LYME DISEASE

CHARLES J. ODGERS, IV, MD, Resident, Orthopaedic Surgery

WILLIAM MACKENZIE, MD, Attending Orthopaedic Surgeon

June 12, 1996

CLINICAL CASE PRESENTATION

ORTHOPAEDIC DEPARTMENT

THE ALFRED I. DUPONT INSTITUTE

WILMINGTON, DELAWARE

CASE HISTORY:

An 8-year-old boy presented with a seven month history of intermittent right elbow pain and swelling. He stated that his discomfort first began when his right arm was twisted by a girl who "got the best of him". This injury was associated with pain, swelling, and loss of ROM of his right elbow. It was initially treated with ice packs with much improvement over the next several days. He had three subsequent episodes of pain and swelling in his right elbow not precipitated by trauma with near-complete resolution of pain between each occurrence. The last episode, occurring three days ago, did not respond to ice packs and significantly limited his elbow ROM. The patient denied history of fevers, chills, or rashes.

PHYSICAL EXAM:

On physical exam, he had a marked effusion of his right elbow, and he was holding his elbow flexed at 90 degrees. His ROM was 50-95 degrees, and was unable to pronate or supinate his forearm. He had no local adenopathy, and the rest of his exam was unremarkable. He did not have any other joint swelling.

DIAGNOSTIC STUDIES

Plain radiographs of his right elbow revealed a posterior fat pad sign consistent with a joint effusion. There was no bony abnormalities on x-ray. An aspiration of his right elbow was performed, the joint fluid being straw colored. It was sent for cell count and lyme titers. Lyme ELISA (on the joint fluid) result was 1:640, and the confirmatory Western blot was positive. The patient was referred to a rheumatologist who placed the patient on doxycycline 100mg BID for 30 days and obtained an EKG to rule out cardiac abnormalities.

LYME DISEASE- ORTHOPAEDIC MANIFESTATIONS

INTRODUCTION

• Recognized in 1975- Dr Alan Steere- investigating a clustering of inflammatory arthropathies in children in Connecticut
• Multisystem infection- spirochete Borrelia burgdorferi- transmitted by Ixodes deer tick
• Disease risks geographically limited- northeast, northcentral, and pacific coast U.S.
• 85% of cases in U.S. occurred in 8 states- mostly northeast
• The "Great Imitator"- mimics many other diseases

CLINICAL PRESENTATION

EARLY LOCALIZED INFECTION: STAGE 1

• Characterized by erythema chronicum migrans(ECM) within 3-30 days of infection
• ECM lesion- red macular rash- exhibited in <50% of children
• B. burgdoferi can be cultured from ECM lesion
• Low grade fever, minor constitutional symptoms, headache, local adenopathy

EARLY DISSEMINATED INFECTION: STAGE 2

• Occurs days to weeks later- spirochete disseminates to multiple organ systems
• Flu-like symptoms- malaise, fever, chills, headaches
• Cutaneous manifestations
• secondary ECM- usually multiple, smaller, and scattered lesions
• seen in ~50% of untreated patients
• Neurologic manifestations
• occurs in 15-20% of patients
• lyme meningitis- waxing and waning in severity- can last for several months
• cranial neuropathy- facial nerve palsy most common
• North American study- headaches, behavioral changes, and facial nerve palsy were most common neurologic manifestations in children
• Cardiac manifestations
• 1. carditis- in 4-8% of pts- usually mild and asymptomatic
• rapidly fluctuating AV block- usually self-remitting
• Musculoskeletal manifestations
• typically migratory lasting hours to days in any single location
• involves both articular and periarticular structures- pain without swelling
• both large and small joints involved- usually one joint at a time

LATE (PERSISTENT) INFECTION: STAGE 3- LYME ARTHRITIS

• Very common presentation for lyme disease in children- 33%(Rose et al)
• Characteristically episodic with mono- or oligoartricular involvement
• Large joints typically involved- 2/3 of pts have knee symptoms
• Joint effusions usually large, out of proportion to pain and brief in duration
• Rose et al- evaluated 44 pts- five different patterns of arthritis
• classical episodic involvement of 1-4 joints for <1 wk separated by asymptomatic intervals of atleast 2 wks(43%)
• acute pauciarticular("pseudoseptic)- continual involvement of 1-4 joints for <4 wks(36%)
• chronic pauciarticular(pauci-JRA-like)- persistent arthritis in 1-4 joints for >4 wks(13%)
• migratory- involvement of 3 or more joints in a sequential pattern in which one "hot joint" predominates at any given time(4%)
• polyarticular- involvement of 5 or more joints(4%)
• Children are less likely to develop chronic lyme arthritis with associated joint destruction
• Differential diagnosis
• septic arthritis
• juvenile rheumatoid arthritis
• reactive arthritis(toxic synovitis, Reiter's syndrome)

CLINICAL DIAGNOSIS- SEROLOGIC TESTING

• ELISA- most common test used for detection
• Detects antibodies(IgG & IgM) of spirochete
• 96% sensitive in late stages-arthritis
• Not sensitive in early stage
• Marked risk of false positive results- need to confirm by Western blot
• Difficult to distinguish active from inactive infection( ? decreased titers)
• Polymerase chain reaction(PCR)
• Can directly detect cellular components of spirochete in joint fluid
• Studies have found that assays turn negative in patients with a successful treatment outcome
• potential pitfalls- contamination of samples, lack of detection due to genetic variation

TREATMENT:

• Antimicrobial therapy- treatment of choice
• Intraarticular steroids and NSAIDs- lessen articular inflammatory response
• Oral steroids- for refractory cardiac involvement
• Synovectomy-only for refractory involvement with persistent effusions and limited function- rarely used in children

REFERENCES:

1. Evans J: Lyme disease. Curr Op in Rheum 1995;7(4):322-8.
2. Jouben LM, Steele RJ, Bono JV: Orthopaedic manifestsations of lyme disease. Orthop Rev 1994; 23(5):395-400.
3. Kalish R: Lyme disease. Rheum Dis Clin North Am 1993; 19(2):399-426.
4. Lawrence SJ: Lyme disease: An orthopaedic perspective. Orthop 1993; 15(11): 1331-5.
5. Rose CD, Fawcett PT, Epps SC, Klein JD, Gibney K, Doughty RA: Pediatric lyme arthritis: clinical spectrum and outcome. J Pediatr Orthop 1994;14(2):238-241.
6. Steere AC: Musculoskeletal manifestations of lyme disease. Am J Med 1995; 98(4A):44s-48s.