Whipple's disease medical therapy: Difference between revisions

Jump to navigation Jump to search
m (Bot: Removing from Primary care)
 
(49 intermediate revisions by 2 users not shown)
Line 1: Line 1:
__NOTOC__
__NOTOC__
{{Whipple's disease}}
{{Whipple's disease}}
{{CMG}};{{AE}}{{SSH}}
{{CMG}}; {{AE}} {{SSH}}


==Overview==
==Overview==
The mainstay of treatment for Whipple's disease is systemic long-term [[antibiotic]] therapy.
[[Antimicrobial]] therapy is the mainstay of [[therapy]] for Whipple's disease. [[Intravenous]] [[ceftriaxone]] or [[penicillin G]] is indicated in the [[acute]] phase of Whipple's therapy. For maintenance therapy, patients are typically treated with [[Sulfamethoxazole-Trimethoprim|sulfamethoxazole-trimethoprim]] for at least 1 year. Patients who experience either Whipple's disease or [[allergy]] to [[Sulfamethoxazole-Trimethoprim|sulfamethoxazole-trimethoprim]] require a combination of [[doxycycline]] and [[hydroxychloroquine]]. [[Dietary supplement|Dietary supplements]] including [[vitamins]], [[iron]], [[folic acid]], [[calcium]] and [[magnesium]] is needed. Following [[antibiotic]] therapy, [[immune reconstitution inflammatory syndrome]] ([[IRIS]]) might occur that requires oral [[corticosteroid]]. Lifelong follow-up is needed to detect [[relapse]].
 
==Medical Therapy==
==Medical Therapy==
*Pharmacologic medical therapy for Whipple's disease includes long-term [[Antibiotic|antibiotics]]. Preferred regimens for initial therapy include [[Ceftriaxone]] or [[Penicillin]] G or [[Meropenem]] if allergic. One year of [[Sulfamethoxazole-Trimethoprim|Trimethoprim-sulfamethoxazole]] is used for maintenance therapy. In case of [[sulfa allergy]], the combination of [[Doxycycline]] and [[Hydroxychloroquine]] is used.<ref name="FeurleJunga2010">{{cite journal|last1=Feurle|first1=Gerhard E.|last2=Junga|first2=Natascha S.|last3=Marth|first3=Thomas|title=Efficacy of Ceftriaxone or Meropenem as Initial Therapies in Whipple's Disease|journal=Gastroenterology|volume=138|issue=2|year=2010|pages=478–486|issn=00165085|doi=10.1053/j.gastro.2009.10.041}}</ref><ref name="pmid9193452">{{cite journal |vauthors=Durand DV, Lecomte C, Cathébras P, Rousset H, Godeau P |title=Whipple disease. Clinical review of 52 cases. The SNFMI Research Group on Whipple Disease. Société Nationale Française de Médecine Interne |journal=Medicine (Baltimore) |volume=76 |issue=3 |pages=170–84 |year=1997 |pmid=9193452 |doi= |url=}}</ref><ref name="SchniderReisinger1997">{{cite journal|last1=Schnider|first1=P. J.|last2=Reisinger|first2=E. C.|last3=Berger|first3=T.|last4=Krejs|first4=G. J.|last5=Auff|first5=E.|title=Treatment guidelines in central nervous system Whipple's disease|journal=Annals of Neurology|volume=41|issue=4|year=1997|pages=561–562|issn=0364-5134|doi=10.1002/ana.410410425}}</ref><ref name="pmid14982759">{{cite journal |vauthors=Boulos A, Rolain JM, Raoult D |title=Antibiotic susceptibility of Tropheryma whipplei in MRC5 cells |journal=Antimicrob. Agents Chemother. |volume=48 |issue=3 |pages=747–52 |year=2004 |pmid=14982759 |pmc=353111 |doi= |url=}}</ref><ref name="pmid7519538">{{cite journal |vauthors=Feurle GE, Marth T |title=An evaluation of antimicrobial treatment for Whipple's Disease. Tetracycline versus trimethoprim-sulfamethoxazole |journal=Dig. Dis. Sci. |volume=39 |issue=8 |pages=1642–8 |year=1994 |pmid=7519538 |doi= |url=}}</ref>


