Infectious Disease · PANCE / PANRE

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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