Latest Review Date: September 2023

Category: Pharmacology                                            

POLICY:

I. An initial 2-4 week course of intravenous antibiotic therapy for Lyme disease may be considered MEDICALLY NECESSARY AND APPROPRIATE when the following criteria are met:

• Diagnosis of Lyme disease has been made by one of the following:
  • Clinical findings of erythema migrans in early infection when patient has had a known tick bite or has been in an area known for Lyme disease and is experiencing signs or symptoms characteristic of Lyme disease; OR
  • Positive or indeterminate enzyme immunoassay (EIA or ELISA) followed by either of the following:
    1. Positive immunoblot (Western Blot); or
    2. Second positive enzyme immunoassay (EIA or ELISA);

 OR

• Polymerase chain reaction (PCR)-based direct detection of B. burgdorferi in CSF samples in lieu of serologic documentation of infection in patients with known exposure and with a short duration of neurologic symptoms (<14 days) during the window between exposure and production of detectable antibodies; 

AND

• Patient has one or more of the following:
  • Neuroborreliosis as evidenced by one of more of the following:

1. Lymphocytic meningitis, with documented cerebrospinal fluid (CSF) abnormalities; or
2. Encephalitis or encephalomyelitis with documented CSF abnormalities; or
3. Cranial neuropathy other than uncomplicated cranial nerve palsy with documented CSF abnormalities; or
4. Radiculopathy; or
5. Polyneuropathy;

OR

• Lyme carditis is suspected based on presence of one or more of the following:

1. A high degree of atrioventricular block; or
2. A PR interval of greater than 0.3 seconds; or
3. Myopericarditis;

OR

• Lyme arthritis with persistent, recurrent, or worsening joint swelling that has not resolved after a recommended course of oral antibiotic therapy.

II. One repeat 2 to 4-week course of IV antibiotic therapy may be considered MEDICALLY NECESSSARY AND APPROPRIATE when ALL of the following criteria are met:

• Criteria in section I for the initial course of IV antibiotic therapy are met; AND
• Laboratory values confirming Lyme disease have been obtained within the past 3 months; AND
• The patient has completed the initial course of IV antibiotic therapy; AND
• One or more of the following are met:
  • The initial infection has relapsed; OR
  • Organ damage as a result of Lyme disease has progressed; OR
  • Finding of a new focus or type of organ damage.

III. Intravenous antibiotic therapy for Lyme disease is considered EXPERIMENTAL/INVESTIGATIVE for all other indications including, but not limited to, the following due to a lack of evidence demonstrating an impact on improved health outcomes:

• A diagnosis of Lyme disease has been made using tests not included in section I. These include the following:
  • Patients with a positive EIA or ELISA test unconfirmed by a second positive test or Western blot test
  • Direct detection of B. burgdorferi in urine samples
  • Urine antigen assay
  • 31kDa Epitope Test for IgM
  • B. burgdorferi antibody index testing or culture
  • C6 peptide ELISA assay
  • CD57+ lymphocyte counts
  • Determination of levels of the B lymphocyte chemoattractant CXCL13
  • Outer surface protein A (OspA) antigen test
  • Genotyping or phenotyping of B. burgdorferi
  • IgA screen (IFA)
  • Lyme dot blot assay for antigen
  • Provocative testing (testing for B. burgdorferi  after antibiotic provocation)
  • Serum borreliacidal assay
  • T-cell proliferation response assay
  • PCR testing, except as described in section I including:

1. Quantification of Lyme disease
2. Repeat PCR-based direct detection of B. burgdorferi in the following situations:

• As a justification for continuation of IV antibiotics beyond 4 weeks in patients with persistent symptoms; or
• As a technique to follow therapeutic response.

• Repeat or prolonged courses beyond two (2-4 week) courses of IV antibiotic therapy.
• In patients with neurological symptoms who are seronegative for Lyme disease in the absence of CSF antibodies.
• Cranial nerve palsy (e.g., Bell's palsy) without clinical evidence of meningitis.
• Patients with symptoms consistent with chronic fatigue syndrome or fibromyalgia, in the absence of objective clinical or laboratory evidence for Lyme disease as defined in section I.
• Initial therapy in patients with Lyme disease.
• Patients with an isolated positive serologic test in the setting of multiple negative serologic studies.
• Patients with vague systemic symptoms without supporting serologic or CSF studies.
• Patients with chronic (≥6 months) subjective symptoms ("post-Lyme syndrome") after receiving recommended treatment regimens for documented Lyme disease.

Note:

Documentation supporting the medical necessity criteria described in the policy must be included in the prior authorization, when prior authorization is required. In addition, the following documentation must also be submitted:

• Written report documenting clinical findings and/or laboratory results including testing method(s), date(s) and results related to Lyme disease diagnosis; AND
• For an initial 2-4-week course, clinical notes describing:

1. Complication(s) of Lyme disease to be treated by IV antibiotic therapy; AND
2. Related test results, if applicable.

• For a repeat 2-4-week course, clinical notes describing:

1. Individual met all criteria for an initial 2-4-week course of IV antibiotic treatment; AND
2. Individual has completed the initial 2-4-week course of IV antibiotic therapy; AND
3. Clinical indications for repeat course (i.e., relapse of initial infection, organ damage due to Lyme has progressed, new focus of organ damage).