==== Classic Whipple's disease ====
=== Whipple's disease ===
*Initial therapy
*Pharmacologic medical therapy for Whipple's disease includes long-term [[Antibiotic|antibiotics]]. Preferred regimens for initial therapy include [[ceftriaxone]] or [[penicillin G]] or [[meropenem]] if allergic. One year of [[Sulfamethoxazole-Trimethoprim|sulfamethoxazole-trimethoprim]] is used for maintenance therapy. In case of [[sulfa allergy]], the combination of [[doxycycline]] and [[hydroxychloroquine]] is used.<ref name="FeurleJunga2010">{{cite journal|last1=Feurle|first1=Gerhard E.|last2=Junga|first2=Natascha S.|last3=Marth|first3=Thomas|title=Efficacy of Ceftriaxone or Meropenem as Initial Therapies in Whipple's Disease|journal=Gastroenterology|volume=138|issue=2|year=2010|pages=478–486|issn=00165085|doi=10.1053/j.gastro.2009.10.041}}</ref><ref name="pmid9193452">{{cite journal |vauthors=Durand DV, Lecomte C, Cathébras P, Rousset H, Godeau P |title=Whipple disease. Clinical review of 52 cases. The SNFMI Research Group on Whipple Disease. Société Nationale Française de Médecine Interne |journal=Medicine (Baltimore) |volume=76 |issue=3 |pages=170–84 |year=1997 |pmid=9193452 |doi= |url=}}</ref><ref name="SchniderReisinger1997">{{cite journal|last1=Schnider|first1=P. J.|last2=Reisinger|first2=E. C.|last3=Berger|first3=T.|last4=Krejs|first4=G. J.|last5=Auff|first5=E.|title=Treatment guidelines in central nervous system Whipple's disease|journal=Annals of Neurology|volume=41|issue=4|year=1997|pages=561–562|issn=0364-5134|doi=10.1002/ana.410410425}}</ref><ref name="pmid14982759">{{cite journal |vauthors=Boulos A, Rolain JM, Raoult D |title=Antibiotic susceptibility of Tropheryma whipplei in MRC5 cells |journal=Antimicrob. Agents Chemother. |volume=48 |issue=3 |pages=747–52 |year=2004 |pmid=14982759 |pmc=353111 |doi= |url=}}</ref><ref name="pmid7519538">{{cite journal |vauthors=Feurle GE, Marth T |title=An evaluation of antimicrobial treatment for Whipple's Disease. Tetracycline versus trimethoprim-sulfamethoxazole |journal=Dig. Dis. Sci. |volume=39 |issue=8 |pages=1642–8 |year=1994 |pmid=7519538 |doi= |url=}}</ref><ref name="pmid2581843">{{cite journal |vauthors=Keinath RD, Merrell DE, Vlietstra R, Dobbins WO |title=Antibiotic treatment and relapse in Whipple's disease. Long-term follow-up of 88 patients |journal=Gastroenterology |volume=88 |issue=6 |pages=1867–73 |year=1985 |pmid=2581843 |doi= |url=}}</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="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>
**Preferred regimen (1): [[Ceftriaxone]] 2 g IV qd for 14 days   
* '''1 Classic Whipple's disease'''
**Preferred regimen (2): [[Penicillin]] G 2 million units IV q4h for 14 days
**'''1.1 Initial therapy'''
**Alternative regimen (1): [[Meropenem]] 1 g IV q8h for 14 days   
***1.1.1 Preferred regimen (1): [[Ceftriaxone]] 2 g IV qd for 14 days   
*Maintenance therapy
***1.1.2 Preferred regimen (2): [[Penicillin]] G 2 million units IV q4h for 14 days
**Preferred regimen (1): [[Sulfamethoxazole-Trimethoprim|Trimethoprim-sulfamethoxazole]] one DS tablet PO q12h for 1 year
***1.1.3 Alternative regimen (1): [[Meropenem]] 1 g IV q8h for 14 days   
**Alternative regimen (1): [[Doxycycline]] 100 mg PO q12h <u>'''AND'''</u> [[Hydroxychloroquine]] 200 mg PO q8h for 1 year
**'''1.2 Maintenance therapy'''
***1.2.1 Preferred regimen (1): [[Sulfamethoxazole-Trimethoprim|Sulfamethoxazole-trimethoprim]] one DS tablet (160 mg TMP/800 mg SMX) PO q12h for 1 year
***1.2.2 Alternative regimen (1): [[Doxycycline]] 100 mg PO q12h <u>'''AND'''</u> [[hydroxychloroquine]] 200 mg PO q8h for 1 year
 
