Whipple's disease natural history, complications and prognosis: Difference between revisions

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==Overview==
==Overview==
[[Tropheryma whipplei|''Tropheryma whipplei'']] [[infection]] has different [[clinical]] manifestations. It could cause [[acute]] [[infection]], localized [[infection]] and the classic Whipple's disease. [[Acute]] [[infection]] might present with [[gastroenteritis]], [[pneumonia]] or [[bacteremia]]. Acute infection might resolve without treatment but usually progress to systemic infection or [[carrier]] state. Classic Whipple's disease has 3 [[clinical]] phases that starts with nonspecific [[symptoms]] and [[joint pain]]. It progresses to [[gastrointestinal]] [[symptoms]] such as [[diarrhea]], [[steatorrhea]], [[malabsorption]], and [[weight loss]]. in the late phase, all the other [[organs]] including [[CNS]], [[joints]], [[eyes]], [[heart]], [[lung]], [[liver]] and [[skin]] might be involved. The risk of [[relapse]] is approximately 40%, if treatment is not completed. [[Relapse]] of Whipple's disease might occur up to 30 years after treatment and it is commonly responsible for [[Morbidity & Mortality|morbidity and mortality]]. Common [[complications]] of Whipple's disease include [[malnutrition]], [[cardiopulmonary]], [[neurologic]] and osteoarticular involvement. [[Malabsorption]] mostly presents with [[fat-soluble]] [[vitamin deficiency]], [[fatigue]], and [[weight loss]]. [[Valvular heart disease]] and [[dementia]] are the most common [[cardiac]] and [[neurologic]] complication, respectively. The prognosis of Whipple's disease is good if diagnosed properly and long-term treatment started early. Without treatment, Whipple's disease is [[fatal]].
==Natural History, Complications, and Prognosis==
==Natural History, Complications, and Prognosis==


===Natural History===
===Natural History===
[[Tropheryma whipplei]] [[infection]] has different clinical manifestations. It could cause acute infection, localized infection and the classic Whipple's disease. Each of them has its own progression and natural history.  
[[Tropheryma whipplei|''Tropheryma whipplei'']] [[infection]] has different clinical manifestations. It could cause [[acute]] [[infection]], localized [[infection]] and the classic Whipple's disease. Each of them has its own progression and natural history. Acute infection might resolve without treatment but usually progress to systemic infection or [[carrier]] state. The risk of [[relapse]] is approximately 40%, if treatment is not completed. [[Relapse]] of Whipple's disease might occur up to 30 years after treatment and it is commonly responsible for [[Morbidity & Mortality|morbidity and mortality]].<ref name="MarthMoos2016" />


