II. Epidemiology

  1. Worldwide: Common
  2. United States: 100-200/year (underestimated)
    1. Hawaii accounts for 50% of cases

III. Pathophysiology

  1. Fresh-waterborne Spirochete (Leptospira interrogans, Leptospira biflexa and 14 other species)
  2. Transmission
    1. Passed via infected animals urine
      1. Rats (most common)
      2. Livestock (e.g. horses, cattle, sheeps, goats)
      3. Dogs (often asymptomatic)
    2. Leptospira remains viable for months in water and soil (esp. tropical and subtropical climates)
    3. Penetrates mucus membranes, broken skin, Conjunctiva
  3. Incubation: 2 to 30 days
  4. Mechanism of injury
    1. Small vessel injury (Vasculitis)

IV. Risk Factors

  1. Water activity in endemic areas (Hawaii, Malaysia)
  2. Fresh-water related activities (contaminated water or soil exposure)
    1. Farming or gardening
    2. Hiking across streams
    3. Swimming
    4. Rafting
    5. Canoeing
    6. Spelunking
    7. Camping
  3. Occupations
    1. Veterinary workers
    2. Agriculture
    3. Sewage workers
    4. Fishing industry
    5. Flood exposure

V. Findings: Symptoms and signs

  1. Follows 7-12 day incubation
  2. Anicteric Leptospirosis (90% of cases)
    1. Phase 1: Initial septicemic phase (lasts 3-7 days)
      1. Headache
      2. High fever
      3. Myalgia
      4. Calf tenderness
      5. Conjunctivitis
      6. Maculopapular rash
    2. Phase 2 (follows brief improvement from phase 1)
      1. Aseptic Meningitis
      2. Uveitis
      3. Chorioretinitis
      4. Hematuria
      5. Hepatomegaly
      6. Splenomegaly
      7. Conjunctival injection
  3. Icteric Leptospirosis (Weil's Syndrome, 10% of symptomatic cases, 5-15% mortality)
    1. Much more severe than anicteric form
    2. Fever
    3. Jaundice
    4. Azotemia
    5. Hypotension
    6. Hemorrhagic Vasculitis

VI. Labs

  1. Start empiric therapy if Leptospirosis suspected
    1. Do not wait for diagnostic tests
  2. Serology
    1. Acute phase serum 1-2 weeks after illness onset
    2. Convalesent serum 3-4 weeks after illness onset
  3. Culture (Growth may require 16 weeks)
    1. Blood Culture
    2. Urine Culture
    3. Cerebrospinal Fluid Culture
  4. Dark field microscopy
    1. Best Test Sensitivity is flow blood or CSF within the first 7-10 days
    2. After 7 days, urine is best sample

VII. Management

  1. Consider empirical treatment if suspected
  2. Mild to moderate infection
    1. Doxycycline 100 mg orally twice daily for 7 days (if age >8 years old) OR
    2. Amoxicillin 16 mg/kg up to 500 mg orally three times daily for 7 days OR
    3. Azithromycin 10 mg/kg up to 1 g on day 1, then 5 mg/kg up to 500 mg daily for 2 days
  3. Severe infection (hospitalized)
    1. Penicillin G 100,000 units/kg up to 1.5 MU IV every 6 hours for 7 days or
    2. Ceftriaxone 100 mg/kg up to 2 g IV every 24 hours for 7 days
    3. Alternatively IV Doxycycline may be used if age >8 years old

VIII. Complications

IX. Prevention

  1. Doxyxycline 200 mg once weekly for prophylaxis if high risk of exposure
  2. Infected pet quarantine
    1. Avoid contact with urine of infected pet and disinfect contaminated areas (with household cleaners)
    2. Infected dogs should be walked to urinate away from water bodies and from human frequented areas
  3. Other measures
    1. Consider Vaccination of dog (covers 4 most common Leptospira species)
    2. Keep dogs away from standing water

