84 Lyme Borreliosis (Lyme Disease)
Lyme borreliosis is a tick-borne spirochetal bacterial disease caused by Borrelia burgdorferi. Lyme borreliosis is a systemic illness with potential involvement of the skin, neurological, cardiac, and articular systems.
It can mimic a variety of other diseases such as juvenile rheumatoid arthritis, multiple sclerosis, and syphilis.Epidemiology
The disease is the most frequently diagnosed tick- transmitted illness in the United States. The three major geographic loci of Lyme disease in the United States are the Northeast and middle Atlantic coastal regions, the upper Midwest, and the Pacific Northwest. The disease is found in Europe, Australia, the former U.S.S.R., China, Japan, and several African countries. The vector of Lyme disease is the tick, Ixodes dammini, or related Ixodes ticks such as pacificus, scapularis, or ricinus. B. burgdorferi has been found in other ticks such as the Dermacentor variabilis, Amblyomma. americanum, and Haema- physalis Ieporispalustris; however, transmission has not been proved.
B. burgdorferi has been found in horseflies, deerflies, and mosquitoes, but proof that these insects are possible secondary vectors has not been established. The reservoirs of B. burgdorferi occur in animals parasitized by infected ticks. The Ixodes tick is a three-host tick with a life cycle of 2 years. Adult ticks mate and feed primarily on deer in late fall; the female deposits eggs on the ground, which produce larvae that are active late the following summer. The tiny larvae obtain a blood meal from infected rodents such as white-footed mice, shrews, chipmunks, and squirrels, which are primary reservoirs for B. burgdorferi. Ground foraging birds are also important hosts for the larvae and nymphs. After a blood meal, the larvae molt to a nymphal form, which is active the following spring and early to midsummer.
It seeks an animal host, obtains a blood meal, and molts to an adult stage to complete the 2year life cycle. The animal hosts can include humans, dogs, deer, cows, horses, raccoons, cats, skunks, black bears, and Virginia opossums.Each developmental stage of the tick requires feeding once and may take several days. B. burgdorferi is transmitted to the host during the blood meal. The longer the time of attachment of the infected tick to the host, the greater probability of transmission.
Etiology
The detection of B. burgdorferi in human tissue or body fluids is the most reliable technique to prove the cause of an infectious disease. In Lyme borreliosis, cultivation of the organism has been difficult, probably because of slow growth of B. burgdorferi and its low density in body tissues.
An alternative method to establish proof of infection by B. burgdorferi relies on the detection of its antibody. In early Lyme borreliosis, the immunoglobulin (Ig) M form of the antibody response appears first, peaks 3 to 6 weeks after exposure, and then declines. The IgG then becomes detectable and is present in late-stage disease states. However, antibodies are frequently not detectable in the early stages of the disease by current techniques. In the late stages of the disease, they are almost always positive. Other spirochetal diseases, such as yaws, pinta, or syphilis, may give false-positive results, but can be excluded by clinical evaluation.
Clinical Manifestations
Lyme borreliosis is a multisystem disease. Its primary target organs include the skin initially and later, potentially, the neurological, cardiac, and articular systems. Lyme borreliosis is categorized in three phases. Arbitrarily:
Stage I involves the dermatologic system and is diagnosed by the classic rash, erythema chronicum migrans (ECM).
Stage II involves the neurological or cardiac system months to years after exposure to the organism.
Stage III, involving the joints, also can occur months to years after the initial exposure.
These three stages may overlap and occasionally can present simultaneously. Moreover, any of these stages may occur in the absence of the others.
Erythema chronicum migrans (ECM) is pathognomonic for Stage I Lyme borreliosis. The average incubation period is 1 to 3 weeks (range: 3 days to 16 weeks). This rash is a diagnostic marker of the disease and begins as a small flat (macule) or swollen (papule) spot at the site of the tick bite and then expands to a very large (10 to 20 centimeters) oval or round lesion with a red to pink outer border and a very clear central area. Viable B. burgdorferi occasionally can be cultured in the advancing margins. Blood-borne spread of the spirochete may produce multiple, secondary lesions days to weeks later. The rash persists for a few days to weeks, and is usually unaccompanied by systemic symptoms, although occasionally fever, chills, and fatigue may occur. Because the rash can be asymptomatic or on areas of the body that may not be observed, many people may go undiagnosed. More than 20 percent of adults who have Lyme borreliosis will not remember having the rash, and the percentage is much higher in children.
