Schistosomiasis
Trematode infection acquired through freshwater contact in endemic regions; causes hepatic, intestinal, and urogenital disease.
Also known as: schistosomiasis, bilharzia, Schistosoma mansoni, Schistosoma haematobium, Schistosoma japonicum, swimmer's itch
Overview
Chronic parasitic infection caused by blood-dwelling trematodes (flukes) of the genus Schistosoma. Three species cause most human disease: S. mansoni (intestinal/hepatic, Africa, Middle East, South America, Caribbean), S. haematobium (urogenital, Africa, Middle East), and S. japonicum (intestinal/hepatic, Asia). S. mekongi and S. intercalatum are regional species.
Epidemiology
Affects more than 200 million people globally; among the most prevalent parasitic diseases worldwide. Transmission requires freshwater contact in endemic regions where intermediate snail hosts are present. Sub-Saharan Africa carries the largest burden. Travelers, refugees, and immigrants are common patients in non-endemic countries.
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Risk factors
- Freshwater contact (swimming, bathing, wading, fishing, occupational exposure) in endemic regions
- Residence in or travel to sub-Saharan Africa, Egypt, Brazil, parts of Asia and the Middle East
- Lack of safe water and sanitation
- Refugee or migrant populations from endemic regions
- Age 10-20 years (peak prevalence)
Pathophysiology
Cercariae released from infected freshwater snails penetrate intact human skin → migrate to lungs and liver → mature into adult worm pairs in mesenteric venules (S. mansoni, japonicum) or vesical venules (S. haematobium). Adult females release eggs that traverse tissue into the bowel or bladder; eggs trapped in tissues drive granulomatous inflammation and fibrosis. Chronic disease reflects egg-induced tissue damage rather than the worms themselves.
Clinical presentation
Symptoms
- Cercarial dermatitis (swimmer's itch) — pruritic papular rash at penetration site within hours to days
- Katayama fever (acute schistosomiasis) 4-8 weeks after exposure: fever, urticaria, eosinophilia, hepatosplenomegaly, cough — coincides with egg laying
- Intestinal: chronic abdominal pain, intermittent diarrhea, blood in stool
- Hepatosplenic: hepatosplenomegaly with periportal fibrosis ('pipestem' or Symmers fibrosis), variceal bleeding, ascites
- Urogenital (S. haematobium): terminal hematuria, dysuria, hematospermia, infertility, bladder polyps and ulcers; long-term: squamous cell carcinoma of the bladder
- Female genital schistosomiasis: pelvic pain, contact bleeding, infertility, increased HIV acquisition risk
- Neuroschistosomiasis: transverse myelitis (S. mansoni/haematobium spinal cord involvement), seizures (S. japonicum cerebral)
Signs / physical exam
- Hepatosplenomegaly, ascites in advanced hepatic disease
- Stigmata of portal hypertension WITHOUT cirrhosis (preserved synthetic function early)
- Bladder tenderness, perineal mass
- Spinal cord findings (weakness, sensory level, bladder dysfunction) in spinal schistosomiasis
- Eosinophilia
Classic findings
Returned traveler or immigrant from sub-Saharan Africa with hematuria (S. haematobium) or hepatosplenomegaly and esophageal varices despite normal synthetic function (S. mansoni). Eosinophilia and characteristic eggs on urine or stool microscopy with the species-specific spine: lateral spine = S. mansoni, terminal spine = S. haematobium, no spine or tiny lateral knob = S. japonicum.
Differential diagnosis
- Acute schistosomiasis (Katayama fever) versus other acute febrile illness — Travel from endemic region 4-8 weeks earlier, eosinophilia, urticaria; serology
- Cirrhosis of other etiology — Hepatitis B/C, alcohol; periportal fibrosis on imaging suggests Schistosoma
- Bladder cancer (other causes) — S. haematobium predisposes to squamous cell carcinoma of the bladder; cystoscopy and biopsy
- Urinary tract infection / hematuria from other causes — Stones, malignancy, glomerular disease
- Hepatosplenic disease from malaria, leishmaniasis, hepatitis — Geography overlap; smear, serology
- Pulmonary hypertension from other causes — Echo, right heart catheterization; schistosomiasis is a leading cause of PAH worldwide
Diagnostic workup
Diagnostic criteria
Identification of characteristic eggs in stool, urine, or biopsy is definitive. Serology supports diagnosis when eggs not recovered, especially in low-burden travelers and migrants.
Labs
- Stool ovum and parasite (S. mansoni, japonicum)
- Urine microscopy for eggs (S. haematobium) — best at midday, collected after exercise
- Schistosoma serology by ELISA — sensitive for screening in low-burden travelers
- Schistosoma PCR (where available)
- CBC: eosinophilia (acute phase prominent, chronic less so)
- LFTs, BMP
- Rectal or bladder biopsy if eggs not recovered but suspicion high
- CSF examination if neurologic symptoms
Imaging
- Abdominal ultrasound: periportal fibrosis ('pipestem'), portal hypertension, splenomegaly, bladder wall thickening, hydronephrosis
- CT/MRI to characterize hepatic, splenic, urinary findings
- Cystoscopy for bladder polyps, ulcers, malignancy
- Echo for pulmonary hypertension
- MRI spine in suspected neuroschistosomiasis
Treatment
First-line
- Praziquantel 40 mg/kg PO single dose (S. mansoni, S. haematobium) or 60 mg/kg PO divided over one day (S. japonicum, S. mekongi)
- Repeat dose in 4-6 weeks may improve cure rates
- For acute schistosomiasis (Katayama fever): supportive care with corticosteroids to dampen inflammatory response; delay praziquantel until 6-12 weeks post-exposure to allow eggs to mature (praziquantel kills only adult worms, not migrating schistosomula) — exact timing varies and some centers treat earlier with steroid coverage
- Neuroschistosomiasis: praziquantel PLUS corticosteroids to reduce inflammation
- Mass drug administration in endemic areas under WHO programs
Second-line / adjunct
- Oxamniquine for S. mansoni in regions with availability (largely supplanted by praziquantel)
- Surveillance for bladder cancer in chronic S. haematobium
- Hepatic decompensation: management of portal hypertension including band ligation, beta-blockers; transplant in select cases
- HIV co-management is important; female genital schistosomiasis facilitates HIV transmission
Complications
- Hepatic fibrosis with portal hypertension, variceal bleeding
- Squamous cell carcinoma of the bladder (chronic S. haematobium)
- Hydronephrosis, chronic kidney disease
- Pulmonary arterial hypertension (a globally important cause)
- Cor pulmonale
- Neuroschistosomiasis with permanent paraplegia or seizures
- Female genital schistosomiasis with infertility, increased HIV risk
PANCE pearls
- S. haematobium causes hematuria and predisposes to SQUAMOUS cell carcinoma of the bladder, in contrast to transitional cell carcinoma in other settings.
- Egg spine identifies the species: lateral spine = mansoni, terminal spine = haematobium.
- Praziquantel acts only on adult worms — early treatment of Katayama fever may worsen symptoms; consider corticosteroids and delayed retreatment.
- Symmers 'pipestem' periportal fibrosis with portal hypertension occurs without true cirrhosis — synthetic function may be preserved.
- Schistosomiasis is one of the leading global causes of pulmonary arterial hypertension.
References
- WHO 2022 — WHO guideline on control and elimination of human schistosomiasis (2022)
- CDC — CDC Parasites — Schistosomiasis: epidemiology, diagnosis, and treatment
- ASTMH — American Society of Tropical Medicine and Hygiene resources on schistosomiasis management
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