CSTE Position Statements 2000 ID-#3

COMMITTEE: Infectious Disease

TITLE: Changes in the case definition for human ehrlichiosis, and addition of a new ehrlichiosis category as a condition placed under surveillance according to the National Public Health Surveillance System (NPHSS)

ISSUE: Human ehrlichiosis is an emerging tick-borne infectious disease in the United States. Human ehrlichiosis caused by Ehrlichia chaffeensis (human monocytic ehrlichiosis [HME]) and Ehrlichia phagocytophila  (human granulocytic ehrlichiosis [HGE]) were placed under national surveillance according to NPHSS via CSTE position statement #6 in 1998; previously, CSTE position statement #17 in 1996 had described a uniform case definition for reporting purposes. Since that time, advances in laboratory diagnostic criteria have expanded to include a range of available tests. In addition, a third Ehrlichia species (Ehrlichia ewingii) has been shown to cause disease in humans, and it is possible that additional pathogenic Ehrlichia species will be identified in the future. Finally, occasional problems with the classification of some ehrlichiosis cases have occurred because of cross-reactivity in serologic assays used to diagnose these diseases. There is a need to revise the current case definition to clarify reporting criteria, and to place cases caused by unspecified or novel Ehrlichia species under national surveillance.

POSITION TO BE ADOPTED:

CSTE and NASPHV recommend:

  1. The ehrlichiosis case definition should be revised to include currently available laboratory techniques, as well as instructions for classifying cases caused by unspecified and novel Ehrlichia species.
  2. Confirmed and probable cases of ehrlichiosis meeting the revised case definition should be placed under national surveillance through NPHSS. Categories for notification should include: HME, HGE, and ehrlichiosis (other or unspecified agent).

GOALS FOR SURVEILLANCE:

HME and HGE have been placed under surveillance according to NPHSS and over 600 cases were reported to state health departments in 1999. However, the current case definition does not allow accurate reporting of cases when an etiologic distinction between E. chaffeensis and E. phagocytophila is not made, or when disease is caused by E. ewingii or a novel Ehrlichia species. At least 48 cases of unspecified ehrlichiosis reported to state health departments in 1999 could not be correctly submitted to the National Electronic Telecommunications System for Surveillance (NETSS) under the current case definition.  The correct classification and reporting of these cases is necessary to further define and monitor the geographic boundaries for these diseases.

METHODS FOR SURVEILLANCE:

Clinician and laboratory reporting. Core surveillance data should be forwarded to the Centers for Disease Control and Prevention (CDC) via the National Electronic Telecommunications System for Surveillance (NETSS) for the purposes of national surveillance.

CASE DEFINITION:

Clinical description

A tick-borne illness characterized by acute onset of fever, headache, myalgia, and/or malaise. Nausea, vomiting, or rash may be present in some cases. Clinical laboratory findings may include thrombocytopenia, leukopenia, and/or elevated liver enzymes. Intracytoplasmic bacterial aggregates (morulae) may be visible in the leukocytes of some patients.

Three categories of confirmed or probable ehrlichiosis should be reported: 1) human ehrlichiosis caused by E. chaffeensis (HME), 2) human ehrlichiosis caused by E. phagocytophila (HGE), and 3) human ehrlichiosis (other or unspecified agent), which includes cases that cannot be easily classified by available laboratory techniques, and cases caused by novel Ehrlichia species such as E. ewingii.

Laboratory criteria for diagnosis

HME:

  • Demonstration of a four-fold change in antibody titer to E. chaffeensis antigen by indirect immunofluorescence assay (IFA) in paired serum samples, or
  • Positive polymerase chain reaction (PCR) assay and confirmation of E. chaffeensis DNA, or
  • Identification of morulae in leukocytes, and a positive IFA titer to E. chaffeensis antigen (based on cutoff titers established by the laboratory performing the assay), or
  • Immunostaining of E. chaffeensis antigen in a biopsy or autopsy sample, or
  • Culture of E. chaffeensis from a clinical specimen.

HGE:

  • Demonstration of a four-fold change in antibody titer to E. phagocytophila antigen by IFA in paired serum samples, or
  • Positive PCR assay and confirmation of E. phagocytophila DNA, or
  • Identification of morulae in leukocytes, and a positive IFA titer to E. phagocytophila antigen (based on cutoff titers established by the laboratory performing the assay), or
  • Immunostaining of E. phagocytophila antigen in a biopsy or autopsy sample, or
  • Culture of E. phagocytophila from a clinical specimen.

Ehrlichiosis (other or unspecified agent):

  • Demonstration of a four-fold change in antibody titer to more than one Ehrlichia species by IFA in paired serum samples, in which a dominant reactivity cannot be established, or
  • Identification of an Ehrlichia species other than E. chaffeensis or E. phagocytophila by PCR, immunostaining, or culture. 

Case classification

Probable: a clinically compatible illness with either a single positive IFA titer (based on cutoff titers established by the laboratory performing the test) or the visualization of morulae in leukocytes.

Confirmed: a clinically compatible illness that is laboratory-confirmed.

DATA TO BE COLLECTED:

NETSS Core Data and supplemental information

PERIOD OF SURVEILLANCE:

Permanent, with review of reporting needs every five years.

COORDINATION WITH OTHER ORGANIZATIONS:

Agency for Response:

James M. Hughes, MD

Centers for Disease Control and Prevention

National Center for Infectious Disease

1600 Clifton Road, NE

M/S C12

Atlanta, GA  30333

Agencies for Information:

Association of Public Health Laboratories (APHL)

CONTACT PERSON:

Matthew Cartter, MD, MPH

Epidemiology Program Infectious Diseases Division

Connecticut Department of Public Health

410 Capitol Ave. MS 11-EPI

P.O. Box 340308

Hartford, CT  06106

Tel:(860) 509-7994

Fax: (860) 509-7910

Email: Matt.cartter@po.state.ct.us