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09-ID-16 Cmmittee: Infectius Title: Public Health Reprting and Natinal Ntificatin fr Sptted Fever Rickettsisis (including Rcky Muntain sptted fever) I. Statement f the Prblem CSTE psitin statement 07-EC-02 recgnized the need t develp an fficial list f natinally ntifiable cnditins and a standardized reprting definitin fr each cnditin n the fficial list. The psitin statement als specified that each definitin had t cmply with American Health Infrmatin Cmmunity recmmended standards t supprt autmated case reprting frm electrnic health recrds r ther clinical care infrmatin systems. In July 2008, CSTE identified sixty-eight cnditins warranting inclusin n the fficial list, each f which nw requires a standardized reprting definitin. II. Backgrund and Justificatin Backgrund 1 Sptted fever rickettsises are respnsible fr several diseases with similar clinical presentatin. Rcky Muntain sptted fever, the mst cmmn and well-knwn sptted fever, is a tick-brne disease caused by Rickettsia rickettsii. While initially recgnized nly in the Western United States, the mst cases are nw fund in the Suth Atlantic States and the west suth-central regin. Rcky Muntain sptted fever is a serius illness with a 23% mrtality rate if untreated. Mrtality in the US is currently less than 5%, with fatal cases ften resulting frm delayed initiatin f antimicrbial therapy because the characteristic rash may be absent early in the curse f disease. There is als increasing awareness that ther, likely less pathgenic sptted fever rickettsises may be respnsible fr human illness frequently cnfused with Rcky Muntain sptted fever, including disease assciated with the tickbrne pathgen Rickettsia parkeri r ther Rickettsia species. nging surveillance is necessary t mnitr the gegraphic and tempral ccurrence f disease t s that clinicians can maintain a high awareness f the disease and the public kept adequately infrmed abut their risk f cntracting the disease. Justificatin Sptted Fever Rickettsisis (including Rcky Muntain sptted fever) meets the fllwing criteria fr a natinally and standard ntifiable cnditin, as specified in CSTE psitin statement 08-EC-02: A majrity f state and territrial jurisdictins r jurisdictins cmprising a majrity f the US ppulatin have laws r regulatins requiring standard reprting f Sptted Fever Rickettsisis (including Rcky Muntain Sptted Fever) t public health authrities 1 Much f the material in the backgrund is directly quted frm the CDC s Rcky Muntain sptted fever website. See the references fr further infrmatin n this surce. 1

CDC requests standard ntificatin f Sptted Fever Rickettsisis (including Rcky Muntain Sptted Fever) t federal authrities CDC has cnditin-specific plicies and practices cncerning the agency s respnse t, and use f, ntificatins. III. Statement f the desired actin(s) t be taken CSTE requests that CDC adpt this standardized reprting and classificatin definitin fr Sptted Fever Rickettsisis (including Rcky Muntain sptted fever) t facilitate mre timely, cmplete, and standardized lcal and natinal reprting f this cnditin. IV. Gals f Surveillance T prvide infrmatin n the tempral, gegraphic, and demgraphic ccurrence f Sptted Fever Rickettsisis (including Rcky Muntain Sptted Fever) t facilitate its preventin and cntrl. V. Methds fr Surveillance Surveillance fr Sptted Fever Rickettsisis (including Rcky Muntain sptted fever) shuld use the surces f data and the extent f cverage listed in Table V belw. Table V. Recmmended surces f data and extent f cverage fr ascertaining cases f Sptted Fever Rickettsises (including Rcky Muntain sptted fever.) Cverage Surce f data fr case ascertainment Ppulatin-wide Sentinel sites clinician reprting labratry reprting reprting by ther entities (e.g., hspitals, veterinarians, pharmacies) death certificates hspital discharge r utpatient recrds extracts frm electrnic medical recrds telephne survey schl-based survey ther 2

