Whipple's disease
- Overview
- Theory
- Diagnosis
- Management
- Follow up
- Resources
Treatment algorithm
Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer
patients without CNS involvement
initial antibiotic therapy
Standard recommendations are initial treatment with either ceftriaxone or benzylpenicillin.[11]Schneider T, Moos V, Loddenkemper C, et al. Whipple's disease: new aspects of pathogenesis and treatment. Lancet Infect Dis. 2008 Mar;8(3):179-90. http://www.ncbi.nlm.nih.gov/pubmed/18291339?tool=bestpractice.com [77]Boulos A, Rolain JM, Raoult D. Antibiotic susceptibility of Tropheryma whipplei in MRC5 cells. Antimicrob Agents Chemother. 2004 Mar;48(3):747-52. http://aac.asm.org/cgi/content/full/48/3/747 http://www.ncbi.nlm.nih.gov/pubmed/14982759?tool=bestpractice.com [78]Boulos A, Rolain JM, Mallet MN, et al. Molecular evaluation of antibiotic susceptibility of Tropheryma whipplei in axenic medium. J Antimicrob Chemother. 2005 Feb;55(2):178-81. http://jac.oxfordjournals.org/cgi/content/full/55/2/178 http://www.ncbi.nlm.nih.gov/pubmed/15650004?tool=bestpractice.com [79]Feurle GE, Junga NS, Marth T. Efficacy of ceftriaxone or meropenem as initial therapies in Whipple's disease. Gastroenterology. 2010 Feb;138(2):478-86; quiz 11-2. https://linkinghub.elsevier.com/retrieve/pii/S0016-5085(09)01938-6 http://www.ncbi.nlm.nih.gov/pubmed/19879276?tool=bestpractice.com [80]Feurle GE, Moos V, Bläker H, et al. Intravenous ceftriaxone, followed by 12 or three months of oral treatment with trimethoprim-sulfamethoxazole in Whipple's disease. J Infect. 2013 Mar;66(3):263-70. http://www.ncbi.nlm.nih.gov/pubmed/23291038?tool=bestpractice.com
Trimethoprim/sulfamethoxazole in combination with intramuscular streptomycin is recommended as an alternative. It can be used in penicillin-allergic patients.
Oral doxycycline plus hydroxychloroquine can be considered as a second-line option in patients who are allergic to ceftriaxone, benzylpenicillin, or trimethoprim/sulfamethoxazole, or who are unable to tolerate parenteral therapy.
Initial antibiotic therapy is for 14 days.
Primary options
ceftriaxone: 2 g intravenously every 24 hours
OR
procaine benzylpenicillin: 1.2 g intramuscularly every 24 hours
OR
benzylpenicillin sodium: 1.2 g intravenously every 4 hours
OR
trimethoprim/sulfamethoxazole: 160/800 mg orally three times daily
and
streptomycin: 500 mg intramuscularly every 12 hours
Secondary options
doxycycline: 100 mg orally twice daily
and
hydroxychloroquine: 200 mg orally three times daily
maintenance antibiotic therapy
Treatment recommended for ALL patients in selected patient group
Standard maintenance therapy is oral trimethoprim/sulfamethoxazole for 1 year, or until bacterial DNA on duodenal biopsies can no longer be seen.[79]Feurle GE, Junga NS, Marth T. Efficacy of ceftriaxone or meropenem as initial therapies in Whipple's disease. Gastroenterology. 2010 Feb;138(2):478-86; quiz 11-2. https://linkinghub.elsevier.com/retrieve/pii/S0016-5085(09)01938-6 http://www.ncbi.nlm.nih.gov/pubmed/19879276?tool=bestpractice.com
Oral doxycycline plus hydroxychloroquine can be used as alternative maintenance therapy in sulfa-allergic patients.
