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Author: Raphael J Kiel, MD, Associate Professor of Medicine, Wayne State University School of Medicine; Associate Program Director, Head of Infectious Disease Section, Department of Internal Medicine, Oakwood Hospital

Raphael J Kiel is a member of the following medical societies: American College of Physicians-American Society of Internal Medicine, American Geriatrics Society, American Medical Association, and American Medical Informatics Association

Editors: Kenneth C Earhart, MD, FACP, Deputy Head, Disease Surveillance Program, United States Naval Medical Research Unit #3; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine; Joseph F John Jr, MD, FACP, FIDSA, FSHEA, Professor of Medicine, Molecular Genetics and Microbiology, Medical University of South Carolina; Associate Chief of Staff for Education, Ralph H Johnson Veteran's Administration Medical Center; Eleftherios Mylonakis, MD, Clinical and Research Fellow, Department of Internal Medicine, Division of Infectious Diseases, Massachusetts General Hospital; Burke A Cunha, MD, Professor of Medicine, State University of New York School of Medicine at Stony Brook; Chief, Infectious Disease Division, Winthrop-University Hospital

Author and Editor Disclosure

Synonyms and related keywords: sarcosporidiosis, sarcocystosis, Sarcocystis species, Sarcocystidae, eosinophilic myositis syndrome, oocysts, sporocysts, intracellular protozoan parasites, myositis, enteritis, trypanosomiasis, Trypanosoma cruzi infection

Background

Sarcosporidiosis (Sarcocystis infection) is caused by an intracellular protozoan parasite that predominantly affects animals. It can rarely be found in human skeletal and cardiac muscle when humans are the intermediate or accidental host. Humans can also serve as the definitive host for this parasite after ingesting the cysts in raw or undercooked beef or pork. After invasion of the GI tract, the infective sporozoites replicate and are subsequently eliminated in the stool. These oocysts are ingested by an intermediate host (eg, usually cow or pig) to complete the life cycle.

Pathophysiology

Sarcosporidiosis in humans has 2 distinct forms. In the first form, ingestion of water or food contaminated with sporocysts from the feces of a carnivore (eg, dog, wolf) is followed by sporocyst penetration of the intestinal wall. Proliferation in vascular endothelium and subsequent hematogenous dissemination leads to invasion of skeletal and cardiac muscle. These cysts subsequently disintegrate with accompanying vasculitis and fibrosis of the tissue (myositis).

The second form of sarcosporidiosis in man occurs after ingestion of meat contaminated by infective oocysts. The oocysts undergo sexual reproduction and maturation in the intestinal tract. Infective oocysts are shed into the stool (enteritis). A systemic phase and a subsequent tissue phase do not occur in this form of the infection.

Frequency

United States

Sarcosporidiosis has worldwide distribution. In the United States, more than 60 cases of muscle involvement by Sarcocystis species have been described, mostly in collections of case reports of 5-10 cases.

Since this finding is often incidental, many more undetected cases probably exist. The definitive form of sarcosporidiosis causes a self-limited nonspecific enteritis and is often not suspected clinically.

International

Most cases of human sarcosporidiosis occur in Southeast Asia. The incidence of intestinal Sarcocystis infection in Thai laborers was measured at 23%. A study of autopsy specimens of patients in Southeast Asia showed a prevalence rate of 21% in 100 consecutive patients evaluated. The seroprevalence of sarcosporidiosis in Malaysia was estimated at 19.8%.

Mortality/Morbidity

Although sarcosporidiosis can involve the heart, no deaths specifically related to its myocardial involvement have been documented. Painful muscle swellings, fever, and weakness can occur with skeletal muscle involvement. Sarcosporidiosis has not been described as causing a chronic diarrhea or malabsorptive state.

Race

The condition has no known race predilection, but most described cases have been from Southeast Asia.

Sex

The condition has no known sex predilection.

Age

The condition has no known age predilection; however, because muscle involvement clinically occurs after cyst deterioration, adults are more likely to present with skeletal muscle involvement than are children.



History

Patient history in the myositic form of sarcosporidiosis includes painful muscle swellings accompanied by erythema, muscle tenderness, generalized muscle weakness, and fever. Bronchospasm can also occur. Cardiac involvement is asymptomatic, but sarcosporidiosis has been known to cause second-degree atrioventricular block in sheep.

