Approach
Treatment with antibiotics should be started if the diagnosis is confirmed. PCR of CSF should be performed in these patients before antibiotic treatment is started, independent of other symptoms. This is because asymptomatic CNS colonisation is common.
In patients with a tentative diagnosis of Whipple's disease, the recommended antibiotic treatment should not be initiated until a confirmed diagnosis is established or all possible differential diagnoses are excluded.
Initial antibiotic therapy following confirmed diagnosis
Standard treatment recommendations are ceftriaxone or benzylpenicillin for 2 weeks.[11][77][78][79][80]
Trimethoprim/sulfamethoxazole in combination with intramuscular streptomycin is recommended as an alternative to ceftriaxone or benzylpenicillin. It can be used in penicillin-allergic patients.
Initial antibiotic therapy is for 14 days. These antibiotic regimens can be given to patients with and without CNS involvement.
Second-line options
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. One small case series of patients with classic Whipple's disease suggests that the oral doxycycline plus hydroxychloroquine is effective.[81]
Initial antibiotic therapy is for 14 days. In patients with CNS involvement, trimethoprim/sulfamethoxazole should be used in addition to the oral doxycycline plus hydroxychloroquine regimen.
Maintenance therapy
Standard maintenance therapy in patients without CNS involvement is oral trimethoprim/sulfamethoxazole for 1 year, or until bacterial DNA on duodenal biopsies can no longer be seen.[79] Oral doxycycline plus hydroxychloroquine can be used as alternative maintenance therapy in sulfa-allergic patients.
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.
Relapsing disease
With sufficient treatment, relapses have become rare. However, a few patients do not respond adequately to the first antibiotic regimen used. If clinical signs or positive PCR for T whipplei persists, the antibiotic regimen should be changed. However, periodic acid-Schiff (PAS)-positive macrophages will be found for years within the duodenal mucosa and are not a sign of relapse.
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