognosis generally good
- Cardiac:
- Tachycardia
- Bradycardia
- Atrioventricular block
- Myopericarditis
- Ophthalmic findings (optic neuritis, keratitis, episcleritis, conjunctivitis)
- Lymphocytic meningitis
- Findings as in Stage I are often present
Stage III (tertiary, late disseminated)
- Onset >1 yr after disease onset
- Neurologic symptoms: Bannwarth syndrome: nerve pain, impairment of motor or sensory functions, Lyme encephalopathy
- Acrodermatitis chronica atrophicans:
- Extensor surfaces of extremities, especially lower leg
- Initial edematous infiltration evolving to atrophic lesions
- Resembles scleroderma
- Arthritis:
- Brief arthritis attacks
- Monoarthritis
- Oligoarthritis
- Occasionally migratory
- Most common joints (descending order):
Other
- GI:
- Hepatitis
- Right upper quadrant pain
- Ocular:
- Keratitis
- Uveitis
- Iritis
- Optic neuritis
- Jarisch-Herxheimer reaction:
- Worsening of symptoms a few hours after treatment initiated
- More common in patients with multiple ECM lesions
Pediatric Considerations |
- More likely than adults to be febrile
- Only 50% of children with arthralgias have history of ECM
- Facial palsy is accompanied by aseptic meningitis in 1/3
- Asymptomatic cardiac involvement with abnormal ECGs
- Appropriately treated children have excellent prognosis for unimpaired cognitive functioning
- Untreated children may have keratitis
|
Pregnancy Prophylaxis |
No clear evidence that Lyme disease during pregnancy causes harm to fetus |
History
- History of tick bite
- Travel to endemic areas
- Flu-like illness in the summer
Physical Exam
- Rash
- Joint, cardiac, and neurologic findings in later organ involvement
Essential Workup
- Clinical diagnosis:
- Presence of ECM obviates serologic tests
- Careful search for tick
- Lumbar puncture when meningeal signs
- Arthrocentesis for acute arthritis
- ECG
Diagnostic Tests & Interpretation
Lab
- CBC:
- Leukocytosis
- Anemia
- Thrombocytopenia
- ESR:
- >30 mm/hr
- Most common lab abnormality
- Electrolytes, BUN, creatinine, glucose
- Liver function tests:
- Elevated liver enzymes (γ-glutamyl transferase most common)
- Culture:
- CSF:
- Pleocytosis
- Elevated protein
- Obtain CSF spirochete antibodies
- Special tests:
- Serology:
- Obtain ELISA or immunofluorescence assay
- Western blot (IgM if symptoms <30 d and IgG if symptoms >30 d) when disease is suggested without ECM lesion
- Antibodies may persist for months to years
- Positive serology or previous Lyme disease does not ensure protective immunity
- Polymerase chain reaction assay:
- Highly specific and sensitive
- Not available for routine use
- Joint fluid:
- Cryoglobulin increased 5-fold compared with serum
- Joint films may show soft tissue, cartilaginous, osseous changes
Differential Diagnosis
- Other tick-borne illnesses:
- Deer tick usually larger (1 cm) than Ixodid ticks (1-2 mm)
- Rocky Mountain spotted fever
- Tularemia
- Relapsing fever
- Colorado tick fever
- Tick-bite paralysis
- STARI: Southern tick-associated rash illness
- Babesiosis
- Ehrlichiosis
- Rheumatic fever:
- Rash of erythema marginatum
- Temporomand ibular joint arthritis more common than in Lyme disease
- Valvular involvement rather than heart block
- Chorea may be isolated finding
- Viral meningitis
- Syphilis
- Septic arthritis
- Parvovirus B19 infection - polyarticular arthritis
- Infectious endocarditis
- Juvenile rheumatoid arthritis
- Reiter syndrome
- Brown recluse spider bite
- Fibromyalgia
- Chronic fatigue syndrome
Initial Stabilization/Therapy
- IV access for neurologic and cardiac involvement
- Cardiac monitoring
- Temporary pacemaker, if heart block
ED Treatment/Procedures
- Remove tick:
- Infection/transmission unlikely if tick attached less than 36 hr
- Disinfect site
- With blunt instrument, grasp tick close to skin and pull upward with gentle pressure
- Make sure entire tick removed
- Stage I:
- Stage II:
- Oral therapy for isolated Bell palsy and mild involvement:
- Amoxicillin with probenecid (30 d) or doxycycline (avoid if pregnant or ≤8 yr old; 10-21 d)
- Parenteral therapy for more severe involvement (meningitis, carditis, severe arthritis):
- For isolated facial palsy treat for 14-21 d
- For arthritis, treat for 28 d
- For AV heart block or carditis treat for 14-21 d
- For meningitis/encephalitis treat for 14-28 d
- Stage III:
- Parenteral therapy:
- Penicillin G, cefotaxime (14-21 d), or ceftriaxone (14-28 d)
Medication
First Line
- Doxycycline: 100 mg PO b.i.d for 14-21 d for children ≥8 yr and adults (except if pregnant)
- Amoxicillin: 500 mg (peds: 50 mg/kg/24 hr) PO t.i.d for those <8 yr of age or unable to tolerate
- Ceftriaxone: 2 g (peds: 100 mg/kg/24 hr) IV daily (1st line for late-term disease especially due to ease of once-daily dosing)
Second Line
- Azithromycin: 500 mg PO daily
- Cefuroxime axetil, 500 mg b.i.d (all ages)
- Cefotaxime: 2 g (peds: 100-150 mg/kg/24 hr) IV q8h
- Penicillin G: 20-24 million units IV q4
- Aspirin as adjunctive therapy for cardiac involvement
- NSAIDs for arthritis or arthralgias
Disposition
Admission Criteria
- Meningoencephalitis
- Telemetry/ICU admission for carditis
Discharge Criteria
Patients treated with oral therapy
- Protective clothing: Light colors, pants tucked into socks, long-sleeves. DEET spray. Tick checks when person/animal returns indoors
- Vaccine (Lymerix) for prevention of disease:
- A recombinant surface protein
- For persons in high/moderate risk areas or travelers to endemic areas
- Withdrawn from the U.S. in 2002 due to allegation of autoimmune side effects. Not proven
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- American Academy of Pediatrics: Red Book 2018-2021. Report of the Committee on Infectious Diseases. 31st ed.Itasca, IL; 2018.
- HalperinJJ, BakerP, WormserGP. Common misconceptions about Lyme disease . Am J Med. 2013;164:264.
- KowalskiTJ, TataS, BerthW, et al. Antibiotic treatment duration and long-term outcomes of patients with early lyme disease from a lyme disease-hyperendemic area . Clin Infect Dis. 2010;50:512-520.
- MarquesAR. Lyme disease: A review . Curr Allergy Asthma Rep. 2010;10:13-20.
- MooreA, NelsonC, MolinsC, et al. Current guidelines, common clinical pitfalls and future directions for laboratory diagnosis of Lyme disease, United States . Emerging Infect Dis. 2016;22:1694.
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See Also (Topic, Algorithm, Electronic Media Element)
The authors gratefully acknowledge Moses S. Lee for his contribution to the previous edition of this chapter.