==== Acute infection ====
==== Acute infection ====
*[[Tropheryma whipplei]] causes acute infections, including:
*[[Tropheryma whipplei|''Tropheryma whipplei'']] causes acute infections, including:
**[[Gastroenteritis]]: Patients have watery [[diarrhea]], [[steatorrhea]], and colicky [[abdominal pain]].<ref name="RaoultFenollar2010">{{cite journal|last1=Raoult|first1=Didier|last2=Fenollar|first2=Florence|last3=Rolain|first3=Jean-Marc|last4=Minodier|first4=Philippe|last5=Bosdure|first5=Emmanuelle|last6=Li|first6=Wenjun|last7=Garnier|first7=Jean-Marc|last8=Richet|first8=Hervé|title=Tropheryma whipplei in Children with Gastroenteritis|journal=Emerging Infectious Diseases|volume=16|issue=5|year=2010|pages=776–782|issn=1080-6040|doi=10.3201/eid1605.091801}}</ref>
**[[Gastroenteritis]]: Patients have watery [[diarrhea]], [[steatorrhea]], and colicky [[abdominal pain]].<ref name="RaoultFenollar2010">{{cite journal|last1=Raoult|first1=Didier|last2=Fenollar|first2=Florence|last3=Rolain|first3=Jean-Marc|last4=Minodier|first4=Philippe|last5=Bosdure|first5=Emmanuelle|last6=Li|first6=Wenjun|last7=Garnier|first7=Jean-Marc|last8=Richet|first8=Hervé|title=Tropheryma whipplei in Children with Gastroenteritis|journal=Emerging Infectious Diseases|volume=16|issue=5|year=2010|pages=776–782|issn=1080-6040|doi=10.3201/eid1605.091801}}</ref>
**[[Pneumonia]]: [[Tropheryma whipplei]] causes [[pneumonia]] in [[HIV]] patients.<ref name="LozuponeCota-Gomez2013">{{cite journal|last1=Lozupone|first1=Catherine|last2=Cota-Gomez|first2=Adela|last3=Palmer|first3=Brent E.|last4=Linderman|first4=Derek J.|last5=Charlson|first5=Emily S.|last6=Sodergren|first6=Erica|last7=Mitreva|first7=Makedonka|last8=Abubucker|first8=Sahar|last9=Martin|first9=John|last10=Yao|first10=Guohui|last11=Campbell|first11=Thomas B.|last12=Flores|first12=Sonia C.|last13=Ackerman|first13=Gail|last14=Stombaugh|first14=Jesse|last15=Ursell|first15=Luke|last16=Beck|first16=James M.|last17=Curtis|first17=Jeffrey L.|last18=Young|first18=Vincent B.|last19=Lynch|first19=Susan V.|last20=Huang|first20=Laurence|last21=Weinstock|first21=George M.|last22=Knox|first22=Kenneth S.|last23=Twigg|first23=Homer|last24=Morris|first24=Alison|last25=Ghedin|first25=Elodie|last26=Bushman|first26=Frederic D.|last27=Collman|first27=Ronald G.|last28=Knight|first28=Rob|last29=Fontenot|first29=Andrew P.|title=Widespread Colonization of the Lung byTropheryma whippleiin HIV Infection|journal=American Journal of Respiratory and Critical Care Medicine|volume=187|issue=10|year=2013|pages=1110–1117|issn=1073-449X|doi=10.1164/rccm.201211-2145OC}}</ref>
**[[Pneumonia]]: [[Tropheryma whipplei|''Tropheryma whipplei'']] causes [[pneumonia]] in [[HIV]] patients.<ref name="LozuponeCota-Gomez2013">{{cite journal|last1=Lozupone|first1=Catherine|last2=Cota-Gomez|first2=Adela|last3=Palmer|first3=Brent E.|last4=Linderman|first4=Derek J.|last5=Charlson|first5=Emily S.|last6=Sodergren|first6=Erica|last7=Mitreva|first7=Makedonka|last8=Abubucker|first8=Sahar|last9=Martin|first9=John|last10=Yao|first10=Guohui|last11=Campbell|first11=Thomas B.|last12=Flores|first12=Sonia C.|last13=Ackerman|first13=Gail|last14=Stombaugh|first14=Jesse|last15=Ursell|first15=Luke|last16=Beck|first16=James M.|last17=Curtis|first17=Jeffrey L.|last18=Young|first18=Vincent B.|last19=Lynch|first19=Susan V.|last20=Huang|first20=Laurence|last21=Weinstock|first21=George M.|last22=Knox|first22=Kenneth S.|last23=Twigg|first23=Homer|last24=Morris|first24=Alison|last25=Ghedin|first25=Elodie|last26=Bushman|first26=Frederic D.|last27=Collman|first27=Ronald G.|last28=Knight|first28=Rob|last29=Fontenot|first29=Andrew P.|title=Widespread Colonization of the Lung byTropheryma whippleiin HIV Infection|journal=American Journal of Respiratory and Critical Care Medicine|volume=187|issue=10|year=2013|pages=1110–1117|issn=1073-449X|doi=10.1164/rccm.201211-2145OC}}</ref>
**[[Bacteremia]]: [[Tropheryma whipplei]] [[infection]] could cause self-limiting [[bacteremia]].