* '''2 CNS infection'''
** '''2.1 Initial therapy'''
*** 2.1.1 Preferred regimen (1): [[Ceftriaxone]] 2 g IV qd for 14-28 days
*** 2.1.2 Preferred regimen (2): [[Penicillin]] G 4 million units IV q4h for 14-28 days
***2.1.3 Alternative regimen (1): [[Meropenem]] 1 g IV q8h for 14-28 days 
**'''2.2 Maintenance therapy'''
***2.2.1 Preferred regimen (1): [[Sulfamethoxazole-Trimethoprim|Sulfamethoxazole-trimethoprim]] one DS tablet (160 mg TMP/800 mg SMX) PO q12h for 1 year   
***2.2.2 Alternative regimen (1): [[Doxycycline]] 100 mg PO q12h '''<u>AND</u>''' [[hydroxychloroquine]] 200 mg PO q8h for 1 year
 
* '''3 Endocarditis'''
** '''3.1 Initial therapy'''
*** 3.1.1 Preferred regimen (1): [[Penicillin]] G 2 million units IV q4h for 28 days
****3.1.2 Preferred regimen (2): [[Ceftriaxone]] 2 g IV qd for 28 days
****3.1.3 Alternative regimen (1): [[Meropenem]] 1 g IV q8h for 28 days 
**'''3.2 Maintenance therapy'''
***3.2.1 Preferred regimen (1): [[Sulfamethoxazole-Trimethoprim|Sulfamethoxazole-trimethoprim]] one DS tablet (160 mg TMP/800 mg SMX) PO q12h for 1 year   
***3.2.2 Alternative regimen (1): [[Doxycycline]] 100 mg PO q12h '''<u>AND</u>''' [[hydroxychloroquine]] 200 mg PO q8h for 1 year
 
* '''4 Relapse'''
**'''4.1 Initial therapy'''
***4.1.1 Preferred regimen (1): [[Penicillin G]] 4 million units IV q4h for 28 days 
***4.1.2 Preferred regimen (2):  [[Ceftriaxone]] 2 g IV qd for 28 days
**'''4.2 Maintenance therapy'''
***4.2.1 Preferred regimen (1): [[Doxycycline]] 100 mg PO q12h '''<u>AND</u>''' [[hydroxychloroquine]] 200 mg PO q8h for 1 year  
***4.2.2 Alternative regimen (1): [[Sulfamethoxazole-Trimethoprim|Sulfamethoxazole-trimethoprim]] one DS tablet (160 mg TMP/800 mg SMX) PO q12h for 1 year
 
'''Note (1):''' Dietary supplements including [[vitamins]], [[iron]], [[folic acid]], [[calcium]], and [[magnesium]] is needed.<ref name="urlwww.cghjournal.org">{{cite web |url=http://www.cghjournal.org/article/S1542-3565(04)00387-8/pdf |title=www.cghjournal.org |format= |work= |accessdate=}}</ref>
 
'''Note (2):''' [[Interferon-gamma|Interferon gamma]] is used in refractory cases.<ref name="Schneider1998">{{cite journal|last1=Schneider|first1=Thomas|title=Treatment of Refractory Whipple Disease with Interferon-γ|journal=Annals of Internal Medicine|volume=129|issue=11_Part_1|year=1998|pages=875|issn=0003-4819|doi=10.7326/0003-4819-129-11_Part_1-199812010-00006}}</ref>
 
'''Note (3):''' Lifelong clinical followup is recommended.<ref name="MarthRaoult2003">{{cite journal|last1=Marth|first1=Thomas|last2=Raoult|first2=Didier|title=Whipple's disease|journal=The Lancet|volume=361|issue=9353|year=2003|pages=239–246|issn=01406736|doi=10.1016/S0140-6736(03)12274-X}}</ref>
 
