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Updated: Dec 30 2016

Rickettsial Diseases

Snapshot
  • A five-year-old boy presents to a hospital in Spain with a five-day-history of fever, abdominal pain, myalgia, and skin rash. There is a history of similar illness in his older brother. Blood and urine cultures, tests for Plasmodia, Widal test,  and IgM ELISA for leptospirosis all proved negative. Further questioning revealed a history of close contacts with dogs and a recent tick bite on one hand. Weil-Felix test was positive and samples were sent for PCR and immunohistochemistry.

Introduction

  • Classification
    • bacteria
      • Rickettsia
        • R. rickettsii
        • R. typhi
        • R. prowazekii
        • Coxiella burnetti
  • Highly pleomorphic and diverse collection of  bacteria 
    • difficult to describe as a single homogenous group 
    • mammals and arthropods are usually natural hosts
    • vary widely in severity 
      • self-limited mild illnesses to fulminant and life-threatening infections
    • obligate intracellular bacteria
    • Gram negative (although they don't Gram stain very well)
    • non-spore forming bacteria
  • Can be subdivided into 3 bio-groups 
    • spotted fever
      • RMSF caused by Rickettsia rickettsii
      • Rickettsialpox caused by Rickettsia akari
      • Boutonneuse fever
    • typhus
      • Louse-borne (epidemic) typhus caused by Rickettsia prowazekii
      • Murine (endemic or flea-borne) typhus caused by Rickettsia typhi
      • Brill-Zinsser disease
    • scrub typhus
      • have a single taxonomic name: Oriential tsutsugamushi
      • 3 major serotypes are Karp, Gilliam, and Kato.
  • Epidemiology
    • intimately related to each specific vector
    • transmission
      • ticks
      • fleas
      • lice
Presentation
  • History
    • tick bite or tick exposure
    • recent travel to endemic areas
    • similar illnesses in family, friends, or pets (especially dogs)
  • Signs & Symptoms
    • early
      • notoriously non-specific
      • mimic benign viral illnesses
    • RMSF 
      • incubation of 2-14 days following tick bite
      • fever, headache, myalgias, confusion
      • rash 
      • abdominal pain and diarrhea
    • Rickettsial pox
      • incubation of 9-14 days following mite bite
      • red papule at site of mite bite 
      • subsequently develops into eschar
      • fluctuating fevers, headache, chills, rigors, diaphoresis
      • rhinorrhea, cough, sore throat
      • nausea, vomiting, abdominal pain
    • Boutonneuse fever
      • incubation usually around 6 days
      • eschar and necrosis at tick bite site 
        • known as tache noire ("black spot"), is pathognomonic 
      • acute onset high fever, headache, myalgias
      • differs from other similar rickettsioses because of
        • painful lymphadenopathy
        • multiple eschars
        • nuchal myalgia
        • occasional sparse vesicular rash
    • Louse-borne (epidemic) typhus
      • abrupt onset 1-2 weeks after louse bite
      • fever
      • headache
      • rash
        • days 4-7 of infection
        • spreads from trunk to extremities
        • spares face, palms, and soles
    • Brill-Zinsser disease
      • milder form of louse-borne typhus
    • Murine (endemic or flea-borne) typhus 
      • milder and shorter form of louse-borne typhus
    • Scrub typhus
      • incubation period approximately 1-2 weeks
      • necrotic eschar at mite bite site
      • enlargement of regional lymph nodes
Evaluation
  • Labs
    • serology
      • possible thrombocytopenia, hypoalbuminemia, hypotension, and coag defects 
      • assays against antibodies to rickettsial antigens
      • can take up to 10-12 days to become positive
      • immunofluorescence assay - considered the reference serologic method
        • cannot determine causitive agent at the species level
    • PCR testing and immunohistochemical staining 
      • help detect rickettsiae in blood or tissue for early diagnosis
  • Diagnosis
    • via clinical suspicion and serologic confirmation
Treatment
  • Lifestyle
    • supportive therapy
  • Pharmacologic
    • doxycycline
      • will treat all rickettsial infections
      • highly effective and best outcomes if started within the first week of illness
      • side effects - rare association with staining of teeth in children younger than 8 years
        •  because of low dose and short duration
    • chloramphenicol
      • alternative to doxycycline
      • rarely used in United States because of adverse effects
      • side effects - potential bone marrow toxicity
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