MEDICAL WRITINGS
Misconceptions about Lyme Disease: Confusions
Hiding behind Ill-Chosen Terminology
Leonard H. Sigal, MD
Pages 413-419
Ann Intern Med. 2002;136:413-419.
"The beginning of wisdom is to call things by their right names."
Ancient Chinese proverb
Nomenclature influences perceptions of reality and frames ensuing
discussions.
Imprecision contributes to misinterpretation of observations and
studies, altering clinicians' approaches. The impact of imprecision
and novel reinterpretation of terminology
can be seen in the Lyme disease debate. A quarter century after its
initial description, a review of the terminology contributing to
confusion about Lyme disease is needed.
Lyme disease is treatable and curable with antibiotics (1-4),
especially if treated promptly, usually with an excellent long-term
prognosis. The term "promptly" taken out of context suggests one
must treat without any delay. In fact, even untreated patients have
a good prognosis. A 10- to 20-year follow-up of patients at Yale's
Lyme Disease Clinic from 1976 to 1983, many of whom were not treated
for early Lyme disease, shows that the patients with erythema
migrans did not differ from normal controls in current symptoms,
physical findings, results of neuropsychological testing, or
responses to the Short-Form
36 Health Assessment Questionnaire (5). However, significant
long-term sequelae occurred in patients with untreated {*filter*} palsy
who probably had disseminated Lyme disease at initial evaluation and
probably required intravenous therapy (5).
"Chronic Lyme Disease": A Term in Search of Definition
Despite this generally optimistic picture, claims of persisting
infection and antibiotic unresponsiveness have contributed to
anxiety. "Chronic Lyme disease" (6, 7) is a common clinical
diagnosis in some geographic areas (8, 9) and is based on thinking
that is at odds with scientifically validated findings. No objective
physical findings or unique historical features define "chronic
Lyme disease," a term used by support groups and their few
physician allies, not the academic medical community. Although the
subject of much debate, "chronic Lyme disease" is not well defined.
The term is usually applied to patients with symptoms, such as fatigue,
achiness, malaise, and
difficulty with concentration and memory, after treatment of documented Lyme
disease
or illnesses thought to be Lyme disease, or in patients without
preceding illness (9). Once the term Lyme disease is applied, it is
Lyme disease, forever and irrefutably, a diagnosis often
"reaffirmed" by cross-referral between "Lyme literate" physicians.
Many patients receive repeated courses of antibiotics, with
transient or waning responses, leading to more or a combination of
antibiotics. Occasionally, this course of treatment can go on for
years, with little relief. Originally, "chronic" was used in the
context of Lyme arthritis, which, in the era before it was
determined that Lyme disease was responsive to antibiotics,
persisted for years, finally resolving spontaneously (10).
Antibiotics are of proven value for Lyme arthritis (11); treatment
of early Lyme disease usually prevents arthritis.
A Nexis-Lexis review reveals that "chronic" was first applied to
Lyme disease in 1985: Lyme disease was "a potentially chronic and
debilitating illness transmitted by tick bites" (12). In 1986,
"chronic" referred to outcomes if antibiotics were not administered
(13). A letter to the editor by Drs. Falvo and Nadelman urged more
support for research: Lyme disease could cause "birth defects, fetal
death, unilateral blindness, and chronic debilitating arthritis"
(14) (the first and second occurrences still unproven, the third
rare, and the last well reported). An adjective appropriate for
Lyme arthritis before the identification of antibiotic
responsiveness (1, 2, 10, 11) was used to describe patients not
responding to antibiotics, suggesting that antibiotics do not kill
Borellia burgdorferi.
Some Internet sites, support and advocacy groups, and some
clinicians claim that the truth is deliberately being obscured: that
"chronic Lyme disease" is far more common than the authorities
allow us to know; antibiotics are often not curative; infection can
be controlled only by long-term antibiotic therapy, often more than
insurance companies allow; serologic tests are inaccurate and often
yield falsely negative results, thereby incorrectly discouraging
diagnosis; the prognosis is not nearly so rosy as "they" (the
nefarious academic experts) claim; many lives have been ruined; and
many people have
died (8-10). None of these claims is supported by scientific
medical literature, yet they disseminate regularly, acquiring
verity by their repetition.
