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Leishmaniasis

Chapter 233 | Part 5: Infectious Diseases

KEY CLINICAL POINTS

  • Leishmaniasis is caused by Leishmania species transmitted via sandfly bites, with three main clinical forms: visceral (VL), cutaneous (CL), and mucosal (ML).
  • VL is caused by Leishmania donovani complex, with high mortality if untreated, while CL and ML have varied presentations and complications.
  • Diagnosis relies on microscopy, PCR, serology, and clinical context, with treatment varying by region and parasite species.
  • Antimonial compounds, amphotericin B, and miltefosine are key therapies, though resistance and drug toxicity pose challenges.
  • Special considerations include HIV co-infection, pregnancy, and drug safety in immunocompromised patients.

1. DEFINITION & OVERVIEW

Leishmaniasis is a complex group of disorders caused by Leishmania protozoa, transmitted via sandfly vectors. It affects the reticuloendothelial system and presents as visceral (VL), cutaneous (CL), or mucosal (ML) forms. VL is the most severe, with high mortality if untreated.

Table 233-1 Geographic Distribution and Characteristic Epidemiology of Leishmaniases

ORGANIS M, ENDEMIC REGION CLINICAL SYNDROM E SPECIES VECTOR RESERVOI R TRANSMIS SION SETTING
Leishmania donovani Complex South Asia VL, PKDL L. donovani Phlebotomu s argentipes Humans Anthropono tic Rural, domestic
Sudan, South Sudan, Somalia, Ethiopia, Kenya, Uganda VL, PKDL L. donovani P. orientalis, P. martini Humans, rodents in Sudan, canines Anthropono tic, occasio nally zoonotic Majority per idomestic, o ccasionally sylvatic
Mediterrane an basin, Middle East, Central Asia, China VL, CL L. infantum P. perniciosus, P. ariasi Dogs, foxes, jackals Zoonotic Domestic, p eridomestic
ORGANIS M, ENDEMIC REGION CLINICAL SYNDROM E SPECIES VECTOR RESERVOI R TRANSMIS SION SETTING
Middle East, Saudi Arabia, Yemen VL L. donovani P. perniciosus, P. ariasi Dogs, foxes, jackals Zoonotic Domestic, p eridomestic
Central and South America VL, CL L. infantum Lutzomyia longipalpis Foxes, dogs, opossums Zoonotic Domestic, p eridomestic , periurban
Azerbaijan, Armenia, Georgia, Ka zakhstan, Kyrgyzstan, Tajikistan, Turkmenist an, Uzbekistan VL L. infantum P. turanicus Humans, dogs, foxes Anthropono tic, zoonotic Domestic
L. tropica Western India to Turkey, parts of North and East Africa CL L. tropica P. sergenti Humans Anthropono tic Urban domestic, p eridomestic
Western and Central Asia, North and sub-Sa haran Africa CL L. major P. papatasi, P. duboscqi Nile rats, rodents Zoonotic Sylvatic, pe ridomestic
Kazakhstan , Turkmenis tan, Uzbekistan CL L. major P. papatasi, P. duboscqi Gerbils Zoonotic Rural
L. aethiopica Ethiopia, Uganda, Kenya CL, DCL L. aethiopica P. longipes, P. pedifer Hyraxes Zoonotic Sylvatic, pe ridomestic
Subspecies Viannia Peru, Ecuador CL, ML L. (V.) peruviana Lutzomyia verrucarum, L. peruensis Wild rodents Zoonotic Andean Valleys
Guyana, Surinam, French Guyana, Ecuador, Brazil, Colombia, Bolivia CL, ML L. (V.) guyanensis L. umbratilis Sloths, arboreal anteaters, opossums Zoonotic Tropical forest
Central America, Ecuador, Colombia CL, ML L. (V.) pana mensis L. trapidoi Sloths Zoonotic Tropical forest and deforested areas
ORGANIS M, ENDEMIC REGION CLINICAL SYNDROM E SPECIES VECTOR RESERVOI R TRANSMIS SION SETTING
South and Central America CL, ML L. (V.) braziliensis Lutzomyia spp., L. umbratilis, Psychodop ygus wellcomei Forest rodents, per idomestic animals Zoonotic Tropical forest and deforested areas
L. mexicana Complex Central America and northern parts of South America CL, ML, DCL L. amazone nsis L. flaviscute llata Forest rodents Zoonotic Tropical forest and deforested areas
CL, ML, DCL L. mexicana L. olmeca Variety of forest rodents and marsupials Zoonotic Tropical forest and deforested areas
CL, DCL L. pifanoi L. olmeca Variety of forest rodents and marsupials Zoon,otic Tropical forest and deforested areas

1.1 Global Distribution

Leishmaniasis occurs in 99 countries, predominantly in tropical and temperate regions. Over 1 billion people live in endemic areas, with 30,000 new VL cases and 1 million CL cases annually.