<ref name="FenollarMediannikov2010">{{cite journal|last1=Fenollar|first1=Florence|last2=Mediannikov|first2=Oleg|last3=Socolovschi|first3=Cristina|last4=Bassene|first4=Hubert|last5=Diatta|first5=Georges|last6=Richet|first6=Hervé|last7=Tall|first7=Adama|last8=Sokhna|first8=Cheikh|last9=Trape|first9=Jean‐François|last10=Raoult|first10=Didier|title=Tropheryma whippleiBacteremia during Fever in Rural West Africa|journal=Clinical Infectious Diseases|volume=51|issue=5|year=2010|pages=515–521|issn=1058-4838|doi=10.1086/655677}}</ref>
**[[Bacteremia]]: [[Tropheryma whipplei|''Tropheryma whipplei'']] [[infection]] could cause self-limiting [[bacteremia]].<ref name="FenollarMediannikov2010">{{cite journal|last1=Fenollar|first1=Florence|last2=Mediannikov|first2=Oleg|last3=Socolovschi|first3=Cristina|last4=Bassene|first4=Hubert|last5=Diatta|first5=Georges|last6=Richet|first6=Hervé|last7=Tall|first7=Adama|last8=Sokhna|first8=Cheikh|last9=Trape|first9=Jean‐François|last10=Raoult|first10=Didier|title=Tropheryma whippleiBacteremia during Fever in Rural West Africa|journal=Clinical Infectious Diseases|volume=51|issue=5|year=2010|pages=515–521|issn=1058-4838|doi=10.1086/655677}}</ref>
==== Classic Whipple's disease ====
==== Classic Whipple's disease ====
Patients who developed the classic Whipple's disease usually have 3 clinical phases:
Patients who developed the classic Whipple's disease usually have 3 clinical phases:
*In the early phase, patients have nonspecific symptoms including [[fever]] and [[arthralgia]] that could last for years.<ref name="BaiMazure2004">{{cite journal|last1=Bai|first1=J|last2=Mazure|first2=R|last3=Vazquez|first3=H|last4=Niveloni|first4=S|last5=Smecuol|first5=E|last6=Pedreira|first6=S|last7=Maurino|first7=E|title=Whipple’s disease|journal=Clinical Gastroenterology and Hepatology|volume=2|issue=10|year=2004|pages=849–860|issn=15423565|doi=10.1016/S1542-3565(04)00387-8}}</ref>
*In the early phase, patients have nonspecific symptoms including [[fever]] and [[arthralgia]] that could last for years.<ref name="BaiMazure2004">{{cite journal|last1=Bai|first1=J|last2=Mazure|first2=R|last3=Vazquez|first3=H|last4=Niveloni|first4=S|last5=Smecuol|first5=E|last6=Pedreira|first6=S|last7=Maurino|first7=E|title=Whipple’s disease|journal=Clinical Gastroenterology and Hepatology|volume=2|issue=10|year=2004|pages=849–860|issn=15423565|doi=10.1016/S1542-3565(04)00387-8}}</ref>
*In the middle phase, patients develop [[gastrointestinal]] symptoms including [[diarrhea]], [[abdominal pain]] and [[weight loss]].<ref name="Puéchal2013">{{cite journal|last1=Puéchal|first1=Xavier|title=Whipple's disease|journal=Annals of the Rheumatic Diseases|volume=72|issue=6|year=2013|pages=797–803|issn=0003-4967|doi=10.1136/annrheumdis-2012-202684}}</ref>
*In the middle phase, patients develop [[gastrointestinal]] symptoms including [[diarrhea]], [[abdominal pain]] and [[weight loss]].<ref name="Puéchal2013">{{cite journal|last1=Puéchal|first1=Xavier|title=Whipple's disease|journal=Annals of the Rheumatic Diseases|volume=72|issue=6|year=2013|pages=797–803|issn=0003-4967|doi=10.1136/annrheumdis-2012-202684}}</ref>
*In the late phase, other organs might be involved. [[Neurological disease|Neurologic]] symptoms including progressive [[dementia]], [[personality changes]] and [[seizures]] might happen. [[Eyes]], [[heart]], [[lung]], [[liver]], and [[skin]] can be involved in this phase.<ref name="FlemingWiesner1988">{{cite journal|last1=Fleming|first1=Jon L.|last2=Wiesner|first2=Russell H.|last3=Shorter|first3=Roy G.|title=Whipple's Disease: Clinical, Biochemical, and Histopathologic Features and Assessment of Treatment in 29 Patients|journal=Mayo Clinic Proceedings|volume=63|issue=6|year=1988|pages=539–551|issn=00256196|doi=10.1016/S0025-6196(12)64884-8}}</ref>
*In the late phase, other organs might be involved. Neurologic symptoms including progressive [[dementia]], [[personality changes]] and [[seizures]] might happen. [[Eyes]], [[heart]], [[lung]], [[liver]], and [[skin]] can be involved in this phase.<ref name="FlemingWiesner1988">{{cite journal|last1=Fleming|first1=Jon L.|last2=Wiesner|first2=Russell H.|last3=Shorter|first3=Roy G.|title=Whipple's Disease: Clinical, Biochemical, and Histopathologic Features and Assessment of Treatment in 29 Patients|journal=Mayo Clinic Proceedings|volume=63|issue=6|year=1988|pages=539–551|issn=00256196|doi=10.1016/S0025-6196(12)64884-8}}</ref>


