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Relapsing Fever

Chapter 190 | Part 5: Infectious Diseases · Part 5 – Infectious Diseases: Bacterial · Chapter 190


Key Clinical Points

  1. Relapsing fever is a recurrent febrile illness caused by Borrelia spirochetes.
  2. Three clinical forms exist: louse-borne (LBRF), soft tick-borne (STRF), and hard tick-borne (HTRF).
  3. Antigenic variation of surface proteins allows Borrelia to evade host immunity, causing the characteristic relapsing pattern.
  4. Diagnosis relies on blood microscopy (thin/thick smears), PCR/NAAT (preferred for HTRF), or GlpQ-based serology.
  5. First-line treatments include penicillin and tetracyclines; erythromycin is used for pregnant/nursing women and children.
  6. LBRF is limited to northeastern Africa; STRF and HTRF are found globally.
  7. Jarisch-Herxheimer reaction (JHR) occurs in ~50% of cases during treatment, requiring monitoring for fever, hypotension, and diaphoresis.
  8. Neurologic complications (e.g., Bell's palsy, meningitis) are more common in STRF than LBRF.
  9. Tick-borne forms may coexist with Lyme disease or other tick-borne infections.
  10. Borrelia spirochetes are resistant to rifampin, sulfonamides, and aminoglycosides.

1. DEFINITION & CLASSIFICATION

Definition: Relapsing fever is a recurrent febrile illness caused by Borriella spirochetes.Clinical Forms: ◦ Louse-borne relapsing fever (LBRF): Caused by Borrelia recurrentis, transmitted by human body lice (Pediculus humanus corporis). ◦ Soft tick relapsing fever (STRF): Transmitted by Ornithodoros ticks; caused by multiple species including B. turicatae and B. duttonii. ◦ Hard tick-borne relapsing fever (HTRF): Transmitted by Ixodes or Amblyomma ticks; includes B. miyamotoi and B. lonestari. • Pathophysiology: ◦ Antigenic variation of surface proteins prevents sustained immunity. ◦ CNS invasion is uncommon in LBRF (indicated by dashed line in Figure 190-3).

Clinical Forms & Table 190-1

LBRF: Limited to northeastern Africa; associated with poverty and overcrowding. ◦ Note: CNS involvement is less common in this form. • STRF: Found on most continents except Arctic regions; transmitted via Ornithodoros ticks. • HTRF: Widespread in North America, Europe, and Asia; B. miyamotoi often coexists with Lyme disease.

Table 190-1 (Relapsing Fever Borrelia Species): ◦ STRF: Includes B. crocidurae, B. turicatae, B. duttonii, B. hermsii, B. mazzotti, B. nietonii, and B. puertoricensis. ◦ HTRF: Includes B. miyamotoi and B. lonestari. ◦ LBRF: Includes B. recurrentis.


2. EPIDEMIOLOGY

LBRF: Limited to Ethiopia, Eritrea, and Somalia. • STRF: Global distribution (except Arctic); transmitted via Ornithodoror ticks in homes or caves. • HTRF: North America, Europe, Asia; B. miyamotoi coexists with Lyme disease. • Transmission Dynamics: ◦ Tick-borne forms involve transovarial transmission in ticks, sustaining infection risk even without a constant mammalian host.


3. ETIOLOGY & PATHOPHYSIOLOGY

Entry: Spirochetes enter via tick saliva during feeding. • Proliferation: STRF spirochetes replicate rapidly (doubling every 6 hours); HTRF grow more slowly. • Hematologic Effects: Binding to erythrocytes causes sequestration in spleen/liver → hepatosplenomegaly and anemia. • Neurotropism: Some species (e.g., B. turicatae) cross the blood-brain barrier, causing meningitis or cranial neuritis. • Proinflammatory Response: Lipoproteins activate Toll-like receptors → cytokine release (TNF-α, IL-6).


4. CLINICAL FEATURES

Fever Pattern: ◦ Sudden high fever (>39°C) with afebrile intervals of 4–14 days. ◦ LBRF: First episode (3–6 days) followed by 1 milder relapse. ◦ STRF: Multiple febrile episodes (1–3 days each), recurring at least twice. • Systemic Symptoms: ◦ Common: Headache, myalgia, arthralgia, vomiting. ◦ LBRF specific: Jaundice, epistaxis, subconjunctival hemorrhages. ◦ General: Splenomegaly and hepatomegaly. • Neurologic Manifestations: ◦ STRF: Bell's palsy (common), aseptic meningitis, cranial neuritis. ◦ LBRF: Altered mental state (likely due to systemic inflammation). • Cardiac Involvement: Gallop rhythm, prolonged QT interval in STRF/LBRF.

Laboratory Findings

Hematology: Mild normocytic anemia, thrombocytopenia (<50,000/μL). ◦ Inflammatory markers: Elevated CRP and procalcitonin. ◦ Organ function: Elevated aminotransferases. ◦ CSF: Mononuclear pleocytosis, elevated protein.


5. DIFFERENTIAL DIAGNOSIS

Tick-borne: Lyme disease (coexists with B. miyamotoi), Rocky Mountain spotted fever, ehrlichiosis, babesiosis. • Louse-borne: Typhus (Rickettsia prowazekii), trench fever (B. quintana). • Co-infections: Malaria, typhoid, leptospirosis.


6. DIAGNOSTIC APPROACH

  1. Microscopy: ◦ Thin blood smear: Detects spirochetes at ≥10^5/mL. ◦ Thick smear (acetic acid or citrated blood wet mount): Higher sensitivity for detection.
  2. Molecular Testing: ◦ PCR/NAAT: Preferred for detecting B. miyamotoi and B. lonestari in blood/CSF, especially between febrile episodes.
  3. Serology: ◦ GlpQ-based assays: Better specificity for STRF/LBRF than Lyme disease.