Stage II of Lyme borreliosis may involve the neurological system. Of patients, 10 to 15 percent may have this involvement and present with a meningitis-type picture or have cranial nerve palsies. The most commonly involved cranial nerve is the seventh (facial) nerve, which results in an inability to control properly the facial musculature. In individuals with meningeal irritation, episodic headaches, neck pain, and stiffness may occur. Cerebrospinal fluid analysis frequently shows a predominance of mononuclear white blood cells. Occasional patients with stroke syndromes including hemiparesis as well as cases mimicking multiple sclerosis or encephalitis have been reported. Individuals may have associated confusion, agitation and disorientation, and memory loss. The symptoms and signs may wax and wane over weeks and months.
Heart involvement is rare as part of Stage II manifestations of Lyme borreliosis. Cardiac manifestations are commonly detected only as first-degree heart block on electrocardiographic tracings, although some patients can have a more serious second- or third-degree heart block and present with episodic fainting spells. Both the neurological and cardiac manifestations may occur within weeks to months or even longer after infection with the B. burgdorferi organism, and they can occur without any antecedent rash.
The most common late manifestation of Lyme borreliosis is arthritis. It usually occurs several months after the tick bite, but the range is 1 week to over 10 years after exposure. It is usually an Oligoarticular form of arthritis (fewer than four joints) involving the Iargejoints such as the knee or ankle. The attacks may last from a few days to several months. Some individuals will have recurrence with variable periods of remission between attacks. The intensity of articular involvement is variable, because some patients complain only of aches {arthralgias), whereas others demonstrate joint swelling {arthritis). In children the symptoms and signs of Stage III Lyme borreliosis can mimic juvenile rheumatoid arthritis. Antibody testing to B. burgdorferi can provide valuable clues to diagnosis of Lyme borreliosis in those individuals who present with Stage II or III without antecedent history of ECM or tick bite exposure.
B. burgdorferi infection can be spread transpla- centally in humans. Infections during pregnancy can result in spontaneous abortion, premature delivery, low birth weight, and congenital malformation. These complications are similar to those caused by the spirochete Treponema pallidum, the causative agent of syphilis.
Lyme borreliosis is treated with antibiotics. The best results are achieved with prompt administration of oral antibiotics at the time of initial infection (Stage I). The duration of the rash and associated symptoms is abbreviated by a 3-week course of oral tetracycline, penicillin, or erythromycin therapy.
In Stage III Lyme borreliosis, 3 weeks of parenteral penicillin or ceftriaxone are the drugs of choice. The earlier antibiotics are instituted, the more likely a cure may be achieved. A delay in starting antibiotics may result in lifelong, intermittent or even chronic, residual symptoms from the disease.History
In 1909, in Stockholm, Swedish dermatologist Arvid Afzelius (1910) described a rash (which he labeled erythema migrans) on a female patient following a bite from an Ixodes tick. In 1913 an Austrian physician described a similar lesion on a patient and labeled it erythema chronicum migrans (ECM). Recognition that ECM was associated with systemic symptoms occurred in France in 1922. Investigators described patients with tick bites who developed subsequent erythema migrans and lymphocytic meningoradiculitis (nervous system involvement). In 1934 a German dentist described six patients with erythema migrans and associated joint symptoms. And in 1951 beneficial effects of penicillin in the treatment of a patient with ECM and meningitis suggested a bacterial etiology.
In the United States the first documented case of ECM occurred in a grouse hunter in Wisconsin in 1969. In 1975, two homemakers reported to the Connecticut State Health Department that several children living close together in Lyme, Connecticut, had developed arthritis. A following investigation by Allen C. Steere revealed an associated rash (ECM) in 25 percent of patients. He named the syndrome Lyme disease in honor of the town in which it was first observed.
In 1977 it was observed that an I. dammini tick bite preceded the ECM rash on a human subject. Willy Burgdorfer and his colleagues (1982) isolated a spirochete from the midgut of the I. dammini tick; this spirochete was subsequently named Borrelia burgdorferi. The following year, Steere isolated the spirochete from blood, skin, and spinal fluid from patients with the Lyme disease syndrome and concluded that it was the causative agent.
Also, in 1983 in West Germany, H. W. Pfister and his colleagues (1984) concluded that B. burgdorferi isolated in spinal fluid from a patient with lymphocytic meningoradiculitis (Bannwarth’s syndrome) was causative, and they implied that the original description of 13 patients provided by A. Bannwarth (1941) may have been due to the same organism. Dermatologist Klaus Weber and his colleagues (1984), working in West Germany, noted an elevation of IgG antibodies in the blood of patients with the skin lesion acrodermatitis chronica atrophicans (ACA). ACA is an uncommon late dermatologic manifestation of Lyme borreliosis. The first description of ACA was given by the German physician Alfred Buchwald (1883), and may have been the first reported case of Lyme borreliosis.Robert D. Leff
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