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Weil's Disease

Leptospirosis

Weil's disease is a severe and potentially life-threatening zoonotic infection caused by Leptospira, a spirochetal bacterial pathogen. Fever, constitutional symptoms, gastrointestinal irritation and muscle ache progressing to hepatorenal failure, pulmonary dysfunction, and involvement of other organs is the typical clinical presentation. Clinical criteria and microbiological studies are needed to make the diagnosis.


Presentation

Leptospirosis is a widely recognized infection caused by a spirochetal bacteria, Leptospira spp., and virtually any region of the world is considered to be endemic for this infection [1] [2] [3] [4]. These microbial organisms are responsible for chronic renal infections in a range of animal hosts (livestock, medium-sized mammals, rodents, bats, amphibians and various other), who excrete them through urine and contaminate soil, water or even food, which are the primary sources of human exposure [1] [2] [5]. After the acquisition of Leptospira through abraded or damaged skin/mucosal membranes when in contact with contaminated water or soil, the mean incubation period is about 7-14 days (ranging from 2-30 days) [2] [4] [5] [6]. Although the clinical presentation can be quite variable, fever accompanied by chills, myalgia, headaches, meningismus, a nonproductive cough and gastrointestinal complaints (diarrhea, nausea, vomiting and abdominal pain) are seen in the majority of cases [2] [3] [6] [7]. In approximately 10% of patients, a severe form of leptospirosis develops and the term Weil's disease is used to describe the life-threatening organ dysfunction that is encountered, primarily involving the liver and the kidneys [2] [3] [4] [5]. Acute kidney injury (AKI) manifesting as oliguria, or even anuria in the setting of renal failure, together with jaundice, are hallmarks of Weil's disease [2] [4] [5] [6]. In addition, thrombocytopenia and several other factors contribute to hemorrhagic episodes, most frequently in the pulmonary system (presenting as hemoptysis), whereas epistaxis, petechiae, ecchymoses, melena or hematemesis can also develop [2]. Multiorgan failure, acute respiratory distress syndrome (ARDS), circulatory collapse and altered consciousness may all ensue in Weil's disease, which is why mortality rates reach up to 30%, especially in the absence of early recognition and proper therapy [3] [6]. Moreover, older patients and those who suffer from loss of consciousness are at a significantly higher risk for fatal outcomes [2] [3].

