Leptospirosis (Weil Disease)


Article Author:
Sicong Wang
Megan Stobart Gallagher


Article Editor:
Noel Dunn


Editors In Chief:
Dustin Constant
Donald Kushner


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Avais Raja
Orawan Chaigasame
Carrie Smith
Abdul Waheed
Khalid Alsayouri
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Trevor Nezwek
Radia Jamil
Erin Hughes
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Saad Nazir
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John Shell
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Daniyal Ameen
Altif Muneeb
Beenish Sohail
Nazia Sadiq
Hajira Basit
Phillip Hynes
Komal Shaheen
Sandeep Sekhon


Updated:
8/20/2019 11:26:46 PM

Introduction

Leptospirosis is an infectious disorder of animals and humans. It is the most common zoonotic infection in the world. It is easily transmitted from infected animals through their urine, either directly or through infected soil or water. [1][2]It can cause a self-limiting influenza-like illness or a much more serious disease. It is known as Weil disease, and it can progress to multiorgan failure with the potential for death.

Etiology

Leptospirosis is caused by an infection with the spirochete bacterium Leptospira. It is most often spread through exposure to the urine of infected animals either from direct contact or from contact with soil or water contaminated by the urine. Common animals that transmit Leptospirosis include farm animals such as cattle, pigs, and horses but can range from wild animals such as raccoons and porcupines to domesticated dogs. [3][4][5]The more than 160 species of animals found to carry the disease show no signs/symptoms if infected. They can be vectors of disease for several months after inoculation, sometimes never showing any signs/symptoms of infection.

Epidemiology

Leptospirosis typically occurs in a temporal climate, during the late summer or early fall in Western countries and the rainy season in the tropics. The incidence in the tropics is almost 10 times that of more temporal climates. It tends to be an unreported disease because its symptoms mimic many other disease processes; however, the World Health Organization has estimated that there are 873,000 cases annually with over 40,000 deaths.[6][7]

In the United States, there are 100 to 200 identified cases annually, and most are documented in Hawaii. However, when testing people in urban centers such as Detroit, many tested positive for past infection!

Pathophysiology

Leptospira can invade nonintact skin and mucous membranes. The infection is acquired by coming into contact with infected animals or their infected urine or body tissues. Sometimes Leptospira can even be acquired after contact with contaminated soil and water. Historically, exposures were primarily from recreational water, but more recently the United States has seen an upswing in occupational exposures from agricultural workers.

When the organism is shed in the infected animal's urine, it can survive in fresh water for up to 16 days and in soil for almost 24 days. They can then enter the human host through open wounds, mucous membranes, or the lungs if infected water is inhaled. It can also be transmitted across the placenta if an infected human is pregnant, leading to a miscarriage in the first two trimesters. If infected during the third trimester, pregnancy can result in stillbirth or intrauterine death.

Once within the body, the bacteria goes into the lymphatics and then into the blood stream. From bloodstream, the infection can spread to the entire body but tends to settle in the liver and kidneys.

It usually takes between 1 to 2 weeks for the infected person to begin to show symptoms, but could take up to a month.

History and Physical

Leptospirosis can present in two distinct clinical syndromes, icteric or anicteric.

  • The anicteric syndrome is self-limited and presents with a nonspecific flu-like illness. The onset is usually sudden and can present with a headache, cough, non-pruritic rash, fever, rigors, muscle pain, anorexia, and diarrhea. This illness may last a few days before resolution of the fever.  This form of the illness is rarely fatal and represents approximately 90% of all documented cases of Leptospirosis.
  • The anicteric syndrome can also have recurrence several days later, and this phase is called the immune stage during which aseptic meningitis can occur.  These patients can recover fully but may suffer from chronic, episodic headaches.
  • The icteric phase of leptospirosis is classically known as Weil's disease. This is a severe infection, and the manifestations include fever, renal failure, jaundice, hemorrhage, and respiratory distress. The icteric phase may also involve the heart, CNS, and muscles. This illness is usually severe and may last weeks or months if the patient survives.

The differential diagnosis for Leptospirosis is extremely large and varies from benign processes like viral upper respiratory tract infections, other viral flu-like illnesses, to severe infections from rarer "travel" conditions including Dengue Fever, malaria, Hanta virus, hemorrhagic fevers, and typhoid fever. Also consider other more common conditions (which one would be likely to consider unless specific exposure history is known) like cholecystitis, mononucleosis, primary HIV, or if unvaccinated measles or rubella.

