The National Organization for Rare Disorders (NORD), P.
O. Box 8923, NewFairfield, CT 06812, (203) 746-6518Lyme DiseaseLyme ArthritisGeneral Discussion——————————–** REMINDER ** The information contained in the Rare Disease Database is provided foreducational purposes only. It should not be used for diagnostic ortreatment purposes. If you wish to obtain more information about thisdisorder, please contact your personal physician and/or the agencies listedin the “Resources” section of this report. Lyme disease is a tick-transmitted inflammatory disorder characterizedby an early focal skin lesion, and subsequently a growing red area on theskin (erythema chronicum migrans or ECM). The disorder may be followedweeks later by neurological, heart or joint abnormalities.
Symptomatology——————————– The first symptom of Lyme disease is a skin lesion. Known as erythemachronicum migrans, or ECM, this usually begins as a red discoloration(macule) or as an elevated round spot (papule). The skin lesion usuallyappears on an extremity or on the trunk, especially the thigh, buttock orthe under arm. This spot expands, often with central clearing, to adiameter as large as 50 cm (c.
12 in. ). Approximately 25% of patients withLyme disease report having been bitten at that site by a tiny tick 3 to 32days before onset of ECM. The lesion may be warm to touch.
Soon afteronset nearly half the patients develop multiple smaller lesions withouthardened centers. ECM generally lasts for a few weeks. Other types oflesions may subsequently appear during resolution. Former skin lesions mayreappear faintly, sometimes before recurrent attacks of arthritis. Lesionsof the mucous membranes do not occur in Lyme disease. The most common symptoms accompanying ECM, or preceding it by a fewdays, may include malaise, fatigue, chills, fever, headache and stiffneck.
Less commonly, backache, muscle aches (myalgias), nausea, vomiting,sore throat, swollen lymph glands, and an enlarged spleen may also bepresent. Most symptoms are characteristically intermittent and changing, butmalaise and fatigue may linger for weeks. Arthritis is present in about half of the patients with ECM, occurringwithin weeks to months following onset and lasting as long as 2 years. Early in the illness, migratory inflammation of many joints(polyarthritis) without joint swelling may occur. Later, longer attacksof swelling and pain in several large joints, especially the knees,typically recur for several years.
The knees commonly are much moreswollen than painful; they are often hot, but rarely red. Baker’s cysts(a cyst in the knee) may form and rupture. Those symptoms accompanying ECM, especially malaise, fatigue and low-grade fever, may also precede or accompany recurrent attacks of arthritis. About 10% of patients develop chronic knee involvement (i. e. unremittentfor 6 months or longer).
Neurological abnormalities may develop in about 15% of patients withLyme disease within weeks to months following onset of ECM, often beforearthritis occurs. These abnormalities commonly last for months, andusually resolve completely. They include:1. lymphocytic meningitis or meningoencephalitis2. jerky involuntary movements (chorea)3. failure of muscle coordination due to dysfunction of the cerebellum (cerebellar ataxia)4.
cranial neuritis including Bell’s palsy (a form of facial paralysis)5. motor and sensory radiculo-neuritis (symmetric weakness, pain, strange sensations in the extremities, usually occurring first in the legs)6. injury to single nerves causing diminished nerve response (mononeuritis multiplex)7. inflammation of the spinal cord (myelitis). Abnormalities in the heart muscle (myocardium) occur in approximately8% of patients with Lyme disease within weeks of ECM. They may includefluctuating degrees of atrioventricular block and, less commonly,inflammation of the heart sack and heart muscle (myopericarditis) withreduced blood volume ejected from the left ventricle and an enlarged heart(cardiomegaly).
When Lyme Disease is contracted during pregnancy, the fetus may or maynot be adversely affected, or may contract congenital Lyme Disease. In astudy of nineteen pregnant women with Lyme Disease, fourteen had normalpregnancies and normal babies. If Lyme Disease is contracted during pregnancy, possible fetalabnormalities and premature birth can occur. Etiology——————————– Lyme disease is caused by a spirochete bacterium (Borrelia Burgdorferi)transmitted by a small tick called Ixodes dammini. The spirochete isprobably injected into the victim’s skin or bloodstream at the time of theinsect bite. After an incubation period of 3 to 32 days, the organismmigrates outward in the skin, is spread through the lymphatic system or isdisseminated by the blood to different body organs or other skin sites.
