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      Drugs & Diseases > Gastroenterology

      Whipple Disease Medication

      Updated: Oct 24, 2019
      • Author: Ingram M Roberts, MD, MBA; Chief Editor: Burt Cagir, MD, FACS  more...
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      Medication

      Medication Summary

      The goals of pharmacotherapy are to reduce morbidity, to prevent complications, and to eradicate the infection.

      The standard initial treament regimen is either (1) intravenous (IV) ceftriaxone 2 g once daily OR (2) 2 million units of penicillin G IV every 4 hours, both for 14 days' duration. After the initial treatment is completed, 1 year of maintenance therapy is with oral double-strength trimethoprim-sulfamethoxazole twice daily. In a recent case report, patients' gastrointestinal symptoms improved with prolonged antibiotic therapy, but macrophage infiltration of duodenal mucosa persisted years after the therapy. [41]

      As seen in other infectious disease with antibiotic or antiviral therapies, immune reconstitution inflammatory syndrome may occur with Whipple Disease during treatment and may be confused with relapse of the disease. Polymerase chain reaction (PCR) testing is useful in these cases to rule out disease relapse. True relapse (as documented by positive PCR testing) may respond to 2 g ceftriaxone IV twice daily OR 4 million U penicillin G IV every 4 hours, both for 4 weeks' duration, followed by 1 year of oral double strength trimethoprim-sulfamethoxazole twice daily.

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      Antibiotics

      Class Summary

      Antibiotics are the mainstay of treatment. Because of the tendency of Whipple disease to relapse on short courses of antibiotics (2 wk to several mo), most authorities suggest a prolonged course (as long as 1 y). Preliminary data suggest that the PCR test for T whipplei is the best way of detecting remission because patients with a clinical relapse have shown histologic improvement but a persistence of T whipplei through PCR. If the PCR test results become negative after therapy, this suggests a true clinical remission and, possibly, cure. However, PCR has been available for only a few years, so data on the long-term clinical course of patients with Whipple disease as followed using PCR remain sparse.

      Patients who have a relapse usually are treated for another 1-2 years and should receive one of the 14-day parenteral regimens listed below.

      Trimethoprim-sulfamethoxazole (Bactrim, Septra)

      Inhibits bacterial growth by inhibiting synthesis of dihydrofolic acid. Lowest incidence of relapse.

      Penicillin G (Pfizerpen)

      Alternative therapy to that of TMP/SMZ, but should be followed by TMP/SMZ for 1 year. Interferes with synthesis of cell wall mucopeptide during active multiplication, resulting in bactericidal activity against susceptible microorganisms.

      Streptomycin

      Alternative to TMP/SMZ therapy but should be followed by TMP/SMZ for 1 year.

      Penicillin VK (Beepen-VK, Betapen-VK, Robicillin VK, Veetids)

      Use in patients who are sulfa allergic. Penicillins inhibit the biosynthesis of cell wall mucopeptide. They are bactericidal against sensitive organisms when adequate concentrations are reached, and they are most effective during the stage of active multiplication. Inadequate concentrations may produce only bacteriostatic effects.

      Amoxicillin (Trimox, Amoxil, Biomox)

      Use in patients who are sulfa allergic. Interferes with synthesis of cell wall mucopeptides during active multiplication, resulting in bactericidal activity against susceptible bacteria.

      Chloramphenicol (Chloromycetin)

      Binds to 50 S bacterial ribosomal subunits and inhibits bacterial growth by inhibiting protein synthesis. Effective against gram-negative and gram-positive bacteria. Alternative to TMP/SMZ therapy but should be followed by TMP/SMZ for 1 year.

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