Lyme Disease (Borrelia burgdorferi)
2011 Case Definition

Background

This surveillance case definition was developed for national reporting of Lyme disease; it is not intended to be used in clinical diagnosis.

Clinical Description

A systemic, tick-borne disease with protean manifestations, including dermatologic, rheumatologic, neurologic, and cardiac abnormalities. The most common clinical marker for the disease is erythema migrans (EM), the initial skin lesion that occurs in 60%-80% of patients.

For purposes of surveillance, EM is defined as a skin lesion that typically begins as a red macule or papule and expands over a period of days to weeks to form a large round lesion, often with partial central clearing. A single primary lesion must reach greater than or equal to 5 cm in size across its largest diameter. Secondary lesions also may occur. Annular erythematous lesions occurring within several hours of a tick bite represent hypersensitivity reactions and do not qualify as EM. For most patients, the expanding EM lesion is accompanied by other acute symptoms, particularly fatigue, fever, headache, mildly stiff neck, arthralgia, or myalgia. These symptoms are typically intermittent. The diagnosis of EM must be made by a physician. Laboratory confirmation is recommended for persons with no known exposure.

For purposes of surveillance, late manifestations include any of the following when an alternate explanation is not found:

• Musculoskeletal system. Recurrent, brief attacks (weeks or months) of objective joint swelling in one or a few joints, sometimes followed by chronic arthritis in one or a few joints. Manifestations not considered as criteria for diagnosis include chronic progressive arthritis not preceded by brief attacks and chronic symmetrical polyarthritis. Additionally, arthralgia, myalgia, or fibromyalgia syndromes alone are not criteria for musculoskeletal involvement.
• Nervous system. Any of the following, alone or in combination: lymphocytic meningitis; cranial neuritis, particularly facial palsy (may be bilateral); radiculoneuropathy; or, rarely, encephalomyelitis. Encephalomyelitis must be confirmed by demonstration of antibody production against Borrelia burgdorferi in the cerebrospinal fluid (CSF), evidenced by a higher titer of antibody in CSF than in serum. Headache, fatigue, paresthesia, or mildly stiff neck alone, are not criteria for neurologic involvement.
• Cardiovascular system. Acute onset of high-grade (2nd-degree or 3rd-degree) atrioventricular conduction defects that resolve in days to weeks and are sometimes associated with myocarditis. Palpitations, bradycardia, bundle branch block, or myocarditis alone are not criteria for cardiovascular involvement.

Laboratory Criteria For Diagnosis

For the purposes of surveillance, the definition of a qualified laboratory assay is

• Positive Culture for B. burgdorferi, OR
• Two-tier testing interpreted using established criteria¹, where:
  • Positive IgM is sufficient only when ≤30 days from symptom onset
  • Positive IgG is sufficient at any point during illness
• Single-tier IgG immunoblot seropositivity using established criteria.^1-4
• CSF antibody positive for B. burgdorferi by Enzyme Immunoassay (EIA) or Immunofluorescence Assay (IFA), when the titer is higher than it was in serum

Exposure

Exposure is defined as having been (less than or equal to 30 days before onset of EM) in wooded, brushy, or grassy areas (i.e., potential tick habitats) in a county in which Lyme disease is endemic. A history of tick bite is not required.

Endemicity

A county in which Lyme disease is endemic is one in which at least two confirmed cases have been acquired in the county or in which established populations of a known tick vector are infected with B. burgdorferi.

Suspected

• A case of EM where there is no known exposure (as defined above) and no laboratory evidence of infection (as defined above), OR
• A case with laboratory evidence of infection but no clinical information available (e.g., a laboratory report).

Probable

Any other case of physician-diagnosed Lyme disease that has laboratory evidence of infection (as defined above).

Confirmed

• A case of EM with a known exposure (as defined above), OR
• A case of EM with laboratory evidence of infection (as defined above) and without a known exposure OR
• A case with at least one late manifestation that has laboratory evidence of infection.

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