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Last updated: 07/24/2024
Years published: 2005, 2009, 2013, 2016, 2019
NORD gratefully acknowledges the Centers for Disease Control and Prevention, Division of Vector-Borne Diseases, for assistance in the preparation of this report.
Tularemia, is caused by infection with the bacterium Francisella tularensis, which is found in small mammals such as rodents and rabbits, and arthropods, such as ticks. The bacterium that causes tularemia is most often transmitted to humans by tick or biting fly bite, handling of an infected animal, or inhalation or ingestion of the bacterium. People have not been known to transmit the infection to others. The severity of tularemia varies greatly. Some cases are mild and self-limiting, others may have serious complications, and a small percentage (less than 2 percent) of cases in the United States is fatal.
The symptoms of tularemia vary greatly among affected individuals. Some individuals may have no apparent symptoms (asymptomatic); others can develop serious complications affecting several organ systems with potentially life-threatening complications. After infection, the period of time it takes symptoms to appear (incubation period) is usually three to five days, but may take as long as two weeks.
Most cases of tularemia begin with rapid onset of nonspecific, flu-like symptoms including fever, chills, headaches, muscle pain (myalgia), joint pain (arthralgia), loss of appetite, and a general feeling of ill health (malaise).
Additional symptoms may occur depending upon how a person is infected. Infection through skin (e.g., tick bite or handling of infectious matter) may result in an ulcer or rash on the skin at the point of infection and swollen, painful lymph nodes (lymphadenopathy). These cases may be referred to as ulceroglandular tularemia. In some cases, individuals will have lymphadenopathy without any skin symptoms. These cases are referred to as glandular tularemia. Ulceroglandular and glandular tularemia account for more than 75 percent of cases.
Infection through breathing in (inhalation) the bacterium may result in infection of the lungs (pneumonia), a collection of fluid around the lungs (pleural effusion), and lung abscesses. These cases are referred to as pneumonic tularