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Treatment of microsporidiosis

Treatment of microsporidiosis
  Drug Adult dose Pediatric dose
Ocular (Encephalitozoon hellem, Encephalitozoon cuniculi, Vittaforma [Nosema] corneae)
Drug of choice: Fumagillin (Fumidil B) in saline equivalent to fumagillin 70 mcg/mL eye drops* 2 drops every 2 hours for 4 days, then 2 drops 4 times per day for 14 days  
PLUS for management of systemic infection
Albendazole¶Δ 400 mg orally with fatty meal twice per day 15 mg/kg per day in 2 divided doses (maximum 400 mg/dose)
Intestinal (Enterocytozoon bieneusi, Encephalitozoon [Septata] intestinalis)
E. bieneusi
Drug of choice: Fumagillin 20 mg orally three times per day for 14 days  
E. intestinalis
Drug of choice: Albendazole¶Δ

Immunocompetent: Symptoms may resolve with no therapy in immunocompetent individuals. If the clinical context or symptoms warrant treatment, we use 400 mg orally on empty stomach twice per day for 7 to 14 days§.

Immunocompromised: 400 mg orally on empty stomach twice per day for 21 days.
15 mg/kg per day in 2 divided doses (maximum 400 mg/dose)
Disseminated or extraintestinal (E. hellem, E. cuniculi, E. intestinalis, Pleistophora spp, Trachipleistophora spp, and Anncaliia [Brachiola] vesicularum)
Drug of choice:¥ Albendazole¶Δ

Immunocompetent: 400 mg orally with fatty meal twice per day for 7 to 14 day§. Occasionally, symptoms may resolve with no therapy.

Immunocompromised: 400 mg orally with fatty meal twice per day for 21 (14 to 28) days. Continue treatment until CD4+ count >200 cells/microL for ≥3 months after initiation of potent antiretroviral therapy.
15 mg/kg per day in 2 divided doses (maximum 400 mg/dose)
The mainstay of treatment of microsporidiosis in patients with HIV and severe immunosuppression is antiretroviral therapy for immune restoration of CD4+ counts to greater than 100 cells/microL, which is associated with resolution of symptoms of enteric microsporidiosis, including that caused by E. bieneusi.

HIV: human immunodeficiency virus.

* Available as an investigational agent (non-US Food and Drug Administration [FDA] approved) in the United States from Leiter's Park Avenue Pharmacy (a custom compounding pharmacy), San Jose, CA (800-292-6773, www.leiterrx.com). Ocular lesions due to E. hellem in patients with HIV have responded to fumagillin eyedrops prepared from Fumidil B (bicyclohexyl ammonium fumagillin) used to control a microsporidial disease of honey bees.[1,2] For lesions due to V. corneae, topical therapy is generally not effective and keratoplasty may be required.[3]

¶ Not FDA approved for this indication.

Δ For treatment of systemic infection, albendazole must be taken with food; a fatty meal increases oral bioavailability. For treatment of intestinal infection with no systemic involvement, albendazole should be taken on an empty stomach.

◊ Oral fumagillin (Flisint, Sanofi-Aventis, France) is not available in the United States. It has been effective in treating E. bieneusi in patients with HIV or solid organ transplant[4,5] but has been associated with thrombocytopenia and neutropenia. Initiation of antiretroviral therapy may lead to microbiologic and clinical response in patients with HIV and microsporidial diarrhea without antiparasitic treatment. Octreotide (Sandostatin) has provided symptomatic relief in some patients with large-volume diarrhea.

§ Duration should depend on the clinical improvement of the patient (eg, resolution of symptoms and inflammatory markers). In severe cases, an additional week of therapy may be indicated.

¥ There is no established treatment for Pleistophora.[6] For disseminated disease due to Trachipleistophora or Anncallia, itraconazole 400 mg orally once per day plus albendazole may also be tried.[7]
References:
  1. Chan CM, Theng JT, Li L, Tan DT. Microsporidial keratoconjunctivitis in healthy individuals: a case series. Ophthalmology 2003; 110:1420.
  2. Garvey MJ, Ambrose PG, Ulmer JL. Topical fumagillin in the treatment of microsporidial keratoconjunctivitis in AIDS. Ann Pharmacother 1995; 29:872.
  3. Davis RM, Font RL, Keisler MS, Shadduck JA. Corneal microsporidiosis. A case report including ultrastructural observations. Ophthalmology 1990; 97:953.
  4. Molina JM, Tourneur M, Sarfati C, et al. Fumagillin treatment of intestinal microsporidiosis. N Engl J Med 2002; 346:1963.
  5. Lanternier F, Boutboul D, Menotti J, et al. Microsporidiosis in solid organ transplant recipients: two Enterocytozoon bieneusi cases and review. Transpl Infect Dis 2009; 11:83.
  6. Molina JM, Oksenhendler E, Beauvais B, et al. Disseminated microsporidiosis due to Septata intestinalis in patients with AIDS: clinical features and response to albendazole therapy. J Infect Dis 1995; 171:245.
  7. Coyle CM, Weiss LM, Rhodes LV 3rd, et al. Fatal myositis due to the microsporidian Brachiola algerae, a mosquito pathogen. N Engl J Med 2004; 351:42.

Adapted from: Drugs for Parasitic Infections, 3rd ed, The Medical Letter 2013.

Additional data from:
  • Panel on Guidelines for the Prevention and Treatment of Opportunistic Infections in Adults and Adolescents with HIV. Guidelines for the prevention and treatment of opportunistic infections in adults and adolescents with HIV. National Institutes of Health, Centers for Disease Control and Prevention, HIV Medicine Association, and Infectious Diseases Society of America. Available at: https://clinicalinfo.hiv.gov/en/guidelines/adult-andadolescent-opportunistic-infection (Accessed on April 4, 2024).
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