X. References

  1. (2016) Sanford Guide to Antibiotics, IOS App accessed 4/14/2016
  2. Anderson (2014) Crit Dec Emerg Med 28(7): 11-9
  3. Tappero in Mandell (2000) Infectious Disease p. 2495
  4. Day (2016) Am Fam Physician 94(10): 794-802 [PubMed]
  5. Joyce (2002) Prim Care 29(4):971-81 [PubMed]
  6. Lo Re (2003) Am Fam Physician 68(7):1343-50 [PubMed]
  7. Perkins (2017) Am Fam Physician 95(9):554-60 [PubMed]

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Ontology: Leptospira (C0023358)

Definition (NCI) A genus of Gram-negative, aerobic, flexible, spiral-shaped, spirochete with internal flagella in the phylum Spirochaetes.
Definition (NCI_CDISC) Any bacteria that is not assigned to the species level but can be assigned to the Leptospira genus level.
Definition (MSH) A genus of aerobic, helical spirochetes, some species of which are pathogenic, others free-living or saprophytic.
Definition (CSP) genus of aerobic, helical spirochetes, some species of which are pathogenic, others free-living or saprophytic.
Concepts Bacterium (T007)
MSH D007919
SnomedCT 26764003
LNC LP18314-2
Spanish género Leptospira, género Leptospira (organismo), Leptospira (organismo), Leptospira
Swedish Leptospira
English Leptospira (organism), Genus Leptospira (organism), Leptospira, leptospira, leptospiral, Leptospira Noguchi 1917 (Approved Lists 1980) emend. Faine and Stallman 1982, LEPTOSPIRA, Leptospira, NOS, Genus Leptospira
Czech Leptospira
Finnish Leptospira
Russian LEPTOSPIRY, LEPTOSPIROZ, VOZBUDITELI, ЛЕПТОСПИРОЗ, ВОЗБУДИТЕЛИ, ЛЕПТОСПИРЫ
Polish Leptospira
Norwegian Leptospira
French Leptospira
German Leptospira, Leptospiren
Italian Leptospira
Dutch Leptospira
Portuguese Leptospira

Ontology: Leptospirosis (C0023364)

Definition (NCI) A contagious bacterial infection caused by spirochetes of the genus Leptospira. Humans are infected by contact with water and soil which have been contaminated with animal waste products. The signs and symptoms include an initial flu-like phase, followed by a second phase in which patients may develop meningitis, liver failure and renal failure.
Definition (MSH) Infections with bacteria of the genus LEPTOSPIRA.
Concepts Disease or Syndrome (T047)
MSH D007922 , C531728
ICD9 100.9, 100
ICD10 A27 , A27.9
SnomedCT 77377001, 186954006, 186953000, 154391003, 186958009, 186959001, 187311001, 266213004, 50574007
LNC LA10484-6
English Leptospiroses, Leptospirosis, Leptospirosis, unspecified, [X]Leptospirosis, unspecified, leptospirosis, leptospirosis (diagnosis), Leptospirosis NOS, Weil Disease, Leptospirosis [Disease/Finding], Swineherd's disease, Rice-field fever, Cane-cutter fever, Stuttgart disease, Mud fever, fort bragg fever, queensland fever, spirochaetal jaundice, swamp fever, red water fever, mud fever, leptospiroses, [X]Leptospirosis, unspecified (disorder), Leptospirosis NOS (disorder), Swamp fever, Red water fever, Cane cutter's fever, Harvest fever, Queensland fever, Rice-field worker's disease, Spirochaetal jaundice, Spirochetal jaundice, Japanese autumnal fever, Infection due to Leptospira, Leptospirosis (disorder), Queensland; fever, fever; Queensland, fever; mud, fever; swamp, leptospirosis, infection; leptospiral, leptospiral; infection, mud; fever, swamp fever; leptospirosis, swamp; fever, leptospirosis, Infection due to Leptospira, NOS, Leptospirosis, NOS, Fever, swamp
Dutch leptospirosis, niet-gespecificeerd, Leptospira; infectie, Queensland; koorts, infectie; Leptospira, koorts; Queensland, koorts; modder, koorts; moeras, leptospirose, modder; koorts, moeras; koorts, leptospirose, moeraskoorts; leptospirose, Leptospirose, niet gespecificeerd, leptospirosis, Leptospirose
French Leptospirose, non précisée, Leptospirose, Infection à Leptospira, Infection à leptospires
German Leptospirose, unspezifisch, Leptospirose, nicht naeher bezeichnet, Leptospirose
Italian Leptospirosi non specificata, Leptospirosi
Portuguese Leptospirose NE, Leptospirose
Spanish Leptospirosis no especificada, fiebre de los pantanos, leptospirosis, SAI, [X]leptospirosis, no especificada (trastorno), [X]leptospirosis, no especificada, leptospirosis, SAI (trastorno), enfermedad de los trabajadores de los campos de arroz, fiebre de Queensland, infección por Leptospira, leptospirosis (trastorno), leptospirosis, Leptospirosis
Japanese レプトスピラ症, レプトスピラ症、詳細不明, レプトスピラショウショウサイフメイ, レプトスピラショウ
Swedish Leptospiros
Czech leptospiróza, Leptospiróza, blíže neurčená, Leptospiróza
Finnish Leptospiroosi
Russian LEPTOSPIROZ, ЛЕПТОСПИРОЗ
Korean 상세불명의 렙토스피라증, 렙토스피라증
Croatian LEPTOSPIROZA
Polish Leptospiroza, Zakażenie Leptospira
Hungarian leptospirosis, Leptospirosis, nem meghatározott
Norwegian Leptospirose