Hemoptysis
  • Pericorneal reddening, Photophobia Iridocyclitis Retro orbital pain, Rash O looks like a heart for bradycardia Weil's disease mnemonic "WEILS" Wet due to hemorrhage E is to remind me of epistaxis and other hemorrhagic manifestations like petechiae, hemoptysis[medicowesome.com]
  • Desmopressin therapy for massive hemoptysis associated with severe leptospirosis. Am J Respir Crit Care Med. 2003;167:726-28. Kishor KK, Rao PV, Bhat KR, et al. Pancreatitis in Weil’s disease. Trop Doct. 2002;32:230-31.[rarediseases.org]
  • In addition, thrombocytopenia and several other factors contribute to hemorrhagic episodes, most frequently in the pulmonary system (presenting as hemoptysis), whereas epistaxis, petechiae, ecchymoses, melena or hematemesis can also develop.[symptoma.com]
Fever
  • As fever still remained and was followed by jaundice, decreasing hemoglobin, increasing bilirubin with abnormal value of liver enzymes; other causes of disease were investigated.[ncbi.nlm.nih.gov]
  • Wikipedia: Leptospirosis Weil's syndrome canicola fever canefield fever nanukayami fever 7-day fever Rat Catcher's Yellows Fort Bragg fever black jaundice ... caused by infection with bacteria of the genus Leptospira and affects humans as well as other[memidex.com]
  • A 44-year-old black male presented with fever, myalgia and weakness. He had elevated blood urea nitrogen, creatine phosphokinase and serum glutamic-oxaloacetic transaminase.[ncbi.nlm.nih.gov]
  • The most common presentation involved fever, malaise and myalgia. Conjunctival congestion was found in 75% of the cases and jaundice was encountered in 90% of the cases.[ncbi.nlm.nih.gov]
  • We describe a case of a 53 year old man presenting with myalgia and fever in whom the diagnosis of leptospirosis was not initially considered.[ncbi.nlm.nih.gov]
Chills
  • \ ˈvī(ə)lz- , ˈwī(ə)lz- \ Medical Definition of Weil's disease : a severe form of leptospirosis that is characterized by jaundice, chills, fever, muscle pain, shortness of breath, and chest pain and that is caused by a spirochete of the genus Leptospira[merriam-webster.com]
  • Leptospirosis may occur in two phases: After the first phase (with fever, chills, headache, muscle aches, vomiting, or diarrhea) the patient may recover for a time but become ill again.[cdc.gov]
  • Although the clinical presentation can be quite variable, fever accompanied by chills, myalgia, headaches, meningismus, a nonproductive cough and gastrointestinal complaints (diarrhea, nausea, vomiting and abdominal pain) are seen in the majority of cases[symptoma.com]
  • SYMPTOMS Symptoms may include fever, headaches, chills, severe muscle pain in the calves and thighs, vomiting, diarrhoea and bloodshot eyes.[hellandback.ie]
  • Nature of the disease Leptospiral infections take many different clinical forms, usually with sudden onset of fever, headache, myalgia, chills, conjunctival suffusion and skin rash.[who.int]
Rigor
  • ) R50.9 ICD-10-CM Diagnosis Code R50.9 Fever, unspecified 2016 2017 2018 2019 Billable/Specific Code Applicable To Fever NOS Fever of unknown origin [FUO] Fever with chills Fever with rigors Hyperpyrexia NOS Persistent fever Pyrexia NOS icterohemorrhagic[icd10data.com]
High Fever
  • Symptoms Symptoms begin between 3 and 21 days after exposure, with an influenza-like illness with severe headache, high fever, tiredness, chills, muscle aches and vomiting.[hnbc.org.uk]
Vomiting
  • Signs included vomiting in 9 patients, haemorrhages in 6, and renal function was impaired in 6. Creatine phosphokinase levels were found above normal limits in 75% of the cases.[ncbi.nlm.nih.gov]
  • Leptospirosis may occur in two phases: After the first phase (with fever, chills, headache, muscle aches, vomiting, or diarrhea) the patient may recover for a time but become ill again.[cdc.gov]
  • Symptoms of leptospirosis (Weils disease) are a flu like illness with persistent and severe headache leading to vomiting and should be reported to your doctor immediately. Make sure you tell the Dr that you might have been in contact with rat urine.[ukmalt.com]
  • Safety Executive publishes information on Leptospirosis at this weblink: ROSPA (The Royal Society for the Prevention of Accidents) has some excellent guidance at this link: sease.aspx Symptoms: Flue/Fever Muscular Pains/Aches Loss of Appetite Sickness/Vomiting[cambridgecanoeclub.org.uk]
  • Although the clinical presentation can be quite variable, fever accompanied by chills, myalgia, headaches, meningismus, a nonproductive cough and gastrointestinal complaints (diarrhea, nausea, vomiting and abdominal pain) are seen in the majority of cases[symptoma.com]
Nausea
  • Although the clinical presentation can be quite variable, fever accompanied by chills, myalgia, headaches, meningismus, a nonproductive cough and gastrointestinal complaints (diarrhea, nausea, vomiting and abdominal pain) are seen in the majority of cases[symptoma.com]
  • After an incubation period that can vary from three days to three weeks, most patients suffer severe headaches, red eyes, muscle pains, fatigue, nausea and a temperature of 39C or above.[theguardian.com]
  • Infection with the bacterium causes an illness which has similar symptoms to flu – temperature, muscle aches and nausea. In mild cases these symptoms can be easily treated and patients will likely recover in a few weeks.[rya.org.uk]
Abdominal Pain
  • Although the clinical presentation can be quite variable, fever accompanied by chills, myalgia, headaches, meningismus, a nonproductive cough and gastrointestinal complaints (diarrhea, nausea, vomiting and abdominal pain) are seen in the majority of cases[symptoma.com]
  • Later symptoms may include jaundice, eye redness, abdominal pain and diarrhoea. Severe cases can cause failure of kidneys or liver. The disease can be fatal – do not ignore symptoms.[hnbc.org.uk]
Jaundice
  • Two patients are described with Weil's disease as a rare cause of jaundice. We discuss some features that can lead to early diagnosis.[ncbi.nlm.nih.gov]
  • Severe leptospirosis characterised by profound jaundice is referred to as Weil's disease.[ncbi.nlm.nih.gov]
  • A case of fulminant leptospirosis is presented, manifesting as rapid progression from acute undifferentiated febrile illness to refractory shock, jaundice, renal failure and massive pulmonary hemorrhage.[ncbi.nlm.nih.gov]