===Adverse effects of treatment and complications===
* [[Immune reconstitution inflammatory syndrome]] ([[IRIS]]) is a side effect that occurred following initiation of [[antibiotic]] therapy in Whipple's disease. It is characterized as a flare-up of [[inflammation]] and considered fatal.<ref name="BiagiTrotta2012">{{cite journal|last1=Biagi|first1=Federico|last2=Trotta|first2=Lucia|last3=Di Stefano|first3=Michele|last4=Balduzzi|first4=Davide|last5=Marchese|first5=Alessandra|last6=Vattiato|first6=Claudia|last7=Bianchi|first7=Paola I.|last8=Fenollar|first8=Florence|last9=Corazza|first9=Gino R.|title=Previous immunosuppressive therapy is a risk factor for immune reconstitution inflammatory syndrome in Whipple's disease|journal=Digestive and Liver Disease|volume=44|issue=10|year=2012|pages=880–882|issn=15908658|doi=10.1016/j.dld.2012.05.008}}</ref><ref name="MoosFeurle2013">{{cite journal|last1=Moos|first1=V.|last2=Feurle|first2=G. E.|last3=Schinnerling|first3=K.|last4=Geelhaar|first4=A.|last5=Friebel|first5=J.|last6=Allers|first6=K.|last7=Moter|first7=A.|last8=Kikhney|first8=J.|last9=Loddenkemper|first9=C.|last10=Kuhl|first10=A. A.|last11=Erben|first11=U.|last12=Fenollar|first12=F.|last13=Raoult|first13=D.|last14=Schneider|first14=T.|title=Immunopathology of Immune Reconstitution Inflammatory Syndrome in Whipple's Disease|journal=The Journal of Immunology|volume=190|issue=5|year=2013|pages=2354–2361|issn=0022-1767|doi=10.4049/jimmunol.1202171}}</ref>
** Previous [[immunosuppressive therapy]] increases the risk of [[IRIS]].
** Clinical features of the [[IRIS]] including:
*** [[Fever]] (common)
*** [[Arthritis]] (common)
*** [[Erythema nodosum]]
*** [[Meningitis]]
*** [[Brain abscess]]
*** [[Pleuritis]]
*** [[Endocarditis]]
*** Orbitopathy
**Treatment for [[IRIS]] includes:<ref name="LagierFenollar2010">{{cite journal|last1=Lagier|first1=Jean-Christophe|last2=Fenollar|first2=Florence|last3=Lepidi|first3=Hubert|last4=Liozon|first4=Eric|last5=Raoult|first5=Didier|title=Successful treatment of immune reconstitution inflammatory syndrome in Whipple's disease using thalidomide|journal=Journal of Infection|volume=60|issue=1|year=2010|pages=79–82|issn=01634453|doi=10.1016/j.jinf.2009.09.017}}</ref><ref name="MoosFeurle2013">{{cite journal|last1=Moos|first1=V.|last2=Feurle|first2=G. E.|last3=Schinnerling|first3=K.|last4=Geelhaar|first4=A.|last5=Friebel|first5=J.|last6=Allers|first6=K.|last7=Moter|first7=A.|last8=Kikhney|first8=J.|last9=Loddenkemper|first9=C.|last10=Kuhl|first10=A. A.|last11=Erben|first11=U.|last12=Fenollar|first12=F.|last13=Raoult|first13=D.|last14=Schneider|first14=T.|title=Immunopathology of Immune Reconstitution Inflammatory Syndrome in Whipple's Disease|journal=The Journal of Immunology|volume=190|issue=5|year=2013|pages=2354–2361|issn=0022-1767|doi=10.4049/jimmunol.1202171}}</ref>
*** Oral [[corticosteroid]]
*** [[Thalidomide]]
<br>
{| class="wikitable"
! rowspan="2" style="text-align: center; font-weight: bold;" | Indication
! colspan="2" style="text-align: center; font-weight: bold;" | Initial therapy
! colspan="2" style="text-align: center; font-weight: bold;" | Maintenance therapy
|-
| style="text-align: center; font-weight: bold;" | Prefered
| style="text-align: center; font-weight: bold;" | Alternative
| style="text-align: center; font-weight: bold;" | Preferred
| style="text-align: center; font-weight: bold;" | Alternative
|-
| style="font-weight: bold;" | Classic Whipple's disease
|[[Ceftriaxone]] 2 g IV qd for 14 days
'''<u>OR</u>'''
 