==== Localized infection ====
==== Localized infection ====
Patients might present with localized infection instead of systemic involvement after being infected by [[Tropheryma whipplei]].
Patients might present with localized infection instead of systemic involvement after being infected by [[Tropheryma whipplei|''Tropheryma whipplei'']].
*[[Endocarditis]]: Patients might develop blood [[culture-negative endocarditis]]. Since it is hard to diagnose [[Tropheryma whipplei]] infection without the systemic symptoms, if left untreated, it could be lethal.<ref name="GeissdorferMoos2011">{{cite journal|last1=Geissdorfer|first1=W.|last2=Moos|first2=V.|last3=Moter|first3=A.|last4=Loddenkemper|first4=C.|last5=Jansen|first5=A.|last6=Tandler|first6=R.|last7=Morguet|first7=A. J.|last8=Fenollar|first8=F.|last9=Raoult|first9=D.|last10=Bogdan|first10=C.|last11=Schneider|first11=T.|title=High Frequency of Tropheryma whipplei in Culture-Negative Endocarditis|journal=Journal of Clinical Microbiology|volume=50|issue=2|year=2011|pages=216–222|issn=0095-1137|doi=10.1128/JCM.05531-11}}</ref>
*[[Endocarditis]]: Patients might develop blood [[culture-negative endocarditis]].<ref name="GeissdorferMoos2011">{{cite journal|last1=Geissdorfer|first1=W.|last2=Moos|first2=V.|last3=Moter|first3=A.|last4=Loddenkemper|first4=C.|last5=Jansen|first5=A.|last6=Tandler|first6=R.|last7=Morguet|first7=A. J.|last8=Fenollar|first8=F.|last9=Raoult|first9=D.|last10=Bogdan|first10=C.|last11=Schneider|first11=T.|title=High Frequency of Tropheryma whipplei in Culture-Negative Endocarditis|journal=Journal of Clinical Microbiology|volume=50|issue=2|year=2011|pages=216–222|issn=0095-1137|doi=10.1128/JCM.05531-11}}</ref>


