TY  - CHAP
M1  - Book, Section
TI  - Whipple’s Disease
A1  - Russo, Thomas A.
A1  - Glassman, Seth R.
A2  - Loscalzo, Joseph
A2  - Fauci, Anthony
A2  - Kasper, Dennis
A2  - Hauser, Stephen
A2  - Longo, Dan
A2  - Jameson, J. Larry
PY  - 2022
T2  - Harrison's Principles of Internal Medicine, 21e
AB  - Whipple’s  disease  (WD),  described  by  George  Whipple  in  1907,  is  a  chronic  infection  caused  by  Tropheryma  whipplei.  Most  commonly,  years  pass  from  the  onset  of  symptoms  to  the  recognition  of  the  disease  because  of  its  rarity,  its  various  manifestations  mimicking  other  conditions,  and  the  need  to  perform  nonroutine  diagnostic  tests.  The  long-held  belief  that  WD  is  an  infection  was  supported  by  observations  on  its  responsiveness  to  antimicrobial  therapy  in  the  1950s  and  the  identification  of  bacilli  via  electron  microscopy  in  small-bowel  biopsy  specimens  in  the  1960s.  This  hypothesis  was  finally  confirmed  by  amplification  and  sequencing  of  a  partial  16S  rRNA  polymerase  chain  reaction  (PCR)–generated  amplicon  from  duodenal  tissue  in  1991.  The  subsequent  successful  cultivation  of  T.  whipplei  enabled  whole-genome  sequencing  and  the  development  of  additional  diagnostic  tests.  The  development  of  PCR-based  diagnostics  has  broadened  our  understanding  of  both  the  epidemiology  of  and  the  clinical  syndromes  attributable  to  T.  whipplei.  Exposure  to  T.  whipplei,  which  appears  to  be  much  more  common  than  has  been  appreciated,  can  be  followed  by  asymptomatic  carriage,  acute  disease,  or  chronic  infection.  Chronic  infection—WD—is  a  rare  development  after  exposure.  “Classic”  WD  is  manifested  by  some  combination  of  arthralgias/arthritis,  weight  loss,  chronic  diarrhea,  abdominal  pain,  and  fever.  Variable  involvement  at  other  sites  also  occurs;  neurologic  and  cardiac  disease  are  most  common.  Acute  infection  and  chronic  organ  disease  in  the  absence  of  intestinal  involvement  (see  “Isolated  Infection,”  below)  are  described  with  increasing  frequency.  Since  untreated  WD  is  often  fatal  and  delayed  diagnosis  may  lead  to  irreparable  organ  damage  (e.g.,  in  the  central  nervous  system  [CNS]),  knowledge  of  the  clinical  scenarios  in  which  Whipple’s  should  be  considered  and  of  an  appropriate  diagnostic  strategy  is  mandatory.  
SN  - 
PB  - McGraw-Hill Education
CY  - New York, NY
Y2  - 2024/04/16
UR  - accessmedicine.mhmedical.com/content.aspx?aid=1198222449
ER  -