Filariasis lymphatic B74.8

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 15.04.2021

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Synonym(s)

elephantiasis arabum; elephantiasis filarica; Filariasis; Filariasis lymphatic; lymphatic; Lymphatic filariasis; tropical elephantiasis

History
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van Linschoten, 1588; Wucherer, 1866; Lewis, 1872; Cobbold, 1877; Manson, 1877; Bancroft, 1878

Definition
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Nematode infection transmitted by blood-sucking mosquitoes of the genus Aedes, Anopheles, Culex, Mansonia Infestation of the lymphatic vessels is in the foreground. Special form: Tropical pulmonary eosinophilia.

Pathogen
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Wuchereria bancrofti (common in humid tropical areas worldwide): filamentous worms, males 4 cm, females up to 10 cm long.

Brugia malayi (found only in Southeast Asia), Brugia timori (restricted to some islands in Indonesia): smaller than Wuchereria.

Classification
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Acute lymphatic filariasis: 4 weeks to 16 months after infection: episodic, occasionally febrile lymphadenitis and lymphangitis, sometimes with accompanying reversible lymphedema. In turn, distal lymph nodes are also affected ("a hot stone rolling down to the foot"). Epididymitis and orchitis are also seen.

Chronic lymphatic filariasis: elephantiasis, hydrocele, chyluria, steatorrhea, chylous ascites, malabsorption, glomerulonephritis.

Tropical pulmonary eosinophilia: pulmonary infiltrates (detectable on chest X-ray), peripheral eosinophilia, cough; asthma attacks predominantly at night after prolonged stay in the tropics without detection of microfilariae in the blood in probable filariasis.

Occurrence/Epidemiology
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Tropical areas, especially South, Southeast and East Asia, North Africa, tropical regions of Africa and Central America. At least 100 million people are infected.

Etiopathogenesis
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Transmission of L3 larvae by various bloodsucking mosquito species (vectors: Aedes, Anopheles, Culex, Mansonia). Settling in the lymph vessels and lymph nodes where they mature and mate. Microfilariae that develop circulate in the blood and are taken up by mosquitoes during sucking. Most filariae harbor bacterial endosymbionts of the genus Wolbachia (related to Rickettsia). These are important for the immunology of the filariae themselves and for their embryogenesis, but also induce disease symptoms in the macro host (e.g. they are blamed for corneal clouding).

Clinical features
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Initially inflammatory-allergic general reaction with fever, pain in the limbs, urticarial skin symptoms, lymphangitis and lymphadenitis. Inflammation of the spermatic cord, testis and epididymis ( Meyer-Kouwenaar's syndrome).

After years to decades of progression, the sequelae of lymphangitis due to chronic inflammation come to the fore: lymphoedema of extremities, scrotum, penis, vulva, mammae, initially as soft, doughy oedematous swellings, later by fibrosis. Lymphuria, lymphoceles due to bursting of lymphatic vessels are possible. Transition to "elephantiasis filarica" (see elephantiasis).

Other symptoms:

"tropical pulmonary eosinophilia" with persistent, especially nocturnal cough with mucupurulent sputum, pulmonary hypertension, hepatosplenomegaly, lymph node swelling, eosinophilia, eosinophilic granulomatous inflammation.

Laboratory
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Eosinophilia.

Diagnosis
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Microfilariae detection in blood:

  • Wuchereria: after 7 months
  • Brugia malayi: after 3-12 months
  • Brugia timori: after 3 months.

Nocturnal/diurnal microfilariae detection (microscopy and PCR) from blood, lymph/lymph nodes and skin (skin snips - to exclude onchocerciasis), blood eosinophilia/eosinophil kinetics, DEC provocation test, serological antibody detection against filarial crude antigen, IgG4 serum antibodies.

Patchy infiltrates in X-ray thorax and eosinophilia in broncho-alveolar lavage in tropical pulmonary hypertension.

Differential diagnosis
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Complication(s)
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Malabsorption, glomerulonephritis.

External therapy
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Possibly surgical intervention for the treatment of larger lymphedema, otherwise compression therapy, manual and possibly apparatus-based intermittent lymph drainage. S.a.u. lymphedema.

Internal therapy
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Doxycycline: the antibiotic elimination of Wolbachia with doxycycline leads to a complete inhibition of embryogenesis and thus to the sterility of the worms.

1st choice therapy: combination of ivermectin (micro- and macrofilaricidal effect; e.g. Mectizan) 1 time 400 μg/kg KG p.o. (blood count and transaminase control required!) and diethylcarbamazine (microfilaricidal effect) 6 mg/kg bw/day p.o. (previously exclude onchocerciasis). Doxycycline 100 mg/day p.o. for 6 weeks.

Alternatively: also in combination: Albendazole (e.g. Eskazole) 1 time / day 400 mg p.o. for 10-14 days.

When using diethylcarbamazine, simultaneous administration of glucocorticoids such as prednisolone (e.g. Decortin H) 1 mg/kg bw/day and, if necessary, antihistamines such as dimetinden (e.g. Fenistil Drg.) to reduce NW such as fever, headache, painful lymph node swelling (allergic reaction to the killed filariae).

Prophylaxis
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Mosquito repellent.

Note(s)
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Remember! Diagnosis and therapy only by experienced tropical physicians.

Literature
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  1. Bancroft J (1878) Cases of filarious disease. Transactions of the Pathological Society (London) 29: 406-419
  2. Burnell AC (1885) The voyage of Jan Hughen van Linschoten to the East Indies. Volume 1, Hakluyt Society, London
  3. Cobbold TS (1877) Discovery of the adult representative of microscopic filariae. Lancet ii: 70-71
  4. Galvez Tan JZ (2003) The Elimination of Lymphatic Filariasis: A Strategy for Poverty Alleviation and Sustainable Development - Perspectives from the Philippines. Filaria J 2: 12
  5. Hoerauf A et al (2001): Depletion of wolbachia enterobacteria in onchocerca vólvulus by doxcycline and microfilaridermia after ivermectin therapy. Lancet 357: 1415-1416
  6. Lewis TR (1872) On a haematozoon inhabiting human blood, its relation to chyluria and other diseases. 8th Annual Report of the Sanitary Commission Government of India. Sanitary Commission Government of India, Calcutta, p. 241-266
  7. Manson P (1878) On the development of Filaria sanguinis hominis and on the mosquito considered as a nurse. J Linn Soc (Zool) 14: 304-311
  8. Melrose WD (2002) Lymphatic filariasis: new insights into an old disease. Int J Parasitol 32: 947-960
  9. Moulia-Pelat JP et al (1993) Long-term efficacy of single-dose treatment with 400 μg/kg of ivermectin in bancroftian filariasis: results at one year. Trop Med Parasitol 44: 333-334
  10. Moulia-Pelat JP et al (1995) Combination ivermectin plus diethylcarbamazine: a new effective tool for control of lymphatic filariasis. Trop Med Parasitol 46: 9-12
  11. Nutman TB (2001) Lymphatic filariasis: new insights and prospects for control. Curr Opin Infect Dis 14: 539-546
  12. Ottesen EA (2002) Major progress towards eliminating lymphatic filariasis. N Engl J Med 347: 1885-1886
  13. Rathaur S et al (2003) Brugia malayi and Wuchereria bancrofti: gene comparison and recombinant expression of pi-class related glutathione S-transferases. Exp Parasitol 103: 177-181
  14. Usurer OE (1866) Sobre a molestia vulgarmente denominada oppilacao ou cancaco. Gaz Med Bahia 1: 27-29, 39-41, 52-54, 63-64

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Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 15.04.2021