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Lymphatic Filiriasis:

Lymphatic Filiriasis:. A Case Report John W. Hariadi , M.D. Emory Family Medicine. History. 35 yo BF with 10 year hx of LLE swelling & foot deformity -Swelling first started 10-12 years ago, initially involving entire leg up to her groin, with “hardening” of the skin

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Lymphatic Filiriasis:

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  1. Lymphatic Filiriasis: A Case Report John W. Hariadi, M.D. Emory Family Medicine

  2. History 35 yo BF with 10 year hx of LLE swelling & foot deformity -Swelling first started 10-12 years ago, initially involving entire leg up to her groin, with “hardening” of the skin -Over the years, swelling subsided some except for L foot deformity -She can ambulate, denies pain -Denies F/C, constitutional sxs -No meds or allergies -Lived her entire life in Buenaventura, Colombia -PMH: Malaria in past, o/w negative -SH: Single –”No one wants to marry me because of my foot”, lives with mother & sister’s family. No T/E/D -FH: Unremarkable Elevation: 49FT Latitude: 3 58 N Longitude: 77 18W

  3. Buenaventura, Colombia

  4. Physical • Vital Signs WNL • LLE: Significant edema from groin distal to her foot (WHO-Grade III) • World Health Organization Grading Classification: • Grade I: Pitting edema reversible by elevation • Grade II: Nonpitting edema, not reversible by elevation • Grade III: Severe swelling with sclerosis and skin changes • L foot severely deformed with complete loss of definition of her toes • Skin warm, w/o erythema, induration, ecchymoses • Entire LE nontender to palpation with FROM of her knee and ankle (dorsiflexion and plantarflexion)

  5. Radiograph

  6. Diagnostic Testing • Urinalysis, CBC and Comprehensive Chemistries WNL • Foot Biopsy: Normal Skin with areas of chronic inflammation • Blood examination for microfilariae was negative, but serology for IgG4 antibodies was positive for Wuchereria bancrofti

  7. Treatment • One time combination therapy of diethylcarbamazine (DEC) and albendazole • Unlikely to reverse her condition due to chronic nature of disease and fibrosis of lymphatics • May kill any viable adult worms and diminish parasite load

  8. Force Protection • Anchorage Security • Shipboard Security Team • 200 yd exclusion zone • 2 armed picket boats • Explosive integrity check inspection area • Fleet Landing Security • Integrity checks • Pier Security Team • 100% vehicle and baggage check • 400 ft standoff radius • Barriers • MEDCAP Security • Armed HN Security Force at site and in transit • 100 yd exclusion zone for helo POC: STGCS Pageau

  9. Lymphatic Filiarisis • Caused by infection with one of 3 nematodes: • Wuchereria bancrofti • Brugia malayi • Brugia timori • 120 million people worldwide are infected, 90% due to W. bancrofti. • W. bancrofti endemic in subsaharan Africa, SE Asia, Indian subcontinent, many Pacific Islands and Greater Amazon Basin • 40 million people disfigured, severely incapacitated • Studies from India (40% of worldwide cases) • 29 days of work lost/year • >$ 843 million due to treatment costs and lost working days • Heavy Social Burden: issues with marriage

  10. Epidemiology • W. bancrofti acquired via bite of mosquitoes • Numerous species can serve as vectors: • Anopheles, Culex, Aedes, Mansonia • Prevalence increasing due to urbanization, increased breeding sites for mosquito vectors

  11. Life Cycle • Larva deposited by mosquito bite • Travel through dermis to lymphatic vessels • Growth (approx 9 months) to mature worms(20-100mm long) • Worms live 5-7 years (occasionally up to15 years) • Mate->Microfilariae (1st stage larva) • Females->release up to 10,000 microfilariae/day into bloodstream • Microfilarie taken up by mosquito bite • Develop into 2nd and 3rd stage larva over 10-14 days inside mosquito vector Hospital

  12. Life Cycle

  13. Clinical Course • Initially asymptomatic • Symptoms develop with increasing numbers of worms • Less than 1/3 of infected individuals have acute symptoms • Clinical Course is 3 phases: • Asymptomatic Microfilaremia • Acute Adenolymphangitis (ADL) • Chronic/Irreversible lymphedema • Superimposed upon repeated episodes of ADL

  14. Acute ADL • Presents with sudden onset of fever and painful lymphadenopathy • Retrograde Lymphangitis • Inflammation spreads distally away from lymph node group • Immune mediated response to dying worms • Most common areas: Inguinal nodes and Lower extremities • Inflammation spontaneously resolve after 4-7 days but can recur frequently • Recurrences usually 1-4 times/year with increasing severity of lymphedema • Secondary bacterial infections in edematous(elephantatic) areas • Filarial fever (fever w/o lymphangitis) • Tropical Pulmonary Eosinophilia • Nocturnal Wheezing (young males) • Hyperresponsiveness to microfilariae trapped in lungs

  15. Chronic Manifestations • Lymphedema • Mostly LE and inguinal, but can affect UE and breast • Initially pitting edema, with gradual hardening of tissues hyperpigmentation & hyperkeratosis • GenitaliaHydroceles • Renal involvement • Chylurialymph discharge into urine • Loss of fat and protein hypoproteinemia & anemia • Hematuria, proteinuria from ?immune complex nephritis • Secondary bacterial/fungal infections

  16. Diagnosis • Mainly Clinical • Eosinophilia, which may exceed 3000/ml • Microscopic hematuria, proteinuria, chyluria may be present • Blood smear for Microfiliariae with Giemsa or Wright’s stains • Blood drawn between 10pm-2am • Serological Testing: • Antifilaria Antibodies (IgG,IgG4,IgE) • IgG4 has 96% sensitivity • Circulating Filarial Antigens (CFA) • ELISA of Og4C3 CFA has 99% sensitivity & specificity • Negative within 12 months of curative treatment • Can be used to follow pts • Becoming gold standard Hotel Estacion

  17. Diagnosis • Ultrasound • Can be used to detect motion of adult worms • Can monitor effectiveness of drug therapy • Contrast Lymphangiography • Can directly visualize lymphatic vessels • Potential risk of causing lymph damage Alvaro Uribe Brian Nichols

  18. Treatment • Drug of Choice is single initial dose of Diethylcarbamazine (DEC) • Dosage 6mg/kg, with possible repeat dose in 6 months • Sustained 90-99% reduction in microfilaremia for at least 1 yr • SEs include fever, headache, anorexia, nausea and arthralgias • SEs not due to drug itself but host response following damage to adult worms and microfilariaerelease of lipopolysaccharide-like proteins from endosymbiotic Wolbachia organisms present within filariae • Symptoms can be treated with antipyretics/anti inflammatory agents • Ivermectin • Albendazole • Combination therapy: DEC & Albendazole has best efficacy92-99% reduction in intensity of microfilariae after 1 year, >75% reduction in annual transmission

  19. Alternate TreatmentStrategies • Attacking the Wolbachia (intracellular bacterial symbiont of worms) • Worms need Wolbachia to mate and reproduce • Doxycycline has good activity against Wolbachia • Tanzanian studies: Combination of 3 weeks of doxycycline followed by DEC/ivermectin /albendazole was efficaceous in reducing microfilarie load • Doxycyline is cheap and well-tolerated, pretreatment prior to DEC shown in small studies to reduce SEs of treatment because of lower Wolbachia load • Community based programs • WHO has ongoing Global initiative to fight lymphatic filiarisis • Massed Administration of medication in endemic areas • Community education programs to raise awareness, promote local hygiene and control mosquito vector populations

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