1.2 Pathogenesis

Leishmania parasites are transmitted via sandfly bites. Promastigotes enter macrophages, multiply as amastigotes, and cause tissue damage. Immune response (T1 cytokines) determines disease outcome.

2. EPIDEMIOLOGY

Leishmaniasis occurs in 99 countries, with 1 billion people at risk. VL is common in South Asia, East Africa, Brazil, and the Indian subcontinent. CL is prevalent in South America, Africa, and Asia. ML is limited to South America. Climate change and HIV co-infection drive disease spread.

Table 233-2 Clinical, Epidemiologic, and Therapeutic Features of Post–Kala-Azar Dermal Leishmaniasis: East Africa and the Indian Subcontinent

FEATURE EAST AFRICA INDIAN SUBCONTINENT
Most affected country Sudan and South Sudan Bangladesh
Interval between VL and PKDL During VL to 6 months 6 months to 3 years
Age distribution Mainly children Any age
History of prior VL Yes Not necessarily
Rashes of PKDL in presence of active VL Yes No
Treatment Sodium stibogluconate for 2–3 months Miltefosine for 12 weeks
FEATURE EAST AFRICA INDIAN SUBCONTINENT
Natural course Spontaneous cure in majority of patients Spontaneous cure rarely

2.1 Risk Factors

Immunocompromised individuals (e.g., HIV, IV drug users), rural/periurban dwellers, and travelers to endemic regions are at highest risk. Zoonotic transmission is common in rural areas.

2.2 Demographics

VL is most common in children and adults in endemic regions. CL affects all ages, while ML is more common in men aged 20–40.

3. ETIOLOGY & PATHOPHYSIOLOGY

Leishmania species (20+ species) are transmitted via sandfly vectors. Pathogenesis involves promastigote entry into macrophages, intracellular amastigote replication, and immune response polarization (T1 vs. T2 cytokines). IL-10 and TGF- β drive immunosuppression in VL.

3.1 Life Cycle

Sandflies transmit promastigotes to humans. Promastigotes are phagocytosed by macrophages, replicate as amastigotes, and are transmitted via sandfly feces. Immune response determines disease outcome.

3.2 Immune Response

T1 response (IFN- γ , TNF- α ) controls infection; T2 response (IL-10, TGF- β ) leads to immunosuppression and disease progression. HIV co-infection exacerbates immunosuppression.

4. CLINICAL FEATURES

VL presents with fever, weight loss, splenomegaly, and hepatomegaly. CL causes skin ulcers, while ML leads to mucosal destruction. PKDL is a post-treatment complication with hypopigmented skin lesions.

4.1 Visceral Leishmaniasis

Fever, weight loss, splenomegaly, anemia, and hypoalbuminemia. Severe cases may present with ascites, thrombocytopenia, and secondary infections.

4.2 Cutaneous Leishmaniasis

Papules/nodules progressing to ulcers. Lesions vary in size and location, with spontaneous healing in most cases. L. tropica causes recidivans (new lesions after healing).

4.3 Mucosal Leishmaniasis

Progressive destruction of nasal/mucosal tissues. Often follows untreated CL. Leads to severe disability and airway obstruction.

4.4 Post-Kala-Azar Dermal Leishmaniasis (PKDL)

Hypopigmented macules/papules after VL recovery. Common in Indian subcontinent and East Africa. Spontaneous cure is rare.

5. DIFFERENTIAL DIAGNOSIS

VL must be differentiated from malaria, typhoid, tuberculosis, and brucellosis. CL may mimic cutaneous tuberculosis, fungal infections, or leprosy. ML is distinguished from sarcoidosis and other mucosal infections.

6. INVESTIGATIONS & DIAGNOSIS

Diagnosis via microscopy (splenic smears), PCR, serology (rK39 RDT), and clinical evaluation. Skin biopsy and culture confirm infection. Imaging (ultrasound) assesses splenomegaly.

6.1 Diagnostic Tests

Microscopy (splenic smears >95% sensitive), PCR (species identification), serology (rK39 RDT 98% sensitive in immunocompetent patients), and skin biopsy.

7. MANAGEMENT & TREATMENT

Treatment varies by region and parasite species. Pentavalent antimonials (SbV) are first-line in many areas, while amphotericin B and miltefosine are alternatives. Resistance and drug toxicity require careful monitoring.