[[penicillin G]] 2 million units IV q4h for 14 days
|[[Meropenem]] 1 g IV q8h for 14 days
| [[Sulfamethoxazole-Trimethoprim|Sulfamethoxazole-trimethoprim]] one DS tablet (160 mg TMP/800 mg SMX) PO q12h for 1 year
|[[Doxycycline]] 100 mg PO q12h <u>'''AND'''</u> [[hydroxychloroquine]] 200 mg PO q8h for 1 year
|-
| style="font-weight: bold;" | CNS Whippl'es disease
| [[Ceftriaxone]] 2 g IV qd for 14-28 days
 
'''<u>OR</u>'''


==== CNS infection ====
[[penicillin G]] 4 million units IV q4h for 14-28 days
*Initial therapy
| [[Meropenem]] 1 g IV q8h for 14-28 days
**Preferred regimen (1): [[Ceftriaxone]] 2 g IV qd for 14-28 days 
| [[Sulfamethoxazole-Trimethoprim|Sulfamethoxazole-trimethoprim]] one DS tablet (160 mg TMP/800 mg SMX) PO q12h for 1 year
**Preferred regimen (2): [[Penicillin]] G 4 million units IV q4h for 14-28 days
|[[Doxycycline]] 100 mg PO q12h '''<u>AND</u>''' [[hydroxychloroquine]] 200 mg PO q8h for 1 year
**Alternative regimen (1): [[Meropenem]] 1 g IV q8h for 14-28 days
|-
*Maintenance therapy
| style="font-weight: bold;" | Endocarditis
**Preferred regimen (1): [[Sulfamethoxazole-Trimethoprim|Trimethoprim-sulfamethoxazole]] one DS tablet PO q12h for 1 year  
| [[Penicillin]] G 2 million units IV q4h for 28 days
**Alternative regimen (1): [[Doxycycline]] 100 mg PO q12h '''<u>AND</u>''' [[Hydroxychloroquine]] 200 mg PO q8h for 1 year


==== Endocarditis ====
'''<u>OR</u>'''
*Initial therapy
**Preferred regimen (1): [[Penicillin]] G 2 million units IV q4h for 28 days 
**Preferred regimen (2): [[Ceftriaxone]] 2 g IV qd for 28 days
**Alternative regimen (1): [[Meropenem]] 1 g IV q8h for 28 days 
*Maintenance therapy
**Preferred regimen (1): [[Sulfamethoxazole-Trimethoprim|Trimethoprim-sulfamethoxazole]] one DS tablet PO q12h for 1 year   
**Alternative regimen (1): [[Doxycycline]] 100 mg PO q12h '''<u>AND</u>''' [[Hydroxychloroquine]] 200 mg PO q8h for 1 year


==== Relapse ====
[[ceftriaxone]] 2 g IV qd for 28 days
*Initial therapy
| [[Meropenem]] 1 g IV q8h for 28 days
**Preferred regimen (1): [[Penicillin]] G 4 million units IV q4h for 28 days  
| [[Sulfamethoxazole-Trimethoprim|Sulfamethoxazole-trimethoprim]] one DS tablet (160 mg TMP/800 mg SMX) PO q12h for 1 year
**Preferred regimen (2):  [[Ceftriaxone]] 2 g IV qd for 28 days
|[[Doxycycline]] 100 mg PO q12h '''<u>AND</u>''' [[hydroxychloroquine]] 200 mg PO q8h for 1 year
*Maintenance therapy
|-
**Preferred regimen (1): [[Doxycycline]] 100 mg PO q12h '''<u>AND</u>''' [[hydroxychloroquine]] 200 mg PO q8h for 1 year  
| style="font-weight: bold;" | Relapse
**Alternative regimen (1): [[Sulfamethoxazole-Trimethoprim|Trimethoprim-sulfamethoxazole]] one DS tablet PO q12h for 1 year
| [[Penicillin G]] 4 million units IV q4h for 28 days
 
'''<u>OR</u>'''
 
[[ceftriaxone]] 2 g IV qd for 28 days
|
| [[Doxycycline]] 100 mg PO q12h '''<u>AND</u>''' [[hydroxychloroquine]] 200 mg PO q8h for 1 year
|[[Sulfamethoxazole-Trimethoprim|Sulfamethoxazole-trimethoprim]] one DS tablet (160 mg TMP/800 mg SMX) PO q12h for 1 year
|}


==References==
==References==
{{reflist|2}}
{{reflist|2}}


 
{{WS}}
{{WS}}
{{WH}}
{{WH}}
[[Category:Medicine]]
[[Category:Gastroenterology]]
[[Category:Infectious disease]]
[[Category:Up-To-Date]]