Lyme Disease was first described in 1909 in European medical journals. The first outbreak in the United States occurred in the early 1970’s in Oldlyme, Connecticut. An unusually high incidence of juvenile arthritis in thearea led scientists to investigate and identify the disorder. In 1981, Dr.
Willy Burgdorfer identified the bacterial spirochete organism (BorreliaBurgdorferi) which causes this disorder. Affected Population——————————– Lyme Disease occurs in wooded areas with populations of mice and deerwhich carry ticks, and can be contracted during any season of the year. Related Disorders——————————– Rheumatoid Arthritis is a disorder similar in appearance to Lymedisease. However, the pain in rheumatoid arthritis is usually morepronounced. Morning stiffness and symmetric joint swelling more commonlyoccur in rheumatoid arthritis, and knotty lumps under the skin may bepresent over bony prominences. Bony decalcification which can beprominent in Rheumatoid Arthritis is detected on X-rays.
Brachial Neuritis, also known as Parsonnage-Turner Syndrome, is acommon inflammation of a group of nerves that supply the arm, forearm, andhand (brachial plexus). It is characterized by severe neck pain in thearea above the collarbone (supraclavicular) that may radiate down the armand into the hand. There also may be weakness and numbness (hyperesthesia)of the fingers and hands. Although many cases have no apparent cause, thissyndrome may occur following an immunization (tetanus or diptheria),surgery, or infection with Lyme Disease.
Therapies: Standard——————————– For adults with Lyme disease the antibiotic tetracycline is the drug ofchoice. Penicillin V and erythromycin have also been used. In childrenpenicillin V is recommended rather than tetracycline. Penicillin V is nowrecommended for neurological abnormalities.
It is not yet clear whetherantibiotic treatment is helpful later in the illness when arthritis is themost predominant symptom. Treatment should be started as soon as the rashappears, even before the Enzyme Linked Immunoabsorbent Assay (ELISA) testis completed. Results of this test may be inaccurate if patients have hadantibiotics soon after contracting Lyme Disease, or in those who haveweakened immune systems. If lyme Disease is contracted during pregnancy, careful monitoring byphysicians is highly recommended to avoid possible fetal abnormalitiesand/or complications.
For tense knee joints due to increased fluid flowing in the jointspaces (effusions), the use of crutches is often helpful. Aspiration offluid and injection of a corticosteroid may be beneficial. If the patientwith Lyme disease has marked functional limitation, excision of themembrane lining the joint (synovectomy) may be performed for chronic (6months or more despite therapy) knee effusions, but spontaneous remissioncan occur after more than a year of continuous knee involvement. When Lyme Disease is contracted during pregnancy, treatment withpenicillin should begin immediately to avoid the possibility of fetalabnormalities.
In 1989 a new Lyme Disease antibody test, manufactured by CambridgeBiosciences Corp. , was approved by the FDA. This test is being used bylocal laboratories throughout the nation, making tests more available tothe general population. However, it is 97% specific for antibodies to Lymedisease when compared to Western blot tests, but it cannot identify thelive bacteria in patients who have not yet developed the antibodies.
Therapies: Investigational——————————– Researchers are trying to develop a test that will identify the Lymedisease bacteria in patients who have not yet developed the antibodies. This would enable doctors to diagnose Lyme disease very early in the courseof the illness. This disease entry is based upon medical information available throughJuly 1989. Since NORD’s resources are limited, it is not possible to keepevery entry in the Rare Disease Database completely current and accurate.
Please check with the agencies listed in the Resources section for the mostcurrent information about this disorder. Resources——————————–For more information on Lyme Disease, please contact:National Organization for Rare DisordersP. O. Box 8923New Fairfield, CT 06812(203) 746-6518Lyme Borreliosis Foundation, Inc.
P. O. Box 462Tolland, CT 06084(203) 871-2900Lyme Disease ClinicMarshfield Clinic1000 North Oak Ave.Marshfield, WI 54449The National Arthritis and Musculoskeletal and Skin Diseases InformationClearinghouseBox AMSBethesda, MD 20892(301) 468-3235Lyme Disease ClinicYale New Haven Hospital333 Cedar StreetNew Haven, CT 06510
