Whipple's disease medical therapy

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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. Without antibiotic therapy Whipple's disease is fatal. Intravenous Ceftriaxone or Penicillin G is indicated in the acute phase of Whipple's therapy. For maintenance therapy, patients are typically treated with Trimethoprim-sulfamethoxazole for at least 1 year. Patients who experience either Whipple's disease or allergy to Trimethoprim-sulfamethoxazole require a combination of Doxycycline and Hydroxychloroquine.

Medical Therapy

Whipple's disease

• Pharmacologic medical therapy for Whipple's disease includes long-term antibiotics. Preferred regimens for initial therapy include Ceftriaxone or Penicillin G or Meropenem if allergic. One year of Trimethoprim-sulfamethoxazole is used for maintenance therapy. In case of sulfa allergy, the combination of Doxycycline and Hydroxychloroquine is used.

• 1 Classic Whipple's disease
  • 1.1 Initial therapy
    • 1.1.1 Preferred regimen (1): Ceftriaxone 2 g IV qd for 14 days
    • 1.1.2 Preferred regimen (2): Penicillin G 2 million units IV q4h for 14 days
    • 1.1.3 Alternative regimen (1): Meropenem 1 g IV q8h for 14 days
  • 1.2 Maintenance therapy
    • 1.2.1 Preferred regimen (1): Trimethoprim-sulfamethoxazole 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 AND 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): Trimethoprim-sulfamethoxazole 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 AND Hydroxychloroquine 200 mg PO q8h for 1 year
  • 2.3 Anticonvulsant therapy
    • 2.3.1 Preferred regimen (1):
    • 2.3.2 Preferred regimen (2):

• 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): Trimethoprim-sulfamethoxazole 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 AND 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 AND hydroxychloroquine 200 mg PO q8h for 1 year
    • 4.2.2 Alternative regimen (1): Trimethoprim-sulfamethoxazole one DS tablet (160 mg TMP/800 mg SMX) PO q12h for 1 year

Note: Lifelong clinical followup is recommended.

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.
  • 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
  • Oral corticosteroid is used to treat IRIS.

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	Trimethoprim-sulfamethoxazole 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	Trimethoprim-sulfamethoxazole 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	Trimethoprim-sulfamethoxazole 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	Trimethoprim-sulfamethoxazole one DS tablet (160 mg TMP/800 mg SMX) PO q12h for 1 year

References

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