7. MANAGEMENT & TREATMENT

  1. Initial Assessment: Identify infection type (Tick vs Louse) and presence of CNS involvement.
  2. Treatment for Meningitis/Encephalitis (Both types): ◦ Ceftriaxone 2 g qd OR Na penicillin G, 5 million U q6h for 14 days.
  3. Treatment for Non-CNS cases:Tick-borne: ◦ Age ≥ 9, not pregnant → Doxycycline 100 mg bid (10 days). ◦ Alternatives: Tetracycline 500 mg qid OR Erythromycin 500 mg qid. ◦ Age < 9 → Erythromycin 12.5 mg/kg per day. • Louse-borne: ◦ Penicillin Allergy → Erythromycin 500 mg qid (≥ 9) or 12.5 mg/kg per day (< 9) for 7 days. ◦ No Allergy (Sequential Therapy): ◦ Initial: Penicillin G 20M IM OR Ceftriaxone 800,000 U (Adults); 125 mg or 200,000–400,000 U (Children). ◦ Follow-up (4–12 h later): Doxycycline/Tetracycline (≥ 9) OR Erythromycin (< 9 or pregnant). ◦ Duration: 7 days.
  4. Monitoring: ◦ Jarisch-Herxheimer Reaction (JHR): Monitor for fever, hypotension, diaphoresis; manage with fluids and antipyretics.

Treatment Algorithm (Figure 190-3)

Step 1: Identify Pathogen Type → Tick-borne vs. Louse-borne. • Step 2: Meningitis/Encephalitis? ◦ YES → Ceftriaxone 2 g qd OR Na penicillin G, 5 million U q6h for 14 days. ◦ NO → Proceed to specific type treatment. • Step 3: Path A (Tick-borne) - No Meningitis: ◦ Age ≥ 9, not pregnant → Doxycycline 100 mg bid (10 days). ◦ Alternative 1 → Tetracycline 500 mg qid. ◦ Alternative 2 → Erythromycin 500 mg qid. ◦ Age < 9 → Erythromycin 12.5 mg/kg per day. • Step 4: Path B (Louse-borne) - No Meningitis: ◦ Decision Node: Penicillin Allergy? ◦ YES → Erythromycin 500 mg qid (≥ 9) or 12.5 mg/kg per day (< 9) for 7 days. ◦ NO → Sequential Therapy (Initial dose + follow-up 4–12 h later). ◦ Initial: Penicillin G 20M IM OR Ceftriaxone 800,000 U (Adults); 125 mg or 200,000–400,000 U (Children). ◦ Follow-up (4–12 h later): Doxycycline/Tetracycline (≥ 9) OR Erythromycin (< 9 or pregnant). ◦ Duration: 7 days.


8. PROGNOSIS & COMPLICATIONS

Prognosis: Rapid cure with antibiotics; mortality <1% with appropriate treatment. ◦ Higher risk in elderly or severe disease. • Jarisch-Herxheimer Reaction (JHR): Occurs in ~50% of cases during treatment. • Complications: Neurologic sequelae (e.g., permanent vision loss), myocarditis, acute respiratory distress syndrome.


9. SPECIAL CONSIDERATIONS

Pregnancy/Pediatrics: Tetracyclines contraindicated; Erythromycin is the only safe option. • Prevention: Avoid tick exposure (insect repellent, protective clothing); louse control in endemic areas.


10. KEY PEARLS & CLINICAL TRAPS

Diagnostic Pearls: PCR/NAAT is more sensitive than blood smear for B. miyamotoi. ◦ GlpQ-based assays are preferred for distinguishing STRF/LBRF from Lyme. • Treatment Pearls: Erythromycin is the only safe option for pregnant/nursing patients. ◦ Doxycycline 100 mg bid (Tick-borne) vs. Sequential Therapy (Louse-borne) is a key distinction. • Clinical Traps: JHR may mimic worsening illness; do not mistake it for treatment failure. ◦ Confusing STRF with Lyme disease in endemic areas.


Reference Tables

TABLE 189-1 Treatment and Chemoprophylaxis of Leptospirosis in Adults a INDICATION Treatment Mild leptospirosis

Harrison's 22e, p.1444

INDICATION REGIMEN
Treatment
Mild leptospirosis Doxycyclineb (100 mg PO bid) or
Amoxicillin (500 mg PO tid) or
Ampicillin (500 mg PO tid)
Moderate/severe
leptospirosis
Penicillin (1.5 million units IV or IM q6h) or
Ceftriaxone (2 g/d IV) or
Cefotaxime (1 g IV q6h) or
Doxycyclineb (loading dose of 200 mg IV, then 100 mg IV
q12h)
Chemoprophylaxis
190 Relapsing Fever
Alan G. Barbour

TABLE 190-1 Relapsing Fever Borrelia Species, by RF Type, Geographic Region, and Vector SPECIES B. crocidurae B.…

Harrison's 22e, p.1445

SPECIES RELAPSING FEVER TYPE REGION(S) ARTHROPOD VECTOR(S)
B. crocidurae Soft tick RF (STRF) West Africa Ornithodoros sonrai
STRF East Africa, southern and central Africa
B. hermsii STRF Western North America O. hermsi
STRF North Africa, southern Europe
B. kalaharica STRF West Africa, southern Africa O. savignyi
Hard tick RF (HTRF) Southern and eastern United States
B. mazzotti STRF Mexico, Central America O. talaje
HTRF North America, Asia, Europe
B. nietonii STRF Western North America O. hermsii
STRF Central Asia, Middle East
B. puertoricensis STRF Central America O. puertoricensis
Louse-borne RF Africa, globala
B. turicatae STRF Southwestern United States, northern Mexico O. turicata
STRF Central America, South America