Ontology: Weil Disease (C0043102)

Definition (MSH) A severe form of LEPTOSPIROSIS, usually caused by LEPTOSPIRA INTERROGANS SEROVAR ICTEROHAEMORRHAGIAE and occasionally other serovars. It is transmitted to humans by the rat and is characterized by hemorrhagic and renal symptoms with accompanying JAUNDICE.
Concepts Disease or Syndrome (T047)
MSH D014895
ICD9 100.0
ICD10 A27.0
SnomedCT 398067003, 69092001, 186954006, 154391003, 266213004, 398222003
English Disease, Weil, Icterohemorrhagic Leptospirosis, Jaundice, Spirochetal, Leptospirosis, Icterohemorrhagic, Spirochetal Jaundice, Weil's Disease, Weils Disease, Disease, Weil's, Leptospirosis icterohaemorrh., Spirochaetal jaundice, Spirochetal jaundice, WEILS DIS, WEIL DIS, Weil's syndrome (diagnosis), Weil's syndrome, Haemorrhagic leptospirosis with jaundice, Hemorrhagic leptospirosis with jaundice, Leptospiros icterohem, Weil Disease, Weil disease, Leptospiral or spirochetal jaundice (hemorrhagic), Weil Disease [Disease/Finding], disease weil, weil's disease, weil disease, spirochaetal jaundice, weils disease, leptospirosis icterohemorrhagica (diagnosis), Leptospirosis icterohemorrhagica, Weil's disease, Hemorrhagic leptospiral jaundice, Infection due to Leptospira icterohemorrhagiae, Infection due to Leptospira interrogans, Leptospirosis icterohaemorrhagica, Icteric leptospirosis, Haemorrhagic leptospiral jaundice, Leptospirosis icterohemorrhagica (disorder), Weil's disease (disorder), spirochetal jaundice, Weil, epidemic; jaundice, leptospiral, icterohemorrhagica; leptospirosis, jaundice; epidemic, leptospiral, jaundice; leptospiral, jaundice; spirochetal, leptospiral; epidemic jaundice, leptospiral; jaundice, leptospirosis; icterohemorrhagica, spirochetal; epidemic jaundice, spirochetal; jaundice, Hemorrhagic leptospiral or spirochetal jaundice, Leptospiral or spirochetal jaundice
Dutch hemorragische leptospirose met geelzucht, leptospirosis icterohaemorrhagica, Leptospira; epidemische geelzucht, Leptospira; geelzucht, epidemisch; geelzucht, Leptospira, geelzucht; Leptospira, geelzucht; epidemisch, Leptospira, geelzucht; spirochetaal, icterohemorrhagica; leptospirose, leptospirose; icterohemorrhagica, spirochetaal; epidemische geelzucht, spirochetaal; geelzucht, Leptospirosis icterohaemorrhagica, ziekte van Weil, Icterohemorragische leptospirosis, Weil, ziekte van, Ziekte van Weil, Geelzucht door spirocheten, Leptospirosis, icterohemorragische
