PEDBASE.org - The Pediatric Database - Detailed information of LYME DISEASE

LYME DISEASE

DEFINITION:

A multiorgan rheumatologic disease characterized by inflammation of the connective tissue within the skin, central nervous system, heart, and joints.

EPIDEMIOLOGY:

incidence: ?
age of onset:
- any age
risk factors:
- genetic - symptoms worse in those with HLA-DR2
- infection - Borrelia burgdorferi
- environment - endemic areas, June/July

PATHOGENESIS:

1. Background

Borrelia burgdorferi is a spirochete which is transmitted in endemic regions to man from ticks of the genus Ixodes - first identified in Lyme, Conn. in 1975 by rheumatologist Dr. Allen Steele of Yale
Dr. Willy Burgdorfer was the first to isolate the spirochete
cattle, dogs, cats, and mice can all act as hosts

CLINICAL FEATURES:

I. Constitutional symptoms

headache
lymphadenoapthy
hepatomegaly/hepatosplenomegaly
migratory musculoskeletal pain
chills
testicular swelling
meningismus
dizziness

1. Stage 1 (Localized Infection)

1. Skin Manifestations (75%)

1. Erythema Chronicum Migrans (ECM)

initially an erythematous macule or papule at site of tick bite
3 to 32 days later expands into a bull's eye lesion
- red annular lesion with central clearing or
- indurated red centre that may become vesicular or necrotic
often reaches a diameter of 16 cm (3-68 cm)
located anywhere but mostly on the thigh, groin, or axillae

2. Secondary Lesions

malar rash, conjunctivitis, and small evanescent red blotches and circles
develop several days after ECM in up to 50% of patients
smaller in size and lack the indurated centres
not associated with the tick bite
skin manifestations resolve within 3-4 weeks
regardless of treatment, skin manifestations may recur

2. Stage 2 (Disseminated Infection)

1. Rheumatological Manifestations (80%)

joint manifestations occur within a week to 2 years after initial illness

1. Migratory Arthralgias

appear early in course of disease

2. Arthritis

begins months after onset of illness
typically involves large joints (knees) but also small joints
lasts for weeks to months and usually recurs for several years; in 10% becomes chronic

2. Neurological (CNS) Manifestations (10%)

occur within 4 weeks of initial illness and resolve over 3 months but can recur and may become chronic

1. Major (Triad)

1. Cranial Neuropathy (Bell's palsy)

2. Meningitis

3. Peripheral Radiculoneuropathy

2. Minor

cerebellar ataxia
chorea
demyelinating encephalopathy
Guillian-Barre
pseudotumor cerebri
seizures

3. Cardiovascular Manifestations (8%)

occur within 5 weeks of initial illness and last from 3 days to 6 weeks and rarely recur

1. Major

AV block (1st, 2nd [Wenkebach], & 3rd degrees)
cardiomegaly
left ventricular dysfunction
myopericarditis

3. Stage 3 (Persistent Infection)

occurs rarely in children
may last for years

1. Cutaneous Manifestations

acrodermatitis chronica atrophicans

2. Rheumatological Manifestations

progressive arthritis leading to permanent disability

3. Neurological Manifestations

depression, intellectual impairment, demyelinating disease

INVESTIGATIONS:

1. Serum

elevated ZSR (50%), IgM (33%), AST (19%)

2. Urine

proteinuria, microscopic hematuria

3. CSF

mononuclear pleocytosis
normal glucose
modest elevation in protein

4. Synovial Fluid

WBC - 500-100,000
- predominance of PMN leukocytes
elevated protein level
slight decrease in C3, C4, and total hemolytic compliment

5. Serologic Tests

developmental stage

MANAGEMENT:

1. Stage 1

po amoxicillin or erythromycin (young) or tetracycline (older) for 10-30 days
- symptoms resolve rapidly with good rate of success
- can prevent the later complications

2. Stage 2

IV ceftriaxone for 14 days
- can cross the blood brain barrier
- IV tetracycline or chloramphenicol for those with a penicillin or cephalosporin allergy

3. Stage 3

IV penicillin or ceftriaxone or
po amoxicillin or tetracycline

Pediatric Database - LYME DISEASE

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