Within a day after ingestion of contaminated beef or pork, subjects who develop the enteritis form of this infection have diaphoresis, chills, fever, vomiting, and diarrhea.

Physical

  • Fever
  • Muscle swelling (1-3 cm in diameter) with erythema and tenderness occur in the myositic form of sarcosporidiosis.
  • Dehydration and diffuse abdominal tenderness occur in patients who ingest the oocyst.

Causes

The following persons have an increased risk of Sarcocystis infection:

  • People who eat raw beef or raw pork, including raw kibbe.
  • Farmers who raise cattle or pigs
  • People living in poor sanitary conditions who can ingest feces contaminated with sporocysts from cattle or dogs



Cryptosporidiosis
Cysticercosis
Giardiasis
Isosporiasis
Toxoplasmosis

Other Problems to be Considered

Trypanosomiasis (Trypanosoma cruzi infection)



Lab Studies

  • In intestinal Sarcocystis infection, sporulated sporocysts in freshly voided stool can be found by flotation technique. Sporocysts contain 4 sporozoites.
  • A complete blood count (CBC) usually reveals eosinophilia. In cases of myositis, creatine kinase levels may be elevated.
  • Polymerase chain reaction (PCR) techniques can be used to exclude Toxoplasma gondii infection.
  • Indirect immunofluorescent antibody test are genus specific but not widely available.
  • A muscle biopsy is diagnostic of myositis.
  • Toxoplasma and Sarcocystis organisms are periodic acid-Schiff (PAS) positive; trypanosomes are PAS negative.
  • Common species found in raw kibbe include Sacrocystis hominis, Sacrocystis hirsuta, and Sacrocystis cruzi.

Imaging Studies

  • An x-ray of the involved extremity may identify calcified cysts of Taenia solium (ie, cysticercosis)
  • CT scan or MRI of extremities may demonstrate the cysts of sarcosporidiosis, which can grow to a diameter of 5 cm.

Procedures

  • Excisional biopsy in the area of the painful muscle swelling is often performed for diagnostic purposes but is not needed therapeutically.

Histologic Findings

Intact sarcocysts observed in muscle are not associated with inflammation. The sarcocysts are rather large (ie, 11 µm X 350 µm) and septate, and they can have a thick, radially striated wall. Seven distinct histopathologic types are described. The tissue form is PAS positive. After the sarcocysts disintegrate, inflammatory cells can be observed, including lymphocytes and neutrophils and an intense eosinophilic infiltrate surrounding the muscle cyst. Localized vasculitis and fibrosis of the muscle are observed.



Medical Care

No specific antiparasitic agent is indicated, as Sarcocystis infection in humans represents the fully formed terminal stage of the parasite. Corticosteroids can be used to reduce the inflammation associated with muscular involvement with this parasite.

Surgical Care

Excisional biopsy in the area of the painful muscle swelling is often performed for diagnostic purposes but is not needed therapeutically.

Consultations

Consultation with an infectious disease physician and a pathologist skilled in tropical medicine is indicated.

Diet

Patients should avoid eating raw pork or raw beef. Patients should not eat food that has been contaminated by feces or dirt.

Activity

No restrictions



Metronidazole and cotrimoxazole are reportedly being used in eosinophilic myositis, although no specific outcomes have been studied. One case report noted cure of Sarcocystis infection with use of cotrimoxazole. Corticosteroids can be used to reduce inflammation associated with muscular involvement.

Drug Category: Corticosteroids

Treat allergic and inflammatory conditions.

Drug NamePrednisone (Deltasone, Orasone, Meticorten)
DescriptionUsed to treat a variety of diseases, including connective tissue disease and inflammatory and allergic disorders. In addition, it is used for adrenocortical insufficiency, neoplastic diseases, and organ transplantation.
Adult Dose20-40 mg/d PO for 7-10 d
Pediatric Dose15-30 mg/d PO adjusted to body weight for 7-10 d
ContraindicationsDocumented hypersensitivity; viral infection, peptic ulcer disease, hepatic dysfunction, connective tissue infections, and fungal or tubercular skin infections; GI disease
InteractionsCoadministration with estrogens may decrease clearance; concurrent use with digoxin, may cause digitalis toxicity secondary to hypokalemia; phenobarbital, phenytoin, and rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics
PregnancyB - Usually safe but benefits must outweigh the risks.
PrecautionsAbrupt discontinuation of glucocorticoids may cause adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, and infections may occur with glucocorticoid use

Drug Category: Antibiotics

Therapy must be comprehensive and cover all likely pathogens in the context of this clinical setting.