  • A 56-year-old previously healthy man was admitted to our hospital for disordered consciousness, anuria, and severe jaundice. The patient had not travelled recently, but he worked at a fish market in Tokyo.[ncbi.nlm.nih.gov]
  • ) Lancereaux-Mathieu A27.0 (leptospiral jaundice) Larrey-Weil A27.0 (leptospiral jaundice) Mathieu's A27.0 (leptospiral jaundice) Wassilieff's A27.0 (leptospiral jaundice) Weil's A27.0 (leptospiral jaundice of lung) Fever (inanition) (of unknown origin[icd10data.com]
Red Eye
  • After an incubation period that can vary from three days to three weeks, most patients suffer severe headaches, red eyes, muscle pains, fatigue, nausea and a temperature of 39C or above.[theguardian.com]
Myalgia
  • A 44-year-old black male presented with fever, myalgia and weakness. He had elevated blood urea nitrogen, creatine phosphokinase and serum glutamic-oxaloacetic transaminase.[ncbi.nlm.nih.gov]
  • The most common presentation involved fever, malaise and myalgia. Conjunctival congestion was found in 75% of the cases and jaundice was encountered in 90% of the cases.[ncbi.nlm.nih.gov]
  • We describe a case of a 53 year old man presenting with myalgia and fever in whom the diagnosis of leptospirosis was not initially considered.[ncbi.nlm.nih.gov]
  • Myalgia and jaundice were recorded in all patients. Signs included vomiting in 9 patients, haemorrhages in 6, and renal function was impaired in 6. Creatine phosphokinase levels were found above normal limits in 75% of the cases.[ncbi.nlm.nih.gov]
  • The patients with thrombocytopenia had higher frequencies of dehydration (53% vs. 35.3%, p 0.001), epistaxis (5.7% vs. 0.8%, p 0.033), hematemesis (13% vs. 4.6%, p 0.006), myalgia (91.5% vs. 84.5%, p 0.038), hematuria (54.8% vs. 37.6%, p 0.011), metabolic[ncbi.nlm.nih.gov]
Petechiae
  • […] conjunctival) Pericorneal reddening, Photophobia Iridocyclitis Retro orbital pain, Rash O looks like a heart for bradycardia Weil's disease mnemonic "WEILS" Wet due to hemorrhage E is to remind me of epistaxis and other hemorrhagic manifestations like petechiae[medicowesome.com]
  • In addition, thrombocytopenia and several other factors contribute to hemorrhagic episodes, most frequently in the pulmonary system (presenting as hemoptysis), whereas epistaxis, petechiae, ecchymoses, melena or hematemesis can also develop.[symptoma.com]
  • The disease is characterized by jaundice, fever, oliguria, headache, myalgia, haemorrhagic tendencies with purpura or petechiae, and enlargement of liver and spleen. The illness lasts from 4 to 9 days.[whonamedit.com]
Purpura
  • The disease is characterized by jaundice, fever, oliguria, headache, myalgia, haemorrhagic tendencies with purpura or petechiae, and enlargement of liver and spleen. The illness lasts from 4 to 9 days.[whonamedit.com]
Epistaxis
  • The patients with thrombocytopenia had higher frequencies of dehydration (53% vs. 35.3%, p 0.001), epistaxis (5.7% vs. 0.8%, p 0.033), hematemesis (13% vs. 4.6%, p 0.006), myalgia (91.5% vs. 84.5%, p 0.038), hematuria (54.8% vs. 37.6%, p 0.011), metabolic[ncbi.nlm.nih.gov]
  • […] through animal urine Occipital headache Suffusion (conjunctival) Pericorneal reddening, Photophobia Iridocyclitis Retro orbital pain, Rash O looks like a heart for bradycardia Weil's disease mnemonic "WEILS" Wet due to hemorrhage E is to remind me of epistaxis[medicowesome.com]
  • In addition, thrombocytopenia and several other factors contribute to hemorrhagic episodes, most frequently in the pulmonary system (presenting as hemoptysis), whereas epistaxis, petechiae, ecchymoses, melena or hematemesis can also develop.[symptoma.com]
Hematuria
  • During the first 6 days of this undiagnosed illness azotemia increased, a pericardial friction rub occurred, and hematuria was present.[ncbi.nlm.nih.gov]
  • The patients with thrombocytopenia had higher frequencies of dehydration (53% vs. 35.3%, p 0.001), epistaxis (5.7% vs. 0.8%, p 0.033), hematemesis (13% vs. 4.6%, p 0.006), myalgia (91.5% vs. 84.5%, p 0.038), hematuria (54.8% vs. 37.6%, p 0.011), metabolic[ncbi.nlm.nih.gov]
  • […] findings for leptospirosis and Weil's disease are leukocytosis, thrombocytopenia, elevated blood urea nitrogen (BUN), creatinine, and liver transaminases (alanine and aspartate aminotransferases, or ALT and AST), hyperbilirubinemia, proteinuria, pyuria, hematuria[symptoma.com]
Headache
  • Mnemonic for leptospirosis "LEPTOSPIRO " Lymphadenopathy Encephalitis (And meningitis) Pain in abdomen Transmitted through animal urine Occipital headache Suffusion (conjunctival) Pericorneal reddening, Photophobia Iridocyclitis Retro orbital pain, Rash[medicowesome.com]
  • Leptospirosis may occur in two phases: After the first phase (with fever, chills, headache, muscle aches, vomiting, or diarrhea) the patient may recover for a time but become ill again.[cdc.gov]
  • Symptoms of leptospirosis (Weils disease) are a flu like illness with persistent and severe headache leading to vomiting and should be reported to your doctor immediately. Make sure you tell the Dr that you might have been in contact with rat urine.[ukmalt.com]
  • Although the clinical presentation can be quite variable, fever accompanied by chills, myalgia, headaches, meningismus, a nonproductive cough and gastrointestinal complaints (diarrhea, nausea, vomiting and abdominal pain) are seen in the majority of cases[symptoma.com]
  • SYMPTOMS Symptoms may include fever, headaches, chills, severe muscle pain in the calves and thighs, vomiting, diarrhoea and bloodshot eyes.[hellandback.ie]
Confusion
  • This interest has prompted confusion and anxiety among recreational water user groups such as canoeists who had not previously seen themselves as being particularly at risk.[ncbi.nlm.nih.gov]
  • Sometimes the fever may fluctuate and other symptoms such as a rash, jaundice, confusion, depression, kidney failure, liver failure and meningitis may occur. The incubation period is usually 10 days but can range from 4 to 19 days.[hellandback.ie]
  • It is vital that the doctor be told of any waterborne activity you have been involved in as the symptoms are easily confused with those of flu and if you have an otherwise ‘clean occupation’ the possibility of Weil’s Disease may be overlooked in the early[rya.org.uk]

