[Echinococcus granulosus] [Echinococcus multilocularis] [Echinococcus oligarthrus] [Echinococcus vogeli]
Human echinococcosis (hydatidosis, or hydatid disease) is caused by the larval stages of cestodes (tapeworms) of the genus Echinococcus. Echinococcus granulosus causes cystic echinococcosis, the form most frequently encountered; E. multilocularis causes alveolar echinococcosis; E. vogeli causes polycystic echinococcosis; and E. oligarthrus is an extremely rare cause of human echinococcosis.
The adult Echinococcus granulosus (3 to 6 mm long) resides in the small bowel of the definitive hosts, dogs or other canids. Gravid proglottids release eggs that are passed in the feces. After ingestion by a suitable intermediate host (under natural conditions: sheep, goat, swine, cattle, horses, camel), the egg hatches in the small bowel and releases an oncosphere that penetrates the intestinal wall and migrates through the circulatory system into various organs, especially the liver and lungs. In these organs, the oncosphere develops into a cyst that enlarges gradually, producing protoscolices and daughter cysts that fill the cyst interior. The definitive host becomes infected by ingesting the cyst-containing organs of the infected intermediate host. After ingestion, the protoscolices evaginate, attach to the intestinal mucosa , and develop into adult stages in 32 to 80 days. The same life cycle occurs with E. multilocularis (1.2 to 3.7 mm), with the following differences: the definitive hosts are foxes, and to a lesser extent dogs, cats, coyotes and wolves; the intermediate host are small rodents; and larval growth (in the liver) remains indefinitely in the proliferative stage, resulting in invasion of the surrounding tissues. With E. vogeli (up to 5.6 mm long), the definitive hosts are bush dogs and dogs; the intermediate hosts are rodents; and the larval stage (in the liver, lungs and other organs) develops both externally and internally, resulting in multiple vesicles. E. oligarthrus (up to 2.9 mm long) has a life cycle that involves wild felids as definitive hosts and rodents as intermediate hosts. Humans become infected by ingesting eggs , with resulting release of oncospheres in the intestine and the development of cysts , , , , , in various organs.
Echinococcus granulosus occurs practically worldwide, and more frequently in rural, grazing areas where dogs ingest organs from infected animals. Echinococcus multilocularis occurs in the northern hemisphere, including central Europe and the northern parts of Europe, Asia, and North America. Echinococcus vogeli and Echinococcus oligarthrus occur in Central and South America.
Echinococcus granulosus infections remain silent for years before the enlarging cysts cause symptoms in the affected organs. Hepatic involvement can result in abdominal pain, a mass in the hepatic area, and biliary duct obstruction. Pulmonary involvement can produce chest pain, cough, and hemoptysis. Rupture of the cysts can produce fever, urticaria, eosinophilia, and anaphylactic shock, as well as cyst dissemination. In addition to the liver and lungs, other organs (brain, bone, heart) can also be involved, with resulting symptoms. Echinococcus multilocularis affects the liver as a slow growing, destructive tumor, with abdominal pain, biliary obstruction, and occasionally metastatic lesions into the lungs and brain. Echinococcus vogeli affects mainly the liver, where it acts as a slow growing tumor; secondary cystic development is common.
Echinococcus granulosus in tissue.
Echinococcus sp. from a liver cyst, stained with Papanicolaou (PAP) stain.
Echinococcus multilocularis in tissue, stained with hematoxylin and eosin (H&E).
Echinococcus eggs in feces.
Echinococcus spp. adults.
The diagnosis of echinococcosis relies mainly on findings by ultrasonography and/or other imaging techniques supported by positive serologic tests. In seronegative patients with hepatic image findings compatible with echinococcosis, ultrasound guided fine needle biopsy may be useful for confirmation of diagnosis; during such procedures precautions must be taken to control allergic reactions or prevent secondary recurrence in the event of leakage of hydatid fluid or protoscolices.
Immunodiagnostic tests can be very helpful in the diagnosis of echinococcal disease and should be used before invasive methods. However, the clinician must have some knowledge of the characteristics of the available tests and the patient and parasite factors associated with false results. False-positive reactions may occur in persons with other helminthic infections, cancer, and chronic immune disorders. Negative test results do not rule out echinococcosis because some cyst carriers do not have detectable antibodies. Whether the patient has detectable antibodies depends on the physical location, integrity, and vitality of the larval cyst. Cysts in the liver are more likely to elicit antibody response than cysts in the lungs, and, regardless of localization, antibody detection tests are least sensitive in patients with intact hyaline cysts. Cysts in the lungs, brain, and spleen are associated with lowered serodiagnostic reactivity, whereas those in bone appear to more regularly stimulate detectable antibody. Fissuration or rupture of a cyst is followed by an abrupt stimulation of antibodies. A patient with senescent, calcified, or dead cysts is generally found to be seronegative.
Cystic echinococcal disease (Echinococcus granulosus). Indirect hemagglutination (IHA), indirect fluorescent antibody (IFA) tests, and enzyme immunoassays (EIA) are sensitive tests for detecting antibodies in serum of patients with cystic disease; sensitivity rates vary from 60% to 90%, depending on the characteristics of the cases. Crude hydatid cyst fluid is generally employed as antigen. At present, the best available serologic diagnosis is obtained by using combinations of tests. EIA or IHA is used to screen all specimens; a positive reaction is confirmed by immunoblot assay or any gel diffusion assay that demonstrates the echinococcal “Arc 5.” Although these confirmatory assays give false-positive reactions with sera of 5% to 25% of persons with neurocysticercosis, the clinical and epidemiological presentation of neurocysticercosis patients should be rarely confused with that of cystic echinococcosis. A commercial EIA kit is available in the United States.
Antibody responses have also been monitored as a way of evaluating the results of treatment, but with mixed results. Following successful radical surgery, antibody titers decline and sometimes disappear; titers rise again if secondary cysts develop. Tests for Arc 5 or IgE antibodies appear to reflect antibody decline during the first 24 months postsurgery, whereas the IHA and other tests remain positive for at least 4 years. Chemotherapy has not been followed by consistent declines in antibody titers. Consequently, the usefulness of serology to monitor the course of disease is limited; imaging techniques provide a more accurate assessment of the patient’s condition.
Alveolar echinococcal disease (Echinococcus multilocularis). Most patients with alveolar disease have detectable antibodies in serologic tests using heterologous E. granulosus or homologous Echinococcus multilocularis antigens. With crude Echinococcus antigens, nonspecific reactions create the same difficulties as described above, however, immunoaffinity-purified E. multilocularis antigens (Em2) used in EIA allow the detection of positive antibody reactions in more than 95% of alveolar cases. Comparing serologic reactivity to Em2 antigen with that to antigens containing components of both E. multilocularis and E. granulosus permits discrimination of patients with alveolar from those with cystic disease. Combining two purified E. multilocularis antigens (Em2 and recombinant antigen II/3-10) in a single immunoassay optimized sensitivity and specificity. These antigens are sold in a commercial EIA kit in Europe, but not in the United States. As in cystic echinococcosis, Em2 tests are more useful for postoperative follow-up than for monitoring the effectiveness of chemotherapy.
Lightowlers MW, Gottstein B. Echinococcosis/hydatidosis: antigens, immunological and molecular diagnosis. In: Thompson RCA, Lymbery AJ, editors. Echinococcus and hydatid disease. Wallingford, UK: CAB International; 1995. p. 355-410.
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