*[[Encephalitis]]: [[Ataxia]] and [[dementia]] are common. Empirical [[antibiotic]] therapy might be considered for rapid resolution.<ref name="FenollarNicoli2011">{{cite journal|last1=Fenollar|first1=Florence|last2=Nicoli|first2=François|last3=Paquet|first3=Claire|last4=Lepidi|first4=Hubert|last5=Cozzone|first5=Patrick|last6=Antoine|first6=Jean-Christophe|last7=Pouget|first7=Jean|last8=Raoult|first8=Didier|title=Progressive dementia associated with ataxia or obesity in patients with Tropheryma whipplei encephalitis|journal=BMC Infectious Diseases|volume=11|issue=1|year=2011|issn=1471-2334|doi=10.1186/1471-2334-11-171}}</ref>
*[[Encephalitis]]: [[Ataxia]] and [[dementia]] are common. Empirical [[antibiotic]] therapy might be considered for rapid resolution.<ref name="FenollarNicoli2011">{{cite journal|last1=Fenollar|first1=Florence|last2=Nicoli|first2=François|last3=Paquet|first3=Claire|last4=Lepidi|first4=Hubert|last5=Cozzone|first5=Patrick|last6=Antoine|first6=Jean-Christophe|last7=Pouget|first7=Jean|last8=Raoult|first8=Didier|title=Progressive dementia associated with ataxia or obesity in patients with Tropheryma whipplei encephalitis|journal=BMC Infectious Diseases|volume=11|issue=1|year=2011|issn=1471-2334|doi=10.1186/1471-2334-11-171}}</ref>
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*Osteoarticular involvement: Isolated [[arthritis]] and [[spondylodiscitis]] are happened without systemic manifestations.<ref name="Bruhlmann2000">{{cite journal|last1=Bruhlmann|first1=P.|title=Diagnosis and therapy monitoring of Whipple's arthritis by polymerase chain reaction|journal=Rheumatology|volume=39|issue=12|year=2000|pages=1427–1428|issn=14602172|doi=10.1093/rheumatology/39.12.1427}}</ref>
*Osteoarticular involvement: Isolated [[arthritis]] and [[spondylodiscitis]] are happened without systemic manifestations.<ref name="Bruhlmann2000">{{cite journal|last1=Bruhlmann|first1=P.|title=Diagnosis and therapy monitoring of Whipple's arthritis by polymerase chain reaction|journal=Rheumatology|volume=39|issue=12|year=2000|pages=1427–1428|issn=14602172|doi=10.1093/rheumatology/39.12.1427}}</ref>


*[[Eyes]] involvement: Patients might present with isolated [[uveitis]] and [[PCR]] of [[Aqueous humour|aqueous humor]] is used to establish the diagnosis.<ref name="BauerfeindKoelz1999">{{cite journal|last1=Bauerfeind|first1=Peter|last2=Koelz|first2=Hans-Rudolf|last3=Altwegg|first3=Martin|title=PCR for Tropheryma whippelii|journal=The Lancet|volume=354|issue=9188|year=1999|pages=1476–1477|issn=01406736|doi=10.1016/S0140-6736(05)77620-0}}</ref>
*[[Eyes]] involvement: Patients might present with isolated [[uveitis]]. [[PCR]] of [[Aqueous humour|aqueous humor]] is used to establish the diagnosis.<ref name="BauerfeindKoelz1999">{{cite journal|last1=Bauerfeind|first1=Peter|last2=Koelz|first2=Hans-Rudolf|last3=Altwegg|first3=Martin|title=PCR for Tropheryma whippelii|journal=The Lancet|volume=354|issue=9188|year=1999|pages=1476–1477|issn=01406736|doi=10.1016/S0140-6736(05)77620-0}}</ref>