French Leptospirose hémorragique avec ictère, Leptospirose ictéro-hémorragique, Maladie de Weil, Leptospirose ictérohémorragique, Maladie des jeunes porchers
German Leptospirosis icterohemorrhagica, haemorrhagische Leptospirose mit Gelbsucht, Leptospirosis icterohaemorrhagica [Weil-Krankheit], Leptospirosis icterohaemorrhagica, Weil-Krankheit, Gelbsucht, Spirochäten-
Italian Leptospirosi ittero-emorragica, Leptospirosi itteroemorragica, Leptospirosi emorragica con ittero, Malattia di Weil
Portuguese Leptospirose hemorrágica com icterícia, Leptospirose icteremorrágica, Leptospirose Íctero-Hemorrágica, Leptospirose Icteroemorrágica, Leptospirose Espiroquetal, Doença de Weil
Spanish Leptospirosis icterohemorrágica, Leptospirosis hemorrágica con ictericia, leptospirosis ictérica, ictericia leptospiral hemorrágica, infección por Leptospira interrogans, infección por Leptospira icterohemorrhagiae, ictericia espiroquetal hemorrágica, enfermedad de Weil (trastorno), enfermedad de Weil, ictericia lepstospirósica hemorrágica, leptospirosis icterohemorrágica (trastorno), leptospirosis icterohemorrágica, Enfermedad de Weil, Ictericia por Espiroquetas, Leptospirosis Icterohemorrágica
Japanese 黄疸を伴う出血性レプトスピラ症, オウダンヲトモナウシュッケツセイレプトスピラショウ, ワイルビョウ, Weil病, スピロヘータ性黄疸, フィードラー病, レプトスピラ性黄疸, ワイル病, 黄疸出血性レプトスピラ病, 黄疸出血性レプトスピラ症, レプトスピラ病-黄疸出血性, 黄疸-スピロヘータ性
Swedish Weils sjukdom
Czech Weilova nemoc, Onemocnění vyvolané infekcí Leptospira icterohaemorrhagica, Hemoragická leptospiróza s icterem, Hemoragická leptospiróza se žloutenkou
Finnish Weilin tauti
Russian LEPTOSPIROZ IKTEROGEMORRAGICHESKII, VASIL'EVA-VEILIA BOLEZN', VEILIA BOLEZN', ВАСИЛЬЕВА-ВЕЙЛЯ БОЛЕЗНЬ, ВЕЙЛЯ БОЛЕЗНЬ, ЛЕПТОСПИРОЗ ИКТЕРОГЕМОРРАГИЧЕСКИЙ
Korean 황달출혈성 렙토스피라증
Polish Choroba Weila, Zakażenie Leptospira icterohaemorrhagiae, Krętkowica żółtaczkopodobna, Żółtaczka zakaźna krętkowa, Żółtaczka leptospirowa
Hungarian Leptospirosis icterohaemorrhagica, Weil-betegség, Haemorrhagiás leptospirosis icterussal, vérzéses leptospirosis sárgasággal
Norwegian Weils sykdom, Leptospirosis icterohaemorrhagica