1. Strongyloides Morning Report Dec 14 th, 2009 Nicole Cullen

2. What is Strongyloides? Parasitic infection –with a predilection for the intestines 2 most common and clinically relevant species are: –Strongyloides stercoralis –Strongyloides fuelleborni Limited to Africa and Papua New Guinea

3. Epidemiology Relatively uncommon in the US BUT, endemic areas in the rural parts of the Southeastern states and the Appalachian mountain area –Certain pockets with prevalence 4% Usually found in tropical and subtropical countries –Prevalence up to 40% in areas of West Africa, the Caribbean, Southeast Asia Affects >100 million worldwide No sexual or racial disparities. All age groups.

4. How Do You Get It? Penetration of intact skin by filiariform larvae in the soil, or ingestion through contaminated food or water Larvae enter the circulation –Lungs  alveoli  ascension up tracheobronchial tree  swallowed  molt in the small bowel and mature into adult female Females enter the intestinal mucosa and produce several eggs daily through parthenogenesis (hatch during transit through the gut)

6. Clinical Presentation Acute infection: –Lower extremity itching (mild erythematous maculopapular rash at the site of skin penetration) –Cough, dyspnea, wheezing –Low-grade fevers –Epigastric discomfort, n/v/d

7. Clinical Presentation Chronic Infection –Can be completely asymptomatic –Abdominal pain that can be very vague, crampy, burning Often worse after eating –Intermittent diarrhea Can alternate with constipation –Occasional n/v –Weight loss (if heavy infestation) –Larva currens (“racing larva” – a recurrent maculopapular or serpiginous rash) Usually begins perianally and extends up the buttocks, upper thighs, abdomen –Chronic urticaria

8. Larva Currens

9. Clinical Presentation Severe infection –Can be abrupt or insidious in onset –N/v/d, severe abdominal pain, distention –Cough, hemoptysis, dyspnea, wheezing, crackles –Stiff neck, headache, MS changes If CNS involved –Fever/chills –Hematemesis, hematochezia –Rash (petechiae, purpura) over the trunk and proximal extremities Caused by dermal blood vessel disruption brought on by massive migration of larvae within the skin Risk factors for severe infection –Immunosuppressant meds (steroids, chemo, TNF modulators, tacro, etc – all BUT cyclosporine) –Malignancy –Malabsorptive state –ESRD –DM –Advanced age –HIV –HTLV1 –Etoh

10. Clinical Presentation Can replicate in the host for decades with minimal or no sx High morbidity and mortality when progresses to hyperinfection syndrome or disseminated strongyloidiasis –Usually in immunocompromised hosts (pregnancy?)

11. Dangerous Complications Hyperinfection Syndrome –Acceleration of the normal life cycle, causing excessive worm burden –Autoinfection (turn into infective filariform larva within the lumen –Spread of larvae outside the usual migration pattern of GI tract and lungs Disseminated strongyloidiasis –Widespread dissemination of larvae to extraintestinal organs CNS (meningitis), heart, urinary tract, bacteremia, etc –Can be complicated by translocation of enteric bacteria Travel on the larvae themselves or via intestinal ulcers –Mortality rate close to 80% Due to delayed diagnosis, immunocompromised state of the host at this point

12. Laboratory Findings CBC –WBC usually wnl for acute and chronic cases, can be elevated in severe cases –Eosinophilia common during acute infection, +/- in chronic infection (75%), usually absent in severe infection

13. Diagnostic Testing Stool O&P –Microscopic ID of S. sterocoralis larvae is the definitive diagnosis –Ova usually not seen (only helminth to secrete larva in the feces) Stool wet mount (direct exam) –In chronic infection, sensitivity only 30%, can increase to 75% if 3 consecutive stool exams –Can enhance larvae recovery with more obscure methods (Baermann funnel, agar plate, Harada-Mori filter paper)

14. Wet Mount Larva seen via direct examination of stool

15. Serology ELISA Most sensitive method (88-95%) –May be lower in immunocompromised patients Cannot distinguish between past and present infections Can cross-react with other nematode infections If results are positive, can move on to try and establish a microscopic dx

16. Imaging CXR – patchy alveolar infiltrates, diffuse interstitial infiltrates, pleural effusions AXR – Loops of dilated small bowel, ileus Barium swallow – stenosis, ulceration, bowel dilitation Small bowel follow-through – worms in the instestine CT abdomen/pelvis – nonspecific thickening of the bowel wall

17. Procedures EGD – duodenitis, edematous mucosa, white villi, erythema Colonoscopy – colitis Duodenal aspiration – examine for larvae Sputum sample, bronchial washings, BAL – show larvae Sputum cx –Nl respiratory flora organisms pushed to the outside in groups as a result of migrating larvae –Characteristic pattern can be diagnostic of S.Stercoralis infection If CNS involved, LP – gram stain, cell count/diff (  protein, ↓ glu, poly predominance), wet mount prep

18. Histology Larvae typically found in proximal portion of small intestine –Embedded in lamina propria Cause edema, cellular infiltration, villous atrophy, ulcerations In-long standing infections, may see fibrosis

19. Treatment Antihelminitic therapy –Ivermectin –Albendazole –Thiabendazole Abx directed toward enteric pathogens if bacteremia or meningitis (2-4wks) Minimize immunosuppression as possible Directed supportive tx –Transfusions if GI bleed, antihistamines for itching, surgery if bowel perf, etc Repeat course of antihelminitic therapy if immunocompromised, as relapse common

20. Follow-Up Repeat stool exams or duodenal aspirations in 2-3 mos to document cure Repeat serologies 4-8 mos after therapy –Ab titer should be low or undetectable 6-18 mos after successful tx If titer not falling, additional antihelminitic tx Precautions for travelers to endemic areas, but no prophylaxis or vaccine available

21. References Arch EL, Schaefer JT and Dahiya A. Cutaneous manifestation of disseminated strongyloidiasis in a patient coinfected with HTLV-1. Dermatology Online Journal. 2008;14(12):6. Chadrasekar PH, Bharadwaj RA, Polenakovik H, Polenakovik S. Emedicine: Strongyloidiasis. April 3, 2009. Concha R, Harrington W and Rogers A. Intestinal Strongyloidiasis. Recognition, Management and Determinants of Outcome. Journal of Clinical Gastroengerology. 2005;39(3):203-211. Greiner K, Bettencourt J, and Semolic C. Strongyloidiasis: A Review and Update by Case Example. Clinical Laboratory Science. 2008;21(2):82-8. Siddiqui AA, Berk SL. Diagnosis of Strongyloides stercoralis infection. Clin Infect Dis. October 1, 2001;33:1040-7. Zeph, Bill. Strongyloides stercoralis Infection Can Be Fatal. American Family Physician. March 15, 2002.