eMedicine Specialties > Gastroenterology > Intestine
Whipple Disease: Treatment & Medication
Updated: Aug 14, 2008
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
Treatment
Medical Care
The mainstay of medical treatment is antibiotic therapy.
Surgical Care
Surgery is not part of the therapy for Whipple disease.
Consultations
Consultations with a gastroenterologist, cardiologist, rheumatologist, orthopedist, and neurosurgeon (who will ask for a small biopsy, especially when there are no GI symptoms) may be necessary for obtaining the appropriate tissue biopsy in selected patients.
Diet
No dietary changes are usually required.
Activity
No activity restrictions are usually required.
Medication
The goals of pharmacotherapy are to reduce morbidity, to prevent complications, and to eradicate the infection.
Antibiotics
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 whippelii is the best way of detecting remission because patients with a clinical relapse have shown histologic improvement but a persistence of T whippelii 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.
Adult
160 mg TMP/800 mg SMZ PO bid for 1-2 y, with folate supplementation
Pediatric
Not established
May increase PT when used with warfarin (perform coagulation tests and adjust dose accordingly); coadministration with dapsone may increase blood levels of both drugs; coadministration of diuretics increases incidence of thrombocytopenic purpura in elderly persons; phenytoin levels may increase with coadministration; may potentiate effects of methotrexate in bone marrow depression; hypoglycemic response to sulfonylureas may increase with coadministration; may increase levels of zidovudine
Documented hypersensitivity; megaloblastic anemia due to folate deficiency
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Discontinue at first appearance of skin rash or sign of adverse reaction; obtain CBCs frequently; discontinue therapy if significant hematologic changes occur; goiter, diuresis, and hypoglycemia may occur with sulfonamides; prolonged IV infusions or high doses may cause bone marrow depression (if signs occur, give 5-15 mg/d leucovorin); caution in folate deficiency (eg, long-term alcoholics, elderly persons, those receiving anticonvulsant therapy, those with malabsorption syndrome); hemolysis may occur in individuals who are G-6-PD deficient; patients with AIDS may not tolerate or respond to TMP-SMZ; caution in renal or hepatic impairment (perform urinalyses and renal function tests during therapy); give fluids to prevent crystalluria and stone formation
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.
Adult
1.2 million U IM qd for 14 d
Pediatric
Not established
Probenecid can increase effects of penicillin; coadministration of tetracyclines can decrease effects of penicillin
Documented hypersensitivity
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Caution in impaired renal function
Streptomycin
Alternative to TMP/SMZ therapy but should be followed by TMP/SMZ for 1 year.
Adult
1 g IM for 14 d
Pediatric
Not established
Nephrotoxicity may be increased with aminoglycosides, cephalosporins, penicillins, amphotericin B, and loop diuretics
Documented hypersensitivity; non–dialysis-dependent renal insufficiency
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Narrow therapeutic index; not intended for long-term therapy; caution in patients on renal failure not on dialysis; caution with myasthenia gravis, hypocalcemia, and conditions that depress neuromuscular transmission
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.
Adult
250 mg PO qid
Pediatric
Not established
Probenecid may increase effectiveness by decreasing clearance; tetracyclines are bacteriostatic, causing a decrease in the effectiveness of penicillins when administered concurrently
Documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Caution in renal impairment
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.
Adult
250 mg PO tid
Pediatric
Not established
Reduces the efficacy of oral contraceptives
Documented hypersensitivity
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Adjust dose in renal impairment
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.
Adult
1 g IV qid for 14 d
Pediatric
Not established
Concurrently with barbiturates, chloramphenicol serum levels may decrease, while barbiturate levels may increase, causing toxicity; manifestations of hypoglycemia may occur with sulfonylureas; rifampin may reduce serum chloramphenicol levels, presumably through hepatic enzyme induction; may increase effects of anticoagulants; may increase serum hydantoin levels, possibly resulting in toxicity; chloramphenicol levels may be increased or decreased
Documented hypersensitivity; not recommended for long-term use because of bone marrow toxicity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Use only for indicated infections or as prophylaxis for bacterial infections; serious and fatal blood dyscrasias (ie, aplastic anemia, hypoplastic anemia, thrombocytopenia, granulocytopenia) can occur; evaluate baseline and perform periodic blood studies approximately every 2 d while in therapy; discontinue upon appearance of reticulocytopenia, leukopenia, thrombocytopenia, anemia, or findings attributable to chloramphenicol; adjust dose in liver or kidney dysfunction; caution in pregnancy at term or during labor because of potential toxic effects on fetus (gray syndrome)
More on Whipple Disease |
| Overview: Whipple Disease |
| Differential Diagnoses & Workup: Whipple Disease |
 Treatment & Medication: Whipple Disease |
| Follow-up: Whipple Disease |
| References |
| « Previous Page | Next Page » |
References
Relman DA, Schmidt TM, MacDermott RP, et al. Identification of the uncultured bacillus of Whipple's disease. N Engl J Med. Jul 30 1992;327(5):293-301. [Medline].