VI. Criteria fr Reprting Reprting refers t the prcess f healthcare prviders r institutins (e.g., clinicians, clinical labratries, hspitals) submitting basic infrmatin t gvernmental public health agencies abut cases f illness that meet certain reprting requirements r criteria. Cases f illness may als be ascertained by the secndary analysis f administrative health data r clinical data. The purpse f this sectin is t prvide thse criteria that shuld be used by humans and machines t determine whether a specific illness shuld be reprted. 2 A. Narrative descriptin f criteria t determine whether a case shuld be reprted t public health authrities Reprt any illness t public health authrities that meets any f the fllwing criteria: 1. Any patient with clinical illness and labratry evidence f Sptted Fever Rickettsisis (including Rcky Muntain sptted fever) including any f the fllwing: A furfld change in immunglbulin G (IgG)-specific antibdy titer reactive with Rickettsia rickettsii r ther sptted fever grup antigen by indirect immunflurescence assay (IFA) between paired serum specimens (ne taken in the first week f illness and a secnd 2-4 weeks later) Elevated IgG antibdy reactive with R. rickettsii r ther sptted fever grup antigen by IFA, enzyme-linked immunsrbent assay (ELISA), dt-elisa, r latex agglutinatin Elevated IgM antibdy reactive with R. rickettsii r ther sptted fever grup antigen by IFA, enzyme-linked immunsrbent assay (ELISA), dt-elisa, r latex agglutinatin Detectin f R. rickettsii r ther sptted fever grup DNA in a clinical specimen via amplificatin f a specific target by PCR assay Demnstratin f sptted fever grup antigen in a bipsy r autpsy specimen by IHC Islatin f R. rickettsii r ther sptted fever grup rickettsia frm a clinical specimen in cell culture. 2. A persn whse healthcare recrd cntains a diagnsis f Sptted Fever Rickettsisis (including Rcky Muntain sptted fever). 3. A persn whse death certificate lists Sptted Fever Rickettsisis (including Rcky Muntain sptted fever) as a cause f death r a significant cnditin cntributing t death. 2 Human-based criteria (described belw under A. Narrative ) can be applied by medical care prviders and labratry staff based n clinical judgment and clinical diagnsis. Machine-based criteria (described belw under B. Table ) can be applied using cmputerized algrithms that perate in electrnic health recrd systems, including cmputerized recrds f labratry test rders and labratry test results; ther clinical data systems (e.g., hspital discharge data systems serving multiple hspitals); r administrative data (e.g., healthcare prvider billing data, vital recrds, and EMS data). 3

ther recmmended reprting prcedures All cases f Sptted Fever Rickettsisis (including Rcky Muntain sptted fever) shuld be reprted. Reprting shuld be n-ging and rutine. Frequency f reprting shuld fllw the state health department s rutine schedule. B. Table f criteria t determine whether a case shuld be reprted t public health authrities Table VI-B. Prpsed Table f criteria t determine whether a case shuld be reprted t public health authrities. Nte: The fllwing criteria are prpsed fr evaluatin befre general implementatin. Fr purpses f currently implementing reprting the narrative descriptin in VI-A, shuld be used. Criterin Clinical Presentatin Healthcare recrd cntains a diagnsis f Sptted Fever Rickettsisis (including Rcky Muntain Sptted Fever) Death certificate lists Sptted Fever Rickettsisis (including Rcky Muntain Sptted Fever) as a cause f death r a significant cnditin cntributing t death Diagnstic Labratry Findings Furfld change in Rickettsia rickettsii-specific r ther sptted fever grup IgG antibdy titer by indirect immunflurescence assay (IFA) with paired serum specimens Elevated Rickettsia rickettsii-specific r ther sptted fever grup IgG antibdy titer by indirect immunflurescence assay (IFA), Elisa, Elisa-dt r latex agglutinatin n a single serum specimen Elevated Rickettsia rickettsii-specific r ther sptted fever grup IgM antibdy titer by indirect immunflurescence assay (IFA), Elisa, Elisa-dt r latex agglutinatin n a single serum specimen Detectin f R. rickettsii r ther sptted fever grup DNA in a clinical specimen by PCR assay Demnstratin f sptted fever grup antigen in a bipsy r autpsy specimen by IHC Reprting S S 4