===Complications===
===Complications===
Common complications of Whipple's disease include:<ref name="FenollarPuéchal2007">{{cite journal|last1=Fenollar|first1=Florence|last2=Puéchal|first2=Xavier|last3=Raoult|first3=Didier|title=Whipple's Disease|journal=New England Journal of Medicine|volume=356|issue=1|year=2007|pages=55–66|issn=0028-4793|doi=10.1056/NEJMra062477}}</ref><ref name="FenollarLagier2014">{{cite journal|last1=Fenollar|first1=Florence|last2=Lagier|first2=Jean-Christophe|last3=Raoult|first3=Didier|title=Tropheryma whipplei and Whipple's disease|journal=Journal of Infection|volume=69|issue=2|year=2014|pages=103–112|issn=01634453|doi=10.1016/j.jinf.2014.05.008}}</ref><ref name="BurešKopáčová2013">{{cite journal|last1=Bureš|first1=Jan|last2=Kopáčová|first2=Marcela|last3=Douda|first3=Tomáš|last4=Bártová|first4=Jolana|last5=Tomš|first5=Jan|last6=Rejchrt|first6=Stanislav|last7=Tachecí|first7=Ilja|title=Whipple’s Disease: Our Own Experience and Review of the Literature|journal=Gastroenterology Research and Practice|volume=2013|year=2013|pages=1–10|issn=1687-6121|doi=10.1155/2013/478349}}</ref><ref name="DutlyAltwegg2001">{{cite journal|last1=Dutly|first1=F.|last2=Altwegg|first2=M.|title=Whipple's Disease and "Tropheryma whippelii"|journal=Clinical Microbiology Reviews|volume=14|issue=3|year=2001|pages=561–583|issn=0893-8512|doi=10.1128/CMR.14.3.561-583.2001}}</ref><ref name="MarthMoos2016">{{cite journal|last1=Marth|first1=Thomas|last2=Moos|first2=Verena|last3=Müller|first3=Christian|last4=Biagi|first4=Federico|last5=Schneider|first5=Thomas|title=Tropheryma whipplei infection and Whipple's disease|journal=The Lancet Infectious Diseases|volume=16|issue=3|year=2016|pages=e13–e22|issn=14733099|doi=10.1016/S1473-3099(15)00537-X}}</ref>
Common complications of Whipple's disease include:<ref name="FenollarLagier2014">{{cite journal|last1=Fenollar|first1=Florence|last2=Lagier|first2=Jean-Christophe|last3=Raoult|first3=Didier|title=Tropheryma whipplei and Whipple's disease|journal=Journal of Infection|volume=69|issue=2|year=2014|pages=103–112|issn=01634453|doi=10.1016/j.jinf.2014.05.008}}</ref><ref name="BurešKopáčová2013">{{cite journal|last1=Bureš|first1=Jan|last2=Kopáčová|first2=Marcela|last3=Douda|first3=Tomáš|last4=Bártová|first4=Jolana|last5=Tomš|first5=Jan|last6=Rejchrt|first6=Stanislav|last7=Tachecí|first7=Ilja|title=Whipple’s Disease: Our Own Experience and Review of the Literature|journal=Gastroenterology Research and Practice|volume=2013|year=2013|pages=1–10|issn=1687-6121|doi=10.1155/2013/478349}}</ref><ref name="DutlyAltwegg2001">{{cite journal|last1=Dutly|first1=F.|last2=Altwegg|first2=M.|title=Whipple's Disease and "Tropheryma whippelii"|journal=Clinical Microbiology Reviews|volume=14|issue=3|year=2001|pages=561–583|issn=0893-8512|doi=10.1128/CMR.14.3.561-583.2001}}</ref><ref name="MarthMoos2016">{{cite journal|last1=Marth|first1=Thomas|last2=Moos|first2=Verena|last3=Müller|first3=Christian|last4=Biagi|first4=Federico|last5=Schneider|first5=Thomas|title=Tropheryma whipplei infection and Whipple's disease|journal=The Lancet Infectious Diseases|volume=16|issue=3|year=2016|pages=e13–e22|issn=14733099|doi=10.1016/S1473-3099(15)00537-X}}</ref><ref name="FenollarPuéchal2007">{{cite journal|last1=Fenollar|first1=Florence|last2=Puéchal|first2=Xavier|last3=Raoult|first3=Didier|title=Whipple's Disease|journal=New England Journal of Medicine|volume=356|issue=1|year=2007|pages=55–66|issn=0028-4793|doi=10.1056/NEJMra062477}}</ref>
*'''Malnutrition'''
*'''Malnutrition'''
**[[Vitamin deficiency]]
**[[Vitamin deficiency]]
***[[Vitamin A deficiency]]
***[[Vitamin A deficiency]] that presents with [[night blindness]].
***[[Vitamin D deficiency]]
***[[Vitamin D deficiency]] that presents with nonspecific symptoms including [[fatigue]], [[muscle pain]] and [[osteomalacia]].
***[[Vitamin E deficiency]]
***[[Vitamin E deficiency]] that presents with [[neuromuscular]] problems and [[immunity]] impairment. 
***[[Vitamin K deficiency]]
***[[Vitamin K deficiency]] that presents with [[bleeding]].
**[[Weight loss]]
**[[Weight loss]]
*'''Cardiac complications'''
*'''Cardiac complications'''
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===Prognosis===
===Prognosis===
*Prognosis is generally very poor, if left untreated.<ref name="FenollarLagier2014">{{cite journal|last1=Fenollar|first1=Florence|last2=Lagier|first2=Jean-Christophe|last3=Raoult|first3=Didier|title=Tropheryma whipplei and Whipple's disease|journal=Journal of Infection|volume=69|issue=2|year=2014|pages=103–112|issn=01634453|doi=10.1016/j.jinf.2014.05.008}}</ref>
*The prognosis of Whipple's disease is good if diagnosed properly and long-term treatment started early.<ref name="BurešKopáčová2013">{{cite journal|last1=Bureš|first1=Jan|last2=Kopáčová|first2=Marcela|last3=Douda|first3=Tomáš|last4=Bártová|first4=Jolana|last5=Tomš|first5=Jan|last6=Rejchrt|first6=Stanislav|last7=Tachecí|first7=Ilja|title=Whipple’s Disease: Our Own Experience and Review of the Literature|journal=Gastroenterology Research and Practice|volume=2013|year=2013|pages=1–10|issn=1687-6121|doi=10.1155/2013/478349}}</ref>
*The prognosis of Whipple's disease is good if diagnosed properly and long-term treatment started early.<ref name="BurešKopáčová2013">{{cite journal|last1=Bureš|first1=Jan|last2=Kopáčová|first2=Marcela|last3=Douda|first3=Tomáš|last4=Bártová|first4=Jolana|last5=Tomš|first5=Jan|last6=Rejchrt|first6=Stanislav|last7=Tachecí|first7=Ilja|title=Whipple’s Disease: Our Own Experience and Review of the Literature|journal=Gastroenterology Research and Practice|volume=2013|year=2013|pages=1–10|issn=1687-6121|doi=10.1155/2013/478349}}</ref>
*The prognosis of Whipple's disease is generally very poor if left untreated.<ref name="FenollarLagier2014">{{cite journal|last1=Fenollar|first1=Florence|last2=Lagier|first2=Jean-Christophe|last3=Raoult|first3=Didier|title=Tropheryma whipplei and Whipple's disease|journal=Journal of Infection|volume=69|issue=2|year=2014|pages=103–112|issn=01634453|doi=10.1016/j.jinf.2014.05.008}}</ref>
*The presence of [[neurologic]] symptoms is associated with a particularly poor prognosis among patients with Whipple's disease.<ref name="DutlyAltwegg2001">{{cite journal|last1=Dutly|first1=F.|last2=Altwegg|first2=M.|title=Whipple's Disease and "Tropheryma whippelii"|journal=Clinical Microbiology Reviews|volume=14|issue=3|year=2001|pages=561–583|issn=0893-8512|doi=10.1128/CMR.14.3.561-583.2001}}</ref>
*The presence of [[neurologic]] symptoms is associated with a particularly poor prognosis among patients with Whipple's disease.<ref name="DutlyAltwegg2001">{{cite journal|last1=Dutly|first1=F.|last2=Altwegg|first2=M.|title=Whipple's Disease and "Tropheryma whippelii"|journal=Clinical Microbiology Reviews|volume=14|issue=3|year=2001|pages=561–583|issn=0893-8512|doi=10.1128/CMR.14.3.561-583.2001}}</ref>
*The risk of [[relapse]] is approximately 40%, if treatment is not completed.<ref name="MarthMoos2016">{{cite journal|last1=Marth|first1=Thomas|last2=Moos|first2=Verena|last3=Müller|first3=Christian|last4=Biagi|first4=Federico|last5=Schneider|first5=Thomas|title=Tropheryma whipplei infection and Whipple's disease|journal=The Lancet Infectious Diseases|volume=16|issue=3|year=2016|pages=e13–e22|issn=14733099|doi=10.1016/S1473-3099(15)00537-X}}</ref>
*[[Relapse]] of Whipple's disease might occur up to 30 years after treatment and it is commonly responsible for [[Morbidity & Mortality|morbidity and mortality]].<ref name="MarthMoos2016">{{cite journal|last1=Marth|first1=Thomas|last2=Moos|first2=Verena|last3=Müller|first3=Christian|last4=Biagi|first4=Federico|last5=Schneider|first5=Thomas|title=Tropheryma whipplei infection and Whipple's disease|journal=The Lancet Infectious Diseases|volume=16|issue=3|year=2016|pages=e13–e22|issn=14733099|doi=10.1016/S1473-3099(15)00537-X}}</ref>