Primary options
trimethoprim/sulfamethoxazole: 160/800 mg orally twice daily
Secondary options
doxycycline: 100 mg orally twice daily
and
hydroxychloroquine: 200 mg orally three times daily
patients with CNS involvement
initial antibiotic therapy
Standard recommendations are initial treatment with either ceftriaxone or benzylpenicillin.[11]Schneider T, Moos V, Loddenkemper C, et al. Whipple's disease: new aspects of pathogenesis and treatment. Lancet Infect Dis. 2008 Mar;8(3):179-90. http://www.ncbi.nlm.nih.gov/pubmed/18291339?tool=bestpractice.com [77]Boulos A, Rolain JM, Raoult D. Antibiotic susceptibility of Tropheryma whipplei in MRC5 cells. Antimicrob Agents Chemother. 2004 Mar;48(3):747-52. http://aac.asm.org/cgi/content/full/48/3/747 http://www.ncbi.nlm.nih.gov/pubmed/14982759?tool=bestpractice.com [78]Boulos A, Rolain JM, Mallet MN, et al. Molecular evaluation of antibiotic susceptibility of Tropheryma whipplei in axenic medium. J Antimicrob Chemother. 2005 Feb;55(2):178-81. http://jac.oxfordjournals.org/cgi/content/full/55/2/178 http://www.ncbi.nlm.nih.gov/pubmed/15650004?tool=bestpractice.com [79]Feurle GE, Junga NS, Marth T. Efficacy of ceftriaxone or meropenem as initial therapies in Whipple's disease. Gastroenterology. 2010 Feb;138(2):478-86; quiz 11-2. https://linkinghub.elsevier.com/retrieve/pii/S0016-5085(09)01938-6 http://www.ncbi.nlm.nih.gov/pubmed/19879276?tool=bestpractice.com [80]Feurle GE, Moos V, Bläker H, et al. Intravenous ceftriaxone, followed by 12 or three months of oral treatment with trimethoprim-sulfamethoxazole in Whipple's disease. J Infect. 2013 Mar;66(3):263-70. http://www.ncbi.nlm.nih.gov/pubmed/23291038?tool=bestpractice.com
Trimethoprim/sulfamethoxazole in combination with intramuscular streptomycin is recommended as an alternative. It can be used in penicillin-allergic patients.
Oral doxycycline plus hydroxychloroquine can be considered as a second-line option in patients who are allergic to ceftriaxone, benzylpenicillin, or trimethoprim/sulfamethoxazole, or who are unable to tolerate parenteral therapy. However, in patients with CNS involvement, trimethoprim/sulfamethoxazole should be used in addition to the oral doxycycline plus hydroxychloroquine regimen.
Initial antibiotic therapy is for 14 days.
Primary options
ceftriaxone: 2 g intravenously every 24 hours
OR
procaine benzylpenicillin: 1.2 g intramuscularly every 24 hours
OR
benzylpenicillin sodium: 1.2 g intravenously every 4 hours
OR
trimethoprim/sulfamethoxazole: 160/800 mg orally three times daily
and
streptomycin: 500 mg intramuscularly every 12 hours
Secondary options
doxycycline: 100 mg orally twice daily
and
hydroxychloroquine: 200 mg orally three times daily
and
trimethoprim/sulfamethoxazole: 160/800 mg orally three times daily
maintenance antibiotic therapy
Treatment recommended for ALL patients in selected patient group
In patients with CNS involvement, maintenance therapy consists of trimethoprim/sulfamethoxazole for 1 year (or until bacterial DNA on duodenal biopsies can no longer be seen), plus doxycycline and hydroxychloroquine.[79]Feurle GE, Junga NS, Marth T. Efficacy of ceftriaxone or meropenem as initial therapies in Whipple's disease. Gastroenterology. 2010 Feb;138(2):478-86; quiz 11-2. https://linkinghub.elsevier.com/retrieve/pii/S0016-5085(09)01938-6 http://www.ncbi.nlm.nih.gov/pubmed/19879276?tool=bestpractice.com
Primary options
doxycycline: 100 mg orally twice daily
and
hydroxychloroquine: 200 mg orally three times daily
and
trimethoprim/sulfamethoxazole: 160/800 mg orally five times daily until PCR of cerebrospinal fluid turns negative, then 160/800 mg twice daily thereafter until end of treatment
Choose a patient group to see our recommendations
Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer
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