Islatin f R. rickettsii r ther sptted fever grup rickettsia frm a clinical specimen in cell culture Ntes: S = This criterin alne is sufficient t reprt a case = At least ne f these criteria in each categry in the same clumn (e.g., clinical presentatin and labratry findings) in cnjunctin with all ther N criteria in the same clumn is required t reprt a case. C. Disease Specific Data Elements: (T be added) VII. Case Definitin fr Case Classificatin Narrative descriptin f criteria t determine whether a case shuld be classified as cnfirmed, prbable (presumptive), r suspected (pssible) Clinical presentatin Sptted fever rickettsises are a grup f tickbrne infectins caused by sme members f the genus Rickettsia. Rcky Muntain sptted fever (RMSF) is an illness caused by Rickettsia rickettsii, a bacterial pathgen transmitted t humans thrugh cntact with ticks. Dermacentr species f ticks are mst cmmnly assciated with infectin, including Dermacentr variabilis (the American dg tick), Dermacentr andersni (the Rcky Muntain wd tick), and mre recently Rhiphicephalus sanguineus (the brwn dg tick). Disease nset averages ne week fllwing a tick bite. Age-specific illness is highest fr children and lder adults. Illness is characterized by acute nset f fever, and may be accmpanied by headache, malaise, myalgia, nausea/vmiting, r neurlgic signs; a macular r maculpapular rash appears 4-7 days fllwing nset in many (~80%) patients, ften present n the palms and sles. RMSF may be fatal in as many as 20% f untreated cases, and severe, fulminant disease can ccur. In additin t RMSF, human illness assciated with ther sptted fever grup Rickettsia species, including infectin with Rickettsia parkeri (assciated with Amblymma maculatum ticks), has als been reprted. In these patients, clinical presentatin appears similar t, but may be milder than, RMSF; the presence f an eschar at the site f tick attachment has been reprted fr sme ther sptted fever rickettsises. Clinical evidence Any reprted fever and ne r mre f the fllwing: rash, eschar, headache, myalgia, anemia, thrmbcytpenia, r any hepatic transaminase elevatin. Epidemilgic evidence Tick expsure in past 2 weeks; a tick bite is nt required. List ccupatin if relevant t expsure. Travel in past 2 weeks; lcatin f travel 5

Labratry evidence The rganism in the acute phase f illness is best detected by plymerase chain reactin (PCR) and immunhistchemical methds (IHC) in skin bipsy specimens, and ccasinally by PCR in apprpriate whle bld specimens taken during the first week f illness, prir t antibitic treatment. Serlgy can als be emplyed fr detectin, hwever an antibdy respnse may nt be detectable in initial samples, and paired acute and cnvalescent samples are essential fr cnfirmatin. Fr the purpses f surveillance, Labratry cnfirmed: Serlgical evidence f a furfld change in immunglbulin G (IgG)-specific antibdy titer reactive with Rickettsia rickettsii r ther sptted fever grup antigen by indirect immunflurescence assay (IFA) between paired serum specimens (ne taken in the first week f illness and a secnd 2-4 weeks later), r Detectin f R. rickettsii r ther sptted fever grup DNA in a clinical specimen via amplificatin f a specific target by PCR assay, r Demnstratin f sptted fever grup antigen in a bipsy r autpsy specimen by IHC, r Islatin f R. rickettsii r ther sptted fever grup rickettsia frm a clinical specimen in cell culture. Labratry supprtive: Has serlgic evidence f elevated IgG r IgM antibdy reactive with R. rickettsii r ther sptted fever grup antigen by IFA, enzyme-linked immunsrbent assay (ELISA), dt-elisa, r latex agglutinatin. Nte: Current cmmercially available ELISA tests are nt quantitative, cannt be used t evaluate changes in antibdy titer, and hence are nt useful fr serlgical cnfirmatin. IgM tests are nt strngly supprted fr use in serdiagnsis f acute disease, as the respnse may nt be specific fr the agent (resulting in false psitives) and the IgM respnse may be persistent. Cmplement fixatin (CF) tests and ther lder test methds are neither readily available nr cmmnly used. CDC uses in-huse IFA IgG testing (cutff f 1:64), preferring simultaneus testing f paired specimens, and des nt use IgM results fr rutine diagnstic testing. Expsure Expsure is defined as having been in ptential tick habitats within the past 14 days befre nset f symptms. ccupatin shuld be recrded if relevant t expsure. A histry f a tick bite is nt required. 6