Evaluation

The diagnosis of leptospirosis is made by growth in a specialized culture or microscopic agglutination test (only available at the CDC in Atlanta). Because multiple organ systems are involved, other bloodwork may include renal and liver function tests, coagulation studies, CBC, CSF, and chest x-ray. If there is a concern for aseptic meningitis in the immune phase, lumbar puncture to sample the CSF is necessary.[1][8]

If questions or concerns arise about how best to make this diagnosis, do not delay treatment if suspected. In severe cases, be sure to contact the Center for Disease Control (CDC) or the World Health Organization who can assist with collection and transportation of specimens for diagnostic testing.

Treatment / Management

The treatment of leptospirosis depends on the severity. Most experts suggest withholding antibiotics in mild cases. These individuals will benefit from fluids as well as pain and fever control. In outpatient cases, antibiotics that may be used include doxycycline, amoxicillin, or ampicillin.[9][2] If the infection is severe, one may use intravenous penicillin G, third-generation cephalosporins, or erythromycin. Patients with icteric leptospirosis usually need intensive care unit admission as multiple organs can be involved and decompensation can occur rapidly. In the presence of renal failure, corticosteroids may be helpful, but their use is controversial. Respiratory distress due to lung involvement may require mechanical ventilation. Additional therapies include the use of ophthalmic drops, diuretics, and inotropic agents including renal dosed dopamine. The mild form of leptospirosis is rarely fatal, but the severe form or Weil disease does carry a high mortality rate.

Pearls and Other Issues

The majority of patients who die from Weil disease have lung involvement. At least one-third of patients who develop aseptic meningitis continue to complain of headaches and other neurological deficits chronically.  It also can deposit in the eyes and stay there chronically, leading to chronic uveitis which can cause painful, blurred vision or cause a patient to see floaters.

Enhancing Healthcare Team Outcomes

Leptospirosis is a common zoonotic disorder and is best managed by a multidisciplinary team that includes the emergency department physician, primary care provider, nurse practitioner, infectious disease expert and the laboratory specialist. The diagnosis can be confirmed by culture but if the disorder is suspected, treatment should be initiated without delay.

The treatment of leptospirosis depends on the severity. Most experts suggest withholding antibiotics in mild cases. These individuals will benefit from fluids as well as pain and fever control. If the infection is severe, one may use intravenous penicillin G, third-generation cephalosporins, or erythromycin.

In severe cases, renal, lung and CNS involvement may occur and are associated with a very high mortality. These patients need ICU admission and monitoring.[10][11][12] (Level V)

 

 


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Leptospirosis (Weil Disease) - Questions

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A patient who has gone hiking in the woods is found to have a tick attached to her neck. Which of the following is NOT a tick-borne disease?



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A patient who works on a dairy farm presents with a 4-day history of fever, headache, myalgias of the calf, and abdominal pain. On physical exam, he is febrile to 102 F (38.8 C) and has conjunctival suffusion without purulent drainage. Leptospirosis is the working diagnosis. What is the most appropriate diagnostic test used to confirm the diagnosis?



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Weil disease is caused by which of the following?



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A patient who has been working on farms presents to the ER with a 1-week history of general malaise, skin rash, adenopathy, low-grade fever, and mild jaundice. He says he has pain in his joints and muscle. He also has a sore throat. He denies the use of any medications. Blood work reveals an elevated WBC, BUN, and creatinine. The only thing he can remember of the past is that he came into contact with milk that was probably contaminated about 2 weeks ago. He most likely has which of the following?



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A 21-year-old city worker is brought to the emergency department with respiratory failure, fever, renal failure, and jaundice. Ten days prior, he was cleaning a rat-infested vacant lot. Several of the crew developed flu-like symptoms but improved. The patient was sick, as well, but became jaundiced. Initial exam showed vitals as follows: temperature 38.6 degrees C, pulse 115, respirations 26, oxygen saturation 90 percent on 100 percent oxygen. The exam shows coarse breath sounds bilaterally and bilateral conjunctival suffusion. The chest x-ray has bilateral diffuse infiltrates. Laboratories show creatinine 2.7 mg/dL, total bilirubin 11.5 mg/dL, AST 45, AST 50, INR 1.2. Select the most important antibiotic for this patient.



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Which of the following organisms causes acute icteric disease with protean manifestations?



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Which of the following organisms is zoonotic and transmitted to humans primarily by contact with urine?



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A 17-year-old previously healthy female develops fever, chills, pharyngitis, muscle pain, scleral injection, headache, cervical lymphadenopathy, and photophobia lasting 7 days and was improving. The patient then seemed to relapse with additional symptoms of nausea, headache, emesis, and moderate neck stiffness. Lumbar puncture showed 180 WBCs per microliter that were all monocytes and elevated protein. Which of the following is true?



