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Lyme Disease (Borrelia burgdorferi)

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2008 Case Definition

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

Clinical presentation

A systemic, tick-borne disease with protean manifestations, including dermatologic, rheumatologic, neurologic, and cardiac abnormalities. The best 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 evidence

For the purposes of surveillance, the definition of a qualified laboratory assay is (1) a positive culture for B. burgdorferi, (2) two-tier testing interpreted using established criteria [1], or (3) single-tier IgG immunoblot seropositivity interpreted using established criteria [1-4].

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.

Disease endemic to county

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.

Case classification

Confirmed: a) a case of EM with a known exposure (as defined above), or b) a case of EM with laboratory evidence of infection (as defined above) and without a known exposure or c) a case with at least one late manifestation that has laboratory evidence of infection.

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

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

Lyme disease reports will not be considered cases if the medical provider specifically states this is not a case of Lyme disease, or the only symptom listed is "tick bite" or "insect bite."

References

Centers for Disease Control and Prevention. Recommendations for test performance and interpretation from the Second National Conference on Serologic Diagnosis of Lyme Disease. MMWR MMWR Morb Mortal Wkly Rep 1995; 44:590–1.
Dressler F, Whalen JA, Reinhardt BN, Steere AC. Western blotting in the serodiagnosis of Lyme disease. J Infect Dis 1993; 167:392–400.
Engstrom SM, Shoop E, Johnson RC. Immunoblot interpretation criteria for serodiagnosis of early Lyme disease. J Clin Microbiol 1995; 33:419–27.
Centers for Disease Control and Prevention. Notice to readers: caution regarding testing for Lyme disease. MMWR Morb Mortal Wkly Rep 2005; 54:125–6.
Centers for Disease Control and Prevention. Lyme Disease — United States, 2003–2005. MMWR Morb Mortal Wkly Rep 2007; 56:573–6.