Case Classificatin Cnfirmed: A clinically cmpatible case (meets clinical evidence criteria) that is labratry cnfirmed. Prbable: A clinically cmpatible case (meets clinical evidence criteria) that has supprtive labratry results. Suspect: A case with labratry evidence f past r present infectin but n clinical infrmatin available (e.g. a labratry reprt). B. Classificatin Tables Table VII-B lists the criteria that must be met fr a case t be classified as cnfirmed, prbable (presumptive), r suspected (pssible). Table VII-B. Prpsed table f criteria t determine whether a case is classified. Nte: The fllwing criteria are prpsed fr evaluatin befre general implementatin. Fr purpses f current ntificatin, the narrative descriptin in VII-A, shuld be used. Case Definitin Criterin Cnfirmed Prbable Suspected Clinical Presentatin Fever N N N N Rash C C Eschar C C Headache C C Myalgia C C Clinical Labratry Findings Anemia C C Thrmbcytpenia C C Increased Hepatic Transaminases C C Diagnstic Labratry Findings Furfld change in Rickettsia rickettsii r ther sptted fever grup-specific IgG antibdy titer by indirect 7

immunflurescence assay (IFA) with paired serum specimens Elevated Rickettsia rickettsiir ther sptted fever grup specific IgG antibdy titer by indirect immunflurescence assay (IFA), Elisa, Elisa-dt r latex agglutinatin n a single serum specimen Elevated Rickettsia rickettsiir ther sptted fever grup specific IgM antibdy titer by indirect immunflurescence assay (IFA), Elisa, Elisa-dt r latex agglutinatin n a single serum specimen Detectin f R. rickettsii r ther sptted fever grup DNA in a clinical specimen by PCR assay Demnstratin f sptted fever grup antigen in a bipsy r autpsy specimen by IHC Islatin f R. rickettsii r ther sptted fever grup rickettsia frm a clinical specimen in cell culture Ntes: N = This criterin in cnjunctin with all ther N and any criteria in the same clumn is required t classify a case. = At least ne f these criteria in each categry in the same clumn (e.g., clinical presentatin and labratry findings) in cnjunctin with all ther N criteria in the same clumn is required t classify a case. C = This finding crrbrates (i.e., supprts) the diagnsis f r is assciated with Rcky Muntain sptted fever, but is nt included in the case definitin and is nt required fr classificatin. VIII. Perid f Surveillance Surveillance shuld be n-ging. 8