This policy is designed to address medical guidelines that are appropriate for the majority of individuals with a particular disease, illness, or condition. Each person's unique clinical circumstances may warrant individual consideration, based on review of applicable medical records.

DESCRIPTION OF PROCEDURE OR SERVICE:

Lyme disease is a multisystem inflammatory disease caused by the spirochete Borrelia burgdorferi and transmitted by the bite of an infected ixodid tick. The disease is characterized by stages, beginning with localized infection of the skin (erythema migrans), followed by dissemination to many sites. Oral antibiotics usually are adequate for treatment of Lyme disease, but in some cases, intravenous antibiotics may be appropriate. Diagnostic testing for Lyme disease is challenging and can lead to overdiagnosis and overtreatment.

Diagnosis of Lyme disease in early infection (e.g., up to 30 days post tick exposure) may be made based on the presence erythema migrans (EM), a painless expanding rash that often grows to look like a bull’s eye. If EM is present and the patient has recently had a tick bite or was in an area known for Lyme disease and is experiencing symptoms of Lyme disease, additional testing is not required. Symptoms in early Lyme disease may include fever, chills, headache, muscle aches, joint pain, neck pain, Bell’s palsy, heart palpitations or dizziness.

U.S. Centers for Disease Control and Prevention (CDC) Recommendations for Serum Testing

The U.S. Centers for Disease Control and Prevention (CDC) currently recommends a two-step process when testing blood for evidence of antibodies against the Lyme disease bacteria. In July 2019, the Food and Drug Administration (FDA) cleared several Lyme disease serologic assays with new indications for use based on a modified two-test methodology, with a sensitive enzyme immunoassay (EIA or ELISA) as the first step followed by a second confirmatory EIA or ELISA. Previously, a western immunoblot test (Western blot) was performed as a second step for specimens yielding positive or equivocal results.

CURRENT CODING:

POLICY HISTORY:

Reviewed and posted July 1, 2021.

Medical Policy Group, March 2022 (2): Quarterly coding update, effective 4/1/22, added new code 0316U to the current coding section.

Medical Policy Group, October 2022 (2): Annual review completed. No change to policy intent.

Medical Policy Group, October 2023 (5): Policy Section updated to include Outer surface protein A (OspA) antigen test to list of experimental/investigative tests. No change to policy intent.

This medical policy is not an authorization, certification, explanation of benefits, or a contract. Eligibility and benefits are determined on a case-by-case basis according to the terms of the member’s plan in effect as of the date services are rendered. All medical policies are based on (i) research of current medical literature and (ii) review of common medical practices in the treatment and diagnosis of disease as of the date hereof. Physicians and other providers are solely responsible for all aspects of medical care and treatment, including the type, quality, and levels of care and treatment.

This policy is intended to be used for adjudication of claims (including pre-admission certification, pre-determinations, and pre-procedure review) in Blue Cross and Blue Shield’s administration of plan contracts.

The plan does not approve or deny procedures, services, testing, or equipment for our members. Our decisions concern coverage only. The decision of whether or not to have a certain test, treatment or procedure is one made between the physician and his/her patient. The plan administers benefits based on the member’s contract and corporate medical policies. Physicians should always exercise their best medical judgment in providing the care they feel is most appropriate for their patients. Needed care should not be delayed or refused because of a coverage determination.

As a general rule, benefits are payable under health plans only in cases of medical necessity and only if services or supplies are not investigational, provided the customer group contracts have such coverage.

The following Association Technology Evaluation Criteria must be met for a service/supply to be considered for coverage:

1. The technology must have final approval from the appropriate government regulatory bodies;

2. The scientific evidence must permit conclusions concerning the effect of the technology on health outcomes;

3. The technology must improve the net health outcome;

4. The technology must be as beneficial as any established alternatives;

5. The improvement must be attainable outside the investigational setting.

Medical Necessity means that health care services (e.g., procedures, treatments, supplies, devices, equipment, facilities or drugs) that a physician, exercising prudent clinical judgment, would provide to a patient for the purpose of preventing, evaluating, diagnosing or treating an illness, injury or disease or its symptoms, and that are:

1. In accordance with generally accepted standards of medical practice; and

2. Clinically appropriate in terms of type, frequency, extent, site and duration and considered effective for the patient’s illness, injury or disease; and

3. Not primarily for the convenience of the patient, physician or other health care provider; and

4. Not more costly than an alternative service or sequence of services at least as likely to produce equivalent 

      therapeutic or diagnostic results as to the diagnosis or treatment of that patient’s illness, injury or disease.