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A 17-year-old male presents to the emergency department complaining of fevers, headache, and lower extremity myalgias 5 days after returning from a camping trip. He reports drinking fresh stream water, sleeping outside on the leaves, and seeing several raccoons, deer, and opossums during his trip. He denies gastrointestinal symptoms and has no rash on physical exam. What is the most likely causative bacterium?



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A patient initially thought to be improving after taking 3 days of doxycycline for suspected leptosporosis suddenly becomes violently ill with multiorgan system failure. What condition has now developed?



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A previously healthy 28-year-old male admitted to hospital with high-grade fever, anorexia, occasionally dry cough, generalized body ache for three days. He gave no history of recent traveling, blood transfusion, contact with a sick person and significant past illness. He is a non-smoker and drinks alcohol in moderation. He worked periodically in a flooded area during rainy season. Physical examination revealed high temperature greater than 38 C, conjunctival congestion, pulse rate 84/min, blood pressure 120/80 mmHg, respiratory rate 28/min, systemic examinations were unremarkable. He was diagnosed initially as a case of Flu and discharged after two days as became afebrile. But he had persistent malaise, and within a day or two, he developed upper abdominal pain, vomiting, yellow coloration of eyes and scanty high colored urine. He had diarrhea 2 to 3 times and started gastrointestinal bleeding in the form of hematemesis and melena. He also noticed hemoptysis once. At readmission, he was found conscious; well alert, deeply icteric with conjunctival suffusion, respiratory rate greater than 46/ min, pulse rate 120 beats/min, blood pressure 100/60 mmHg, temperature 38-degrees Celsius, bilateral lung base crepitations, tender epigastrium, and hepatomegaly. What is the gold standard for characterization of the pathogen that infected this patient and what is the management for this patient?



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Leptospirosis (Weil Disease) - References

References

Russell CD,Jones ME,O'Shea DT,Simpson KJ,Mitchell A,Laurenson IF, Challenges in the diagnosis of leptospirosis outwith endemic settings: a Scottish single centre experience. The journal of the Royal College of Physicians of Edinburgh. 2018 Mar;     [PubMed]
Jiménez JIS,Marroquin JLH,Richards GA,Amin P, Leptospirosis: Report from the task force on tropical diseases by the World Federation of Societies of Intensive and Critical Care Medicine. Journal of critical care. 2018 Feb;     [PubMed]
Lokida D,Budiman A,Pawitro UE,Gasem MH,Karyana M,Kosasih H,Siddiqui S, Case report: Weil's disease with multiple organ failure in a child living in dengue endemic area. BMC research notes. 2016 Aug 15;     [PubMed]
Mazhar M,Kao JJ,Bolger DT Jr, A 23-year-old Man with Leptospirosis and Acute Abdominal Pain. Hawai'i journal of medicine     [PubMed]
Pothuri P,Ahuja K,Kumar V,Lal S,Tumarinson T,Mahmood K, Leptospirosis Presenting with Rapidly Progressing Acute Renal Failure and Conjugated Hyperbilirubinemia: A Case Report. The American journal of case reports. 2016 Aug 10;     [PubMed]
Allan KJ,Halliday JE,Cleaveland S, Renewing the momentum for leptospirosis research in Africa. Transactions of the Royal Society of Tropical Medicine and Hygiene. 2015 Oct;     [PubMed]
Rodríguez-Vidigal FF,Vera-Tomé A,Nogales-Muñoz N,Muñoz-García-Borruel M,Muñoz-Sanz A, Leptospirosis in South-western Spain. Revista clinica espanola. 2014 Jun-Jul;     [PubMed]
Shivalli S, Diagnostic evaluation of rapid tests for scrub typhus in the Indian population is needed. Infectious diseases of poverty. 2016 May 12;     [PubMed]
Guidelines for the control of leptospirosis. WHO offset publication. 1982;     [PubMed]
Nafeev AA,Nechaeva AS,Salina GV,Abbiazova VI,Vetlugin NI, [Irreversible sequels of leptospirosis]. Meditsinskaia parazitologiia i parazitarnye bolezni. 2012 Jan-Mar;     [PubMed]
Nafeev AA,Vetlugin NI,Feofanova SG,Nechaeva AS,Savinova GA, [Leptospirosis and its complications]. Terapevticheskii arkhiv. 2011;     [PubMed]
Andrade L,Cleto S,Seguro AC, Door-to-dialysis time and daily hemodialysis in patients with leptospirosis: impact on mortality. Clinical journal of the American Society of Nephrology : CJASN. 2007 Jul;     [PubMed]

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