Latest revision as of 00:44, 30 July 2020

Whipple's disease Microchapters

Home

Patient Information

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Whipple's disease from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Criteria

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

X Ray

CT

MRI

Ultrasound

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

Whipple's disease medical therapy On the Web

Most recent articles

Most cited articles

Review articles

CME Programs

Powerpoint slides

Images

American Roentgen Ray Society Images of Whipple's disease medical therapy

All Images
X-rays
Echo & Ultrasound
CT Images
MRI

Ongoing Trials at Clinical Trials.gov

US National Guidelines Clearinghouse

NICE Guidance

FDA on Whipple's disease medical therapy

CDC on Whipple's disease medical therapy

Whipple's disease medical therapy in the news

Blogs on Whipple's disease medical therapy

Directions to Hospitals Treating Whipple's disease

Risk calculators and risk factors for Whipple's disease medical therapy

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

Overview

Antimicrobial therapy is the mainstay of therapy for Whipple's disease. Intravenous ceftriaxone or penicillin G is indicated in the acute phase of Whipple's therapy. For maintenance therapy, patients are typically treated with sulfamethoxazole-trimethoprim for at least 1 year. Patients who experience either Whipple's disease or allergy to sulfamethoxazole-trimethoprim require a combination of doxycycline and hydroxychloroquine. Dietary supplements including vitamins, iron, folic acid, calcium and magnesium is needed. Following antibiotic therapy, immune reconstitution inflammatory syndrome (IRIS) might occur that requires oral corticosteroid. Lifelong follow-up is needed to detect relapse.

Medical Therapy

Whipple's disease

  • 2 CNS infection
    • 2.1 Initial therapy
      • 2.1.1 Preferred regimen (1): Ceftriaxone 2 g IV qd for 14-28 days
      • 2.1.2 Preferred regimen (2): Penicillin G 4 million units IV q4h for 14-28 days
      • 2.1.3 Alternative regimen (1): Meropenem 1 g IV q8h for 14-28 days
    • 2.2 Maintenance therapy
  • 3 Endocarditis
    • 3.1 Initial therapy
      • 3.1.1 Preferred regimen (1): Penicillin G 2 million units IV q4h for 28 days
        • 3.1.2 Preferred regimen (2): Ceftriaxone 2 g IV qd for 28 days
        • 3.1.3 Alternative regimen (1): Meropenem 1 g IV q8h for 28 days
    • 3.2 Maintenance therapy
  • 4 Relapse
    • 4.1 Initial therapy
      • 4.1.1 Preferred regimen (1): Penicillin G 4 million units IV q4h for 28 days
      • 4.1.2 Preferred regimen (2): Ceftriaxone 2 g IV qd for 28 days
    • 4.2 Maintenance therapy

Note (1): Dietary supplements including vitamins, iron, folic acid, calcium, and magnesium is needed.[9]

Note (2): Interferon gamma is used in refractory cases.[10]

Note (3): Lifelong clinical followup is recommended.[11]

Adverse effects of treatment and complications


Indication Initial therapy Maintenance therapy
Prefered Alternative Preferred Alternative
Classic Whipple's disease Ceftriaxone 2 g IV qd for 14 days

OR

penicillin G 2 million units IV q4h for 14 days

Meropenem 1 g IV q8h for 14 days Sulfamethoxazole-trimethoprim one DS tablet (160 mg TMP/800 mg SMX) PO q12h for 1 year Doxycycline 100 mg PO q12h AND hydroxychloroquine 200 mg PO q8h for 1 year
CNS Whippl'es disease Ceftriaxone 2 g IV qd for 14-28 days

OR

penicillin G 4 million units IV q4h for 14-28 days

Meropenem 1 g IV q8h for 14-28 days Sulfamethoxazole-trimethoprim one DS tablet (160 mg TMP/800 mg SMX) PO q12h for 1 year Doxycycline 100 mg PO q12h AND hydroxychloroquine 200 mg PO q8h for 1 year
Endocarditis Penicillin G 2 million units IV q4h for 28 days