==References==
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[[Category:Medicine]]
[[Category:Gastroenterology]]
[[Category:Infectious disease]]
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Latest revision as of 00:44, 30 July 2020

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sadaf Sharfaei M.D.[2]

Overview

Tropheryma whipplei infection has different clinical manifestations. It could cause acute infection, localized infection and the classic Whipple's disease. Acute infection might present with gastroenteritis, pneumonia or bacteremia. Acute infection might resolve without treatment but usually progress to systemic infection or carrier state. Classic Whipple's disease has 3 clinical phases that starts with nonspecific symptoms and joint pain. It progresses to gastrointestinal symptoms such as diarrhea, steatorrhea, malabsorption, and weight loss. in the late phase, all the other organs including CNS, joints, eyes, heart, lung, liver and skin might be involved. The risk of relapse is approximately 40%, if treatment is not completed. Relapse of Whipple's disease might occur up to 30 years after treatment and it is commonly responsible for morbidity and mortality. Common complications of Whipple's disease include malnutrition, cardiopulmonary, neurologic and osteoarticular involvement. Malabsorption mostly presents with fat-soluble vitamin deficiency, fatigue, and weight loss. Valvular heart disease and dementia are the most common cardiac and neurologic complication, respectively. The prognosis of Whipple's disease is good if diagnosed properly and long-term treatment started early. Without treatment, Whipple's disease is fatal.

Natural History, Complications, and Prognosis

Natural History

Tropheryma whipplei infection has different clinical manifestations. It could cause acute infection, localized infection and the classic Whipple's disease. Each of them has its own progression and natural history. Acute infection might resolve without treatment but usually progress to systemic infection or carrier state. The risk of relapse is approximately 40%, if treatment is not completed. Relapse of Whipple's disease might occur up to 30 years after treatment and it is commonly responsible for morbidity and mortality.[1]

Acute infection

Classic Whipple's disease

Patients who developed the classic Whipple's disease usually have 3 clinical phases:

Localized infection

Patients might present with localized infection instead of systemic involvement after being infected by Tropheryma whipplei.

Complications

Common complications of Whipple's disease include:[13][14][15][1][16]

Prognosis

  • The prognosis of Whipple's disease is good if diagnosed properly and long-term treatment started early.[14]
  • The prognosis of Whipple's disease is generally very poor if left untreated.[13]
  • The presence of neurologic symptoms is associated with a particularly poor prognosis among patients with Whipple's disease.[15]