Workup

Because of the possibly life-threatening outcome of Weil's disease, the diagnosis must be made as early as possible. Signs and symptoms of Weil's disease and the initial stages of leptospirosis are not specific for the disease, making a detailed patient history pivotal in identifying recent exposure to contaminated waters, either through the occupational setting (veterinarians, farm and rice workers, hunters, shelter workers, etc.) or during recreational activities (swimming, fishing, caving, rowing) [2]. Furthermore, a thorough physical examination can reveal the potential site where the bacteria may have gained entry into the circulation, but also assess the extent of symptoms and organ involvement, after which a meticulous laboratory workup is necessary. A complete blood count (CBC) renal and kidney function tests, urinalysis and in the setting of meningitis-like symptoms, a lumbar puncture are vital [4]. Typical findings for leptospirosis and Weil's disease are leukocytosis, thrombocytopenia, elevated blood urea nitrogen (BUN), creatinine, and liver transaminases (alanine and aspartate aminotransferases, or ALT and AST), hyperbilirubinemia, proteinuria, pyuria, hematuria, and pleocytosis in the cerebrospinal fluid (CSF), respectively [4] [6] [7]. Moreover, serum inflammatory markers, such as erythrocyte sedimentation rate (ESR), will also be raised [4] [6]. A definite diagnosis, however, is made after confirming Leptospira in urine, blood, or CSF obtained from the patient. In the initial stages of the infection (first 10 days), microscopic detection of bacteria from any of the mentioned samples is widely recommended, primarily because results are provided within the hour, whereas cultivation, equally effective in the first several days, takes at least a few weeks to yield conclusive results [2] [3] [4]. However, low specificity/sensitivity rates require additional testing in most cases [3]. Thus, confirmation of immunoglobulin (Ig) M-specific antibodies through microscopic agglutination test (MAT) or enzyme-linked immunoassay (ELISA) and use of molecular methods - polymerase chain reaction (PCR), are advocated in the later stages of the disease [3] [4] [5] [6].