By focusing on terminology, we may understand how some confusion has been
promulgated and exacerbated. Insight may aid in clarification and be
useful in addressing non-Lyme disease areas of contention. The
contents of Table 1 are probably acceptable to most researchers and
clinicians who think about Lyme disease. Beyond these "absolute"
facts lie concepts involving terms such as "very unlikely," "has
been reported," "usually," or "in most patients"modifiers describing
"shades of gray." Physician-scientists are good at communicating
facts, but "shades of gray" are often difficult to convey; the more
precise one tries to be about the limits of our knowledge, the more
doubts are planted, and the more misinterpretations occur. This is
the root of endless debate, the home of a Cartesian dualism of sorts.
Rationalists versus Empiricists
The opposing sides in this debate about the true nature of Lyme disease can
be
described as "rationalists" and "empiricists." Rationalists use
scientific studies, both clinical and molecular, to develop models
of disease and appropriate diagnostic and therapeutic responses.
Empiricists base models on community events, developing diagnostic
and management schemas that are compatible with observations, but
often at odds with scientific conclusions. In conveying their
message, empiricists often adopt terminology that contradicts the
terminology's intended meaning. Most published clinical and basic
research on Lyme disease is from rationalists, physicians searching
for objective evidence of infection. The empiricists' ranks
include support groups and
physicians in practices devoted to the care of patients with
Quote:
>"chronic Lyme disease," who are given a diagnosis and are treated on
the basis of nonspecific symptoms, such as fatigue, cognitive
dysfunction, and pain, rather than objective evidence of infection.
Empiricists "listen to the patient" rather than follow the advice of
Quote:
>scientific studies, as if these were mutually exclusive.
Rationalists fear that physicians, with the help of misinterpreted
test results, occasionally misdiagnose serious illnesses as "chronic
Lyme
disease." Empiricists often diagnose without formulating a
differential diagnosisthis is Lyme disease. Some call these two
opposing views "two schools of thought," but I prefer to call them
proponents of "reality" and "alternative reality." The sage of
Baltimore, H.L. Mencken, could have been referring to this divide
when he penned his introduction to the first American edition of The
Antichrist by Nietzsche: "The majority of men prefer delusion to
truth. It is easier to grasp. Above all, it fits more snugly into a
universe of false appearances ... ."
"Delusions" may satisfy needs; facts offer cold comfort to the
sufferer. When false appearances assume the cloak of "reality,"
"alternative reality" is established.
The debate between these two groups includes diagnosing the illness,
use of testing in diagnosis and management, duration and forms of
therapy, prognosis, and defining a cure. Inattention to details and
facts, their manipulation, and incorrect citation have fed this
occasionally rancorous disputation (12), further confusing most
clinicians and patients on the sidelines and causing the suffering
of innocent patients and families.
Lyme Disease as "The Great Imitator"
The term "The Great Imitator" as applied to Lyme disease (an attempt
to form an analogy with another spirochetal disease, syphilis [19])
contributed to confusion. The comparison was meant not to denote
clinical similarities between these diseases but to suggest that, as
with syphilis in a previous era, Lyme disease included a broad range
of findings and mimicked other diseases. However, it soon became
clear that most cases of Lyme disease are recognizable in a
well-described spectrum (20-25), the rare exceptions being, by
definition, outliers (26). Most patients have objective
abnormalities (2). Used correctly, testing is helpful: Immunologic
(antibodies in serum and cerebrospinal and
synovial fluids) (27), molecular biological (polymerase chain
reaction identification of specific DNA), electrophysiologic (heart
and neurologic), and neuropsychological (28) tests can support the
diagnosis (8). Instead, "The Great Imitator" was misinterpreted as
suggesting that Lyme disease routinely mimics and is mimicked by
many other diseases. Some empiricists believed Lyme disease was
difficult to explicitly diagnose and had to be part of the
differential diagnosis of all problems of all diseases it might
imitate (11).
Lyme disease is often considered in many patients whose symptoms do
not explicitly suggest Lyme disease and who receive that diagnosis
merely because no other diseases can be explicitly diagnosed.
Centers for Disease Control and Prevention Criteria: Use and Misuse
The belief that Lyme disease is often overlooked is expressed as
dissatisfaction with (even anger at) the Centers for Disease
Control and Prevention (CDC) surveillance criteria as dangerous
stricture, inexplicably designed to minimize reports of "accepted"
cases (29). The criteria were designed for surveillance (and are
useful as entry criteria for studies) but were not meant for
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Sigal: "Misconceptions about LD" '02