References

  1. ↑ 1.0 1.1 Marth, Thomas; Moos, Verena; Müller, Christian; Biagi, Federico; Schneider, Thomas (2016). "Tropheryma whipplei infection and Whipple's disease". The Lancet Infectious Diseases. 16 (3): e13–e22. doi:10.1016/S1473-3099(15)00537-X. ISSN 1473-3099.
  2. ↑ Raoult, Didier; Fenollar, Florence; Rolain, Jean-Marc; Minodier, Philippe; Bosdure, Emmanuelle; Li, Wenjun; Garnier, Jean-Marc; Richet, Hervé (2010). "Tropheryma whipplei in Children with Gastroenteritis". Emerging Infectious Diseases. 16 (5): 776–782. doi:10.3201/eid1605.091801. ISSN 1080-6040.
  3. ↑ Lozupone, Catherine; Cota-Gomez, Adela; Palmer, Brent E.; Linderman, Derek J.; Charlson, Emily S.; Sodergren, Erica; Mitreva, Makedonka; Abubucker, Sahar; Martin, John; Yao, Guohui; Campbell, Thomas B.; Flores, Sonia C.; Ackerman, Gail; Stombaugh, Jesse; Ursell, Luke; Beck, James M.; Curtis, Jeffrey L.; Young, Vincent B.; Lynch, Susan V.; Huang, Laurence; Weinstock, George M.; Knox, Kenneth S.; Twigg, Homer; Morris, Alison; Ghedin, Elodie; Bushman, Frederic D.; Collman, Ronald G.; Knight, Rob; Fontenot, Andrew P. (2013). "Widespread Colonization of the Lung byTropheryma whippleiin HIV Infection". American Journal of Respiratory and Critical Care Medicine. 187 (10): 1110–1117. doi:10.1164/rccm.201211-2145OC. ISSN 1073-449X.
  4. ↑ Fenollar, Florence; Mediannikov, Oleg; Socolovschi, Cristina; Bassene, Hubert; Diatta, Georges; Richet, Hervé; Tall, Adama; Sokhna, Cheikh; Trape, Jean‐François; Raoult, Didier (2010). "Tropheryma whippleiBacteremia during Fever in Rural West Africa". Clinical Infectious Diseases. 51 (5): 515–521. doi:10.1086/655677. ISSN 1058-4838.
  5. ↑ Bai, J; Mazure, R; Vazquez, H; Niveloni, S; Smecuol, E; Pedreira, S; Maurino, E (2004). "Whipple's disease". Clinical Gastroenterology and Hepatology. 2 (10): 849–860. doi:10.1016/S1542-3565(04)00387-8. ISSN 1542-3565.
  6. ↑ Puéchal, Xavier (2013). "Whipple's disease". Annals of the Rheumatic Diseases. 72 (6): 797–803. doi:10.1136/annrheumdis-2012-202684. ISSN 0003-4967.
  7. ↑ Fleming, Jon L.; Wiesner, Russell H.; Shorter, Roy G. (1988). "Whipple's Disease: Clinical, Biochemical, and Histopathologic Features and Assessment of Treatment in 29 Patients". Mayo Clinic Proceedings. 63 (6): 539–551. doi:10.1016/S0025-6196(12)64884-8. ISSN 0025-6196.
  8. ↑ Geissdorfer, W.; Moos, V.; Moter, A.; Loddenkemper, C.; Jansen, A.; Tandler, R.; Morguet, A. J.; Fenollar, F.; Raoult, D.; Bogdan, C.; Schneider, T. (2011). "High Frequency of Tropheryma whipplei in Culture-Negative Endocarditis". Journal of Clinical Microbiology. 50 (2): 216–222. doi:10.1128/JCM.05531-11. ISSN 0095-1137.
  9. ↑ Fenollar, Florence; Nicoli, François; Paquet, Claire; Lepidi, Hubert; Cozzone, Patrick; Antoine, Jean-Christophe; Pouget, Jean; Raoult, Didier (2011). "Progressive dementia associated with ataxia or obesity in patients with Tropheryma whipplei encephalitis". BMC Infectious Diseases. 11 (1). doi:10.1186/1471-2334-11-171. ISSN 1471-2334.
  10. ↑ Urbanski, Geoffrey; Rivereau, Philippe; Artru, Laure; Fenollar, Florence; Raoult, Didier; Puéchal, Xavier (2012). "Whipple Disease Revealed by Lung Involvement". Chest. 141 (6): 1595–1598. doi:10.1378/chest.11-1812. ISSN 0012-3692.
  11. ↑ Bruhlmann, P. (2000). "Diagnosis and therapy monitoring of Whipple's arthritis by polymerase chain reaction". Rheumatology. 39 (12): 1427–1428. doi:10.1093/rheumatology/39.12.1427. ISSN 1460-2172.
  12. ↑ Bauerfeind, Peter; Koelz, Hans-Rudolf; Altwegg, Martin (1999). "PCR for Tropheryma whippelii". The Lancet. 354 (9188): 1476–1477. doi:10.1016/S0140-6736(05)77620-0. ISSN 0140-6736.
  13. ↑ 13.0 13.1 Fenollar, Florence; Lagier, Jean-Christophe; Raoult, Didier (2014). "Tropheryma whipplei and Whipple's disease". Journal of Infection. 69 (2): 103–112. doi:10.1016/j.jinf.2014.05.008. ISSN 0163-4453.
  14. ↑ 14.0 14.1 Bureš, Jan; Kopáčová, Marcela; Douda, Tomáš; Bártová, Jolana; Tomš, Jan; Rejchrt, Stanislav; Tachecí, Ilja (2013). "Whipple's Disease: Our Own Experience and Review of the Literature". Gastroenterology Research and Practice. 2013: 1–10. doi:10.1155/2013/478349. ISSN 1687-6121.
  15. ↑ 15.0 15.1 Dutly, F.; Altwegg, M. (2001). "Whipple's Disease and "Tropheryma whippelii"". Clinical Microbiology Reviews. 14 (3): 561–583. doi:10.1128/CMR.14.3.561-583.2001. ISSN 0893-8512.
  16. ↑ Fenollar, Florence; Puéchal, Xavier; Raoult, Didier (2007). "Whipple's Disease". New England Journal of Medicine. 356 (1): 55–66. doi:10.1056/NEJMra062477. ISSN 0028-4793.

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