OR

ceftriaxone 2 g IV qd for 28 days

Meropenem 1 g IV q8h for 28 days Sulfamethoxazole-trimethoprim one DS tablet (160 mg TMP/800 mg SMX) PO q12h for 1 year Doxycycline 100 mg PO q12h AND hydroxychloroquine 200 mg PO q8h for 1 year
Relapse Penicillin G 4 million units IV q4h for 28 days

OR

ceftriaxone 2 g IV qd for 28 days

Doxycycline 100 mg PO q12h AND hydroxychloroquine 200 mg PO q8h for 1 year Sulfamethoxazole-trimethoprim one DS tablet (160 mg TMP/800 mg SMX) PO q12h for 1 year

References

  1. Feurle, Gerhard E.; Junga, Natascha S.; Marth, Thomas (2010). "Efficacy of Ceftriaxone or Meropenem as Initial Therapies in Whipple's Disease". Gastroenterology. 138 (2): 478–486. doi:10.1053/j.gastro.2009.10.041. ISSN 0016-5085.
  2. Durand DV, Lecomte C, Cathébras P, Rousset H, Godeau P (1997). "Whipple disease. Clinical review of 52 cases. The SNFMI Research Group on Whipple Disease. Société Nationale Française de Médecine Interne". Medicine (Baltimore). 76 (3): 170–84. PMID 9193452.
  3. Schnider, P. J.; Reisinger, E. C.; Berger, T.; Krejs, G. J.; Auff, E. (1997). "Treatment guidelines in central nervous system Whipple's disease". Annals of Neurology. 41 (4): 561–562. doi:10.1002/ana.410410425. ISSN 0364-5134.
  4. Boulos A, Rolain JM, Raoult D (2004). "Antibiotic susceptibility of Tropheryma whipplei in MRC5 cells". Antimicrob. Agents Chemother. 48 (3): 747–52. PMC 353111. PMID 14982759.
  5. Feurle GE, Marth T (1994). "An evaluation of antimicrobial treatment for Whipple's Disease. Tetracycline versus trimethoprim-sulfamethoxazole". Dig. Dis. Sci. 39 (8): 1642–8. PMID 7519538.
  6. Keinath RD, Merrell DE, Vlietstra R, Dobbins WO (1985). "Antibiotic treatment and relapse in Whipple's disease. Long-term follow-up of 88 patients". Gastroenterology. 88 (6): 1867–73. PMID 2581843.
  7. 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.
  8. 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.
  9. "www.cghjournal.org".
  10. Schneider, Thomas (1998). "Treatment of Refractory Whipple Disease with Interferon-γ". Annals of Internal Medicine. 129 (11_Part_1): 875. doi:10.7326/0003-4819-129-11_Part_1-199812010-00006. ISSN 0003-4819.
  11. Marth, Thomas; Raoult, Didier (2003). "Whipple's disease". The Lancet. 361 (9353): 239–246. doi:10.1016/S0140-6736(03)12274-X. ISSN 0140-6736.
  12. Biagi, Federico; Trotta, Lucia; Di Stefano, Michele; Balduzzi, Davide; Marchese, Alessandra; Vattiato, Claudia; Bianchi, Paola I.; Fenollar, Florence; Corazza, Gino R. (2012). "Previous immunosuppressive therapy is a risk factor for immune reconstitution inflammatory syndrome in Whipple's disease". Digestive and Liver Disease. 44 (10): 880–882. doi:10.1016/j.dld.2012.05.008. ISSN 1590-8658.
  13. 13.0 13.1 Moos, V.; Feurle, G. E.; Schinnerling, K.; Geelhaar, A.; Friebel, J.; Allers, K.; Moter, A.; Kikhney, J.; Loddenkemper, C.; Kuhl, A. A.; Erben, U.; Fenollar, F.; Raoult, D.; Schneider, T. (2013). "Immunopathology of Immune Reconstitution Inflammatory Syndrome in Whipple's Disease". The Journal of Immunology. 190 (5): 2354–2361. doi:10.4049/jimmunol.1202171. ISSN 0022-1767.
  14. Lagier, Jean-Christophe; Fenollar, Florence; Lepidi, Hubert; Liozon, Eric; Raoult, Didier (2010). "Successful treatment of immune reconstitution inflammatory syndrome in Whipple's disease using thalidomide". Journal of Infection. 60 (1): 79–82. doi:10.1016/j.jinf.2009.09.017. ISSN 0163-4453.

Template:WS Template:WH