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Lyme disease

Background

Overview

Definition
Lyme disease is a tick-borne infectious disease that principally affects the skin, nervous system, musculoskeletal system, and heart.
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Pathophysiology
Lyme disease is caused by the spirochete Borrelia burgdorferi, Borrelia afzelii, and Borrelia garinni.
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Epidemiology
The incidence of Lyme disease in the US is estimated at 15.9-381.4 cases per 100,000 person-years.
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Disease course
The host-pathogen interaction elicits an inflammatory response with the production of cytokines, matrix glycosaminoglycans, extracellular matrix proteins, antibodies resulting in erythema migrans, and dissemination of the disease leads to multiple erythema migrans, meningitis, cranial nerve palsies, carditis, and arthritis.
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Prognosis and risk of recurrence
Doxycycline, amoxicillin, and cefuroxime axetil are about 90% effective in treating erythema migrans.
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Guidelines

Key sources

The following summarized guidelines for the evaluation and management of Lyme disease are prepared by our editorial team based on guidelines from the Infectious Diseases Society of America (IDSA/ACR/AAN 2021), the Wilderness Medical Society (WMS 2021), and the American Heart Association (AHA/HRS/ACC 2019).
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Screening and diagnosis

Indications for screening, neurological manifestations: as per AAN/ACR/IDSA 2021 guidelines, test for Lyme disease in patients with epidemiologically plausible exposure to ticks infected with Borrelia burgdorferi and presenting with one or more of the following:
acute meningitis
painful radiculoneuritis
mononeuropathy multiplex including confluent mononeuropathy multiplex
acute cranial neuropathies (particularly VII, VIII, less commonly III, V, VI, and others)
evidence of spinal cord (or rarely brain) inflammation, particularly in association with painful radiculitis involving related spinal cord segments.
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  • Indications for screening (psychiatric, developmental, and behavioral disorders)

  • Indications for screening (heart diseases of unknown cause)

Diagnostic investigations

Tick testing
As per AAN/ACR/IDSA 2021 guidelines:
Send the removed tick for species identification.
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Do not test the removed Ixodes tick for Borrelia burgdorferi.
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  • Testing of asymptomatic patients

  • Testing for erythema migrans

  • Testing for Lyme neuroborreliosis

  • Testing for Lyme carditis

  • Testing for Lyme arthritis

  • Evaluation for co-infection

Medical management

Antibiotic therapy, erythema migrans
As per AAN/ACR/IDSA 2021 guidelines:
Initiate oral antibiotic therapy with doxycycline, amoxicillin, or cefuroxime axetil in patients with erythema migrans.
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Complete a 10-day course of doxycycline or a 14-day course of amoxicillin or cefuroxime axetil rather than longer treatment courses in patients with erythema migrans.
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  • Antibiotic therapy (southern tick-associated rash illness)

  • Antibiotic therapy (neurological manifestations)

  • Antibiotic therapy (Lyme carditis)

  • Antibiotic therapy (Lyme arthritis)

  • Antibiotic therapy (borrelial lymphocytoma)

  • Antibiotic therapy (acrodermatitis chronica atrophicans)

  • Corticosteroids

Therapeutic procedures

Cardiac pacing, temporary: as per AAN/ACR/IDSA 2021 guidelines, perform temporary rather than permanent pacing in patients with symptomatic bradycardia due to Lyme carditis not being manageable medically.
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  • Cardiac pacing (permanent)

Patient education

General counseling: as per WMS 2021 guidelines, implement and encourage educational programs to change behavior and lower rates of tick-borne illnesses.
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Preventative measures

Immunization
As per WMS 2021 guidelines:
Recognize that there are no vaccines for tick-borne encephalitis currently available in the US, even though vaccines such as Encepur appear effective for inducing seroconversion against tick-borne encephalitis.
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Recognize that Lyme vaccination is effective at reducing the risk of infection, although not currently available in the US.
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  • Personal protective measures

  • Tick repellents

  • Showering and cloth washing

  • Removal of attached ticks

  • Antibiotic prophylaxis

Follow-up and surveillance

Indications for referral: as per AAN/ACR/IDSA 2021 guidelines, consider referring patients who have failed one course of oral antibiotics and one course of IV antibiotics to a rheumatologist or other trained specialist for consideration of the use of DMARDs, biologic agents, intraarticular steroids, or arthroscopic synovectomy.
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  • Management of persistent symptoms