Raoult D, Birg ML, La Scola B, et al. Cultivation of the bacillus of Whipple's disease. N Engl J Med. Mar 2 2000;342(9):620-5. [Medline].
Dray X, Vahedi K, Delcey V, et al. Mycobacterium avium duodenal infection mimicking Whipple's disease in a patient with AIDS. Endoscopy. Feb 2007;39 Suppl 1:E296-7. [Medline].
Patel SJ, Huard RC, Keller C, et al. Possible Case of CNS Whipple's Disease in an Adolescent With AIDS. J Int Assoc Physicians AIDS Care (Chic Ill). Jun 2008;7(2):69-73. [Medline].
Ramzan NN, Loftus E Jr, Burgart LJ, et al. Diagnosis and monitoring of Whipple disease by polymerase chain reaction. Ann Intern Med. Apr 1 1997;126(7):520-7. [Medline].
Marth T, Schneider T. Whipple disease. Curr Opin Gastroenterol. Mar 2008;24(2):141-8. [Medline].
Schneider T, Moos V, Loddenkemper C, et al. Whipple's disease: new aspects of pathogenesis and treatment. Lancet Infect Dis. Mar 2008;8(3):179-90. [Medline].
O'Duffy JD, Griffing WL, Li CY, et al. Whipple's arthritis: direct detection of Tropheryma whippelii in synovial fluid and tissue. Arthritis Rheum. Apr 1999;42(4):812-7. [Medline].
Celard M, de Gevigney G, Mosnier S, et al. Polymerase chain reaction analysis for diagnosis of Tropheryma whippelii infective endocarditis in two patients with no previous evidence of Whipple's disease. Clin Infect Dis. Nov 1999;29(5):1348-9. [Medline].
Gubler JG, Kuster M, Dutly F, et al. Whipple endocarditis without overt gastrointestinal disease: report of four cases. Ann Intern Med. Jul 20 1999;131(2):112-6. [Medline].
Gerard A, Sarrot-Reynauld F, Liozon E, et al. Neurologic presentation of Whipple disease: report of 12 cases and review of the literature. Medicine (Baltimore). Nov 2002;81(6):443-57. [Medline].
Kelly CA, Egan M, Rawlinson J. Whipple's disease presenting with lung involvement. Thorax. Mar 1996;51(3):343-4. [Medline].
Dobbins WO 3rd. HLA antigens in Whipple's disease. Arthritis Rheum. Jan 1987;30(1):102-5. [Medline].
Fenollar F, Puéchal X, Raoult D. Whipple's disease. N Engl J Med. Jan 4 2007;356(1):55-66. [Medline].
Durand DV, Lecomte C, Cathebras P, et al. Whipple disease. Clinical review of 52 cases. The SNFMI Research Group on Whipple Disease. Société Nationale Française de Médecine Interne. Medicine (Baltimore). May 1997;76(3):170-84. [Medline].
Keinath RD, Merrell DE, Vlietstra R, et al. Antibiotic treatment and relapse in Whipple's disease. Long-term follow-up of 88 patients. Gastroenterology. Jun 1985;88(6):1867-73. [Medline].
Fleming JL, Wiesner RH, Shorter RG. Whipple's disease: clinical, biochemical, and histopathologic features and assessment of treatment in 29 patients. Mayo Clin Proc. Jun 1988;63(6):539-51. [Medline].
Sheib JS. Whipple disease revisited. Radiographic features of a patient with 35 years of undiagnosed arthritis. J Clin Rheumatol. 2004;10:69-73.
Matthews BR, Jones LK, Saad DA, et al. Cerebellar ataxia and central nervous system Whipple disease. Arch Neurol. Apr 2005;62(4):618-20. [Medline].
Süzer T, Demirkan N, Tahta K, et al. Whipple's disease confined to the central nervous system: case report and review of the literature. Scand J Infect Dis. 1999;31(4):411-4. [Medline].
Ehrbar HU, Bauerfeind P, Dutly F, et al. PCR-positive tests for Tropheryma whippelii in patients without Whipple's disease. Lancet. Jun 26 1999;353(9171):2214. [Medline].
Street S, Donoghue HD, Neild GH. Tropheryma whippelii DNA in saliva of healthy people. Lancet. Oct 2 1999;354(9185):1178-9. [Medline].
Swartz MN. Whipple's disease--past, present, and future. N Engl J Med. Mar 2 2000;342(9):648-50. [Medline].
Further Reading
Keywords
Whipple disease, Whipple's disease, Tropheryma whippelii, T whippelii, intestinal lipodystrophy, WD, polyarthralgias, chronic diarrhea, Whipple disease with symptomatic CNS involvement, fever of unknown origin