Table 233-1 Geographic Distribution and Characteristic Epidemiology of Leishmaniases

ORGANIS M, ENDEMIC REGION CLINICAL SYNDROM E SPECIES VECTOR RESERVOI R TRANSMIS SION SETTING
Leishmania donovani Complex South Asia VL, PKDL L. donovani Phlebotomu s argentipes Humans Anthropono tic Rural, domestic
Sudan, South Sudan, Somalia, Ethiopia, Kenya, Uganda VL, PKDL L. donovani P. orientalis, P. martini Humans, rodents in Sudan, canines Anthropono tic, occasio nally zoonotic Majority per idomestic, o ccasionally sylvatic
Mediterrane an basin, Middle East, Central Asia, China VL, CL L. infantum P. perniciosus, P. ariasi Dogs, foxes, jackals Zoonotic Domestic, p eridomestic
Middle East, Saudi Arabia, Yemen VL L. donovani P. perniciosus, P. ariasi Dogs, foxes, jackals Zoonotic Domestic, p eridomestic
Central and South America VL, CL L. infantum Lutzomyia longipalpis Foxes, dogs, opossums Zoonotic Domestic, p eridomestic , periurban
ORGANIS M, ENDEMIC REGION CLINICAL SYNDROM E SPECIES VECTOR RESERVOI R TRANSMIS SION SETTING
Azerbaijan, Armenia, Georgia, Ka zakhstan, Kyrgyzstan, Tajikistan, Turkmenist an, Uzbekistan VL L. infantum P. turanicus Humans, dogs, foxes Anthropono tic, zoonotic Domestic
L. tropica Western India to Turkey, parts of North and East Africa CL L. tropica P. sergenti Humans Anthropono tic Urban domestic, p eridomestic
Western and Central Asia, North and sub-Sa haran Africa CL L. major P. papatasi, P. duboscqi Nile rats, rodents Zoonotic Sylvatic, pe ridomestic
Kazakhstan , Turkmenis tan, Uzbekistan CL L. major P. papatasi, P. duboscqi Gerbils Zoonotic Rural
L. aethiopica Ethiopia, Uganda, Kenya CL, DCL L. aethiopica P. longipes, P. pedifer Hyraxes Zoonotic Sylvatic, pe ridomestic
Subspecies Viannia Peru, Ecuador CL, ML L. (V.) peruviana Lutzomyia verrucarum, L. peruensis Wild rodents Zoonotic Andean Valleys
Guyana, Surinam, French Guyana, Ecuador, Brazil, Colombia, Bolivia CL, ML L. (V.) guyanensis L. umbratilis Sloths, arboreal anteaters, opossums Zoonotic Tropical forest
Central America, Ecuador, Colombia CL, ML L. (V.) pana mensis L. trapidoi Sloths Zoonotic Tropical forest and deforested areas
South and Central America CL, ML L. (V.) braziliensis Lutzomyia spp., L. umbratilis, Psychodop ygus wellcomei Forest rodents, per idomestic animals Zoonotic Tropical forest and deforested areas
ORGANIS M, ENDEMIC REGION CLINICAL SYNDROM E SPECIES VECTOR RESERVOI R TRANSMIS SION SETTING
L. mexicana Complex Central America and northern parts of South America CL, ML, DCL L. amazone nsis L. flaviscute llata Forest rodents Zoonotic Tropical forest and deforested areas
CL, ML, DCL L. mexicana L. olmeca Variety of forest rodents and marsupials Zoonotic Tropical forest and deforested areas
CL, DCL L. pifanoi L. olmeca Variety of forest rodents and marsupials Zoonotic Tropical forest and deforested areas

7.1 Visceral Leishmaniasis

Pentavalent antimonials (20 mg/kg for 28–30 days), amphotericin B (0.75–1.0 mg/kg alternate days), or miltefosine (2.5 mg/kg for 28 days). LAmB preferred in Mediterranean regions.

7.2 Cutaneous Leishmaniasis

Topical paromomycin, intralesional antimonials, or systemic miltefosine. Localized lesions may respond to imiquimod cream.

7.3 Mucosal Leishmaniasis

Long-term antimonial therapy, AmB, or miltefosine. Surgical intervention may be required for severe airway obstruction.

7.4 PKDL

Prolonged antimonial therapy (up to 120 days) or miltefosine. No curative treatment exists for PKDL.

8. PROGNOSIS & COMPLICATIONS

VL has high mortality without treatment. Complications include secondary infections, anemia, and organ failure. PKDL may persist for years. ML leads to severe disfigurement and airway obstruction.

9. SPECIAL CONSIDERATIONS

HIV co-infection increases VL severity and resistance. Pregnancy requires caution with antimonials. Children and elderly may need adjusted dosages. Travelers to endemic regions should use repellents and avoid sandfly bites.

10. KEY POINTS & CLINICAL PEARLS

  • Leishmaniasis is a vector-borne disease with three main forms (VL, CL, ML).
  • Diagnosis requires microscopy, PCR, and clinical context.
  • Treatment varies by region and parasite species.
  • HIV co-infection worsens prognosis.
  • PKDL is a common post-treatment complication.
  • Miltefosine and amphotericin B are key therapies in resistant cases.