Current Concepts
A Critical Appraisal of Chronic Lyme Disease
Henry M. Feder, Jr., M.D., Barbara J.B. Johnson, Ph.D., Susan OConnell, M.D., Eugene D. Shapiro, M.D., Allen C. Steere, M.D., Gary P. Wormser, M.D., and the Ad Hoc International Lyme Disease Group*
Lyme disease, the most common tick-borne infection in the north- ern hemisphere, is a serious public health problem. In North America, it is caused exclusively by Borrelia burgdorferi sensu stricto (hereafter referred to as B. burgdorferi), whereas in Europe it is caused by B. afzelii, B. garinii, B. burgdorferi, and occasionally by other species of borrelia.1
This complex infection has a number of objective manifestations, including a char-
acteristic skin lesion called erythema migrans (the most common presentation of
early Lyme disease), certain neurologic and cardiac manifestations, and pauciarticu-
lar arthritis (the most common presentation of late Lyme disease), all of which usu-
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ally respond well to conventional antibiotic therapy. Despite resolution of the objec- tive manifestations of infection after antibiotic treatment, a minority of patients have fatigue, musculoskeletal pain, difficulties with concentration or short-term memory, or all of these symptoms. In this article, we refer to these usually mild and self-limiting subjective symptoms as postLyme disease symptoms, and if they last longer than 6 months, we call them postLyme disease syndrome.
The word chronic has been applied to Lyme disease in a wide variety of contexts and is sometimes used interchangeably with the preferred term late Lyme disease. For example, in Europe, certain late neurologic manifestations of previously untreated or inadequately treated infection, such as borrelial encephalomyelitis or long-standing meningitis, have been referred to as chronic neuroborreliosis (Table 1).1-3 In the United States, reports have described untreated patients with recurrent or persistent arthritis that lasts for up to several years, presumably because of active infection.4 The focus of this review, however, is not the objective manifestations of late Lyme disease but rather the imprecisely defined condition referred to as chronic Lyme disease. This term is used by a small number of practitioners (often self-designated as Lyme-literate physicians) to describe patients whom they believe have persistent B. burgdorferi infection, a condition they suggest requires long-term antibiotic treat- ment and may even be incurable.5 Although chronic Lyme disease clearly encom- passes postLyme disease syndrome, it also includes a broad array of illnesses or symptom complexes for which there is no reproducible or convincing scientific evi- dence of any relationship to B. burgdorferi infection. Chronic Lyme disease is used in North America and increasingly in Europe as a diagnosis for patients with persistent pain, neurocognitive symptoms, fatigue, or all of these symptoms, with or without clinical or serologic evidence of previous early or late Lyme disease.