Drug NameSulfamethoxazole (SMZ) and trimethoprim (TMP) (Bactrim, Bactrim DS, Septra, Sept
DescriptionUsed to treat a variety of infectious diseases, including urinary tract infections (not due to pseudomonads), Pneumocystis carinii infection, and staphylococcal infections.
Adult Dose160 mg TMP/800 mg SMZ PO q12h for 10-14 d
Pediatric Dose<2 months: Do not administer
>2 months: 15-20 mg/kg/d (based on TMP) PO tid/qid for 14 d
ContraindicationsDocumented hypersensitivity; megaloblastic anemia due to folate deficiency
InteractionsMay 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 thrombocytopenia purpura in elderly patients; phenytoin levels may increase with coadministration; may potentiate effects of methotrexate in bone marrow suppression; hypoglycemic response to sulfonylureas may increase with coadministration; may increase levels of zidovudine
PregnancyC - Safety for use during pregnancy has not been established.
PrecautionsDiscontinue 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 suppression (if signs occur, administer 5-15 mg/d leucovorin); caution in folate deficiency (eg, patients with chronic alcoholism, elderly patients, those receiving anticonvulsant therapy, or those with malabsorption syndrome); hemolysis may occur in patients with G-6-PD deficiency; 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); administer fluids to prevent crystalluria and stone formation

Drug NameMetronidazole (Flagyl)
DescriptionImidazole ring-based antibiotic active against various anaerobic bacteria and protozoa. Used in combination with other antimicrobial agents (except for Clostridium difficile enterocolitis).
Adult DoseLoading dose: 15 mg/kg IV or 1 g for 70-kg adult IV over 1 h
Maintenance dose (6 h following loading dose): 7.5 mg/kg or 500 mg for 70-kg adult IV infusion over 1 h q6-8h; not to exceed 4 g/d
Pediatric DoseAdminister as in adults using body weight
ContraindicationsDocumented hypersensitivity
InteractionsMay increase toxicity of anticoagulants, lithium, and phenytoin; cimetidine may increase toxicity of metronidazole; disulfiram reaction may occur with orally ingested ethanol
PregnancyB - Usually safe but benefits must outweigh the risks.
PrecautionsAdjust dose in hepatic disease; monitor for seizures and development of peripheral neuropathy



Further Inpatient Care

  • Patients are usually treated as outpatients.

Further Outpatient Care

  • Test patients with the enteric form of the infection to document clearance of the sarcocyst from their stool. Shedding can continue for up to 6 months.

In/Out Patient Meds

  • Oral corticosteroids are indicated in the outpatient setting for symptomatic inflammatory muscular involvement.

Deterrence/Prevention

  • Instruct patients not to eat raw beef or pork if the risk of sarcosporidiosis is present in the community.
  • Practice good food hygiene to prevent fecal-oral transmission of this parasite.
  • Freezing meat to -20°C can prevent transmission.

Complications

  • Dehydration
  • Eosinophilic enteritis
  • Ulcerative obstructive enterocolitis

Prognosis

  • Sarcosporidiosis is a self-limited disease with an excellent prognosis. Rarely, eosinophilic myositis symptoms can persist for many years, recurring when further cyst wall deterioration occurs.

Patient Education

  • Sarcosporidiosis is a significant food-borne zoonotic infection and is a risk in travelers to and from Southeast Asia. The patient who sheds infected oocysts in their stool can spread the infection to others through the fecal-oral route if sanitation is poor. Instruct patients not to eat raw beef or pork and to practice good food hygiene. People should understand that this infection does not necessitate routine treatment and usually is an incidental finding discovered on muscle biopsy.



Medical/Legal Pitfalls

  • Failure to recognize the infection: Sarcocystis infection may be mistaken for Toxoplasma infection, since both are PAS positive.
  • Failure to provide the proper treatment: Sarcosporidiosis can be distinguished serologically and by PCR techniques, but diagnostic confusion can lead to mistreatment of Sarcocystis infection with medications designed for Toxoplasma infection.



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Sarcosporidiosis excerpt

Article Last Updated: Feb 15, 2006