Pyuria
  • Typical findings for leptospirosis and Weil's disease are leukocytosis, thrombocytopenia, elevated blood urea nitrogen (BUN), creatinine, and liver transaminases (alanine and aspartate aminotransferases, or ALT and AST), hyperbilirubinemia, proteinuria, pyuria[symptoma.com]
Salmonella Typhi
  • A normal leukocyte count with neutrophilia and negative dengue NS1, dengue IgM, and Salmonella typhi IgM on admission should raise suspicion of leptospirosis, and prompt diagnostic assays for leptospirosis should be conducted.[ncbi.nlm.nih.gov]

Treatment

  • Later, in the convalescent phase after treatment, the plasma levels of D-dimer returned to normal. Plasma D-dimer can be used as a marker of fibrin formation and degradation.[ncbi.nlm.nih.gov]
  • From his history and clinical observation we suspected Weil's disease and started the corresponding treatment. Leptospira infection was ultimately confirmed by a serological study.[ncbi.nlm.nih.gov]
  • Treatment of his condition led to complete resolution after a brief stay on the intensive care unit.[ncbi.nlm.nih.gov]
  • […] anatomy; the distribution of the organism in various organs and tissues; the excretion of the spirochete, and its division, filterability, and morphological characteristics; and the clinical picture, laboratory findings, diagnosis, prophylaxis, and treatment[ncbi.nlm.nih.gov]
  • We describe the successful medical treatment of invasive aspergillosis in a patient suffering from Weil's disease with a combined therapy based on amphotericin B lipid complex and voriconazole.[ncbi.nlm.nih.gov]

Prognosis

  • The prognosis of all these patients, including that of Weil's disease was excellent. Early recognition and initiation of antibiotic therapy were found to be important.[ncbi.nlm.nih.gov]

Etiology

  • Inada and his co-workers 2 in a remarkable series of reports delineated the clinical and pathological picture, described the etiological agent and named it Spirochaeta icterohaemorrhagica .[annals.org]

Epidemiology

  • The epidemiological distribution and clinical features of 12 cases of Weil's disease from Turkey, are reviewed. The disease is most common in male farmers from rural areas. Myalgia and jaundice were recorded in all patients.[ncbi.nlm.nih.gov]
  • This review examines the epidemiology of leptospirosis in the UK, over the period 2006-10, the clinical features, diagnostic techniques and treatment.[ncbi.nlm.nih.gov]
Sex distribution
Age distribution

Prevention

  • Gaps in understanding and of adherence to preventive advice were identified. Accordingly, it was concluded that health education for canoeists could be improved.[ncbi.nlm.nih.gov]
  • A short cut review was carried out to establish whether prophylactic antibiotics should be given to prevent Weil's disease after a fall into standing water. 30 papers were found using the reported searches, of which 4 presented the best evidence to answer[ncbi.nlm.nih.gov]
  • Disease Control and Prevention Center . " Leptospira " . NCBI Taxonomy Browser . 171.[en.wikipedia.org]
  • WEIL’S DISEASE Edward Cook Any protection you can give ferrets to prevent them falling foul of Weil’s Disease has got to be money well spent.[shootinguk.co.uk]
  • Useful links: The Health & Safety Executive publishes information on Leptospirosis at this weblink: ROSPA (The Royal Society for the Prevention of Accidents) has some excellent guidance at this link: sease.aspx Symptoms: Flue/Fever Muscular Pains/Aches[cambridgecanoeclub.org.uk]

References

Article

  1. Niloofa R, Fernando N, de Silva NL, et al. Diagnosis of Leptospirosis: Comparison between Microscopic Agglutination Test, IgM-ELISA and IgM Rapid Immunochromatography Test. Dellagostin OA, ed. PLoS ONE. 2015;10(6):e0129236.
  2. Haake DA, Levett PN. Leptospirosis in Humans. Curr Top Microbiol Immunol. 2015;387:65-97.
  3. Musso D, La Scola B. Laboratory diagnosis of leptospirosis: a challenge. J Microbiol Immunol Infect. 2013;46(4):245-252.
  4. Budihal SV, Perwez K. Leptospirosis Diagnosis: Competancy of Various Laboratory Tests. J Clin Diagn Res. 2014;8(1):199-202.
  5. Kokudo T, Nakamura I, Nakamura-Uchiyama F, Komiya N, Ohnishi K. Weil's disease in a patient living in Tokyo. Intern Med. 2009;48(18):1707-1710.
  6. Mandell GL, Bennett JE, Dolin R. Mandel, Douglas and Bennett's Principles and Practice of Infectious Diseases. 8th ed. Philadelphia, Pennsylvania: Churchill Livingstone; 2015.
  7. Murray PR, Rosenthal KS, Pfaller MA. Medical Microbiology. Seventh edition. Philadelphia: Elsevier/Saunders; 2013.

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Last updated: 2018-06-22 12:22