I. Data sharing/release and print criteria Ntificatin t CDC f prbable and cnfirmed cases f Sptted Fever Rickettsisis is recmmended. Summaries and analyses f reprted cases f Sptted Fever Rickettsisis, including RMSF, are cmpiled and published peridically dependent upn accumulatin f data and changes in disease activity and reginal incidence. A manuscript cvering diagnsis and management f Rickettsial Diseases in the U.S. was published in 2006. Annual state case ttals and natinal incidence rates are available via MMWR. Final verificatin f case cunts with SHDs is usually cmpleted by August f the year fllwing the surveillance year and reprted annually in the Natinal Summary f Ntifiable Diseases - United States published in March r April f the subsequent year. Aggregate numbers f cases the United States are available t WH via MMWR.. References 1. Centers fr Disease Cntrl and Preventin (CDC). Case definitins fr infectius cnditins under public health surveillance. MMWR 1997; 46(N. RR-10):1 57. Available frm: http://www.cdc.gv/mmwr/ 2. Centers fr Disease Cntrl and Preventin (CDC). Natinal ntifiable diseases surveillance system: case definitins. Atlanta: CDC. Available frm: http://www.cdc.gv/ncphi/disss/nndss/casedef/index.htm Last updated: 2008 Jan 9. Accessed: 3. Cuncil f State and Territrial Epidemilgists (CSTE). CSTE fficial list f natinally ntifiable cnditins. CSTE psitin statement 07-EC-02. Atlanta: CSTE; June 2007. Available frm: http://www.cste.rg. 4. Cuncil f State and Territrial Epidemilgists (CSTE). Criteria fr inclusin f cnditins n CSTE natinally ntifiable cnditin list and fr categrizatin as immediately r rutinely ntifiable. CSTE psitin statement 08-EC-02. Atlanta: CSTE; June 2008. Available frm: http://www.cste.rg. 5. Cuncil f State and Territrial Epidemilgists (CSTE). Rcky Muntain sptted fever. 03-ID-08. Atlanta: CSTE; June 2003. Available frm: http://www.cste.rg. 6. Cuncil f State and Territrial Epidemilgists (CSTE). Data Release Guidelines f the Cuncil f State & Territrial Epidemilgists fr the Natinal Public Health System. Atlanta: CSTE; June 1996. 9

7. Cuncil f State and Territrial Epidemilgists, Centers fr Disease Cntrl and Preventin. CDC-CSTE Intergvernmental Data Release Guidelines Wrking Grup (DRGWG) Reprt: CDC-ATSDR Data Release Guidelines and Prcedures fr Re-release f State-Prvided Data. Atlanta: CSTE; 2005. Available frm: http://www.cste.rg/pdffiles/2005/drgwgreprt.pdf r http://www.cdc.gv/d/fia/plicies/drgwg.pdf. 8. Heymann DL, editr. Cntrl f cmmunicable diseases manual. 18th editin. Washingtn: American Public Health Assciatin; 2004. 9. Walker DH, Rault D. Rickettsia rickettsii and ther Sptted Fever Grup Rickettsiae (Rcky Muntain Sptted Fever and ther Sptted Fevers) In: Mandell GL, Bennett JE, Dlin R, editrs. Principles and Practice f Infectius Diseases, 6th editin. Philadelphia: Churchill Livingstne; 2005. I. Crdinatin: Agencies fr Respnse: (1) Thmas R. Frieden, MD, MPH Directr Centers fr Disease Cntrl and Preventin 1600 Cliftn Rad, NE Atlanta GA 30333 (404) 639-7000 txf2@cdc.gv II. Submitting Authr: (1) Lauri Smithee Chief, Acute Disease Service klahma State Department f Health 1000 NE 10 th St. klahma City, K 73117-1299 LauriS@health.k.gv C-Authrs: (1) Assciate Member Harry F. Hull, Medical Epidemilgist HF Hull & Assciates, LLC 1140 St. Dennis Curt Saint Paul, MN 55116 (651) 695-8114 hullhf@msn.cm 10

(2) Assciate Member Cecil Lynch, Medical Infrmaticist ntreasn 7292 Shady Wds Circle Midvale, UT 84047 (916) 412.5504 clynch@ntreasn.cm (3) Assciate Member R. Gibsn Parrish, Medical Epidemilgist P.. Bx 197 480 Bayley Hazen Rad Peacham, VT 05862 (802) 592-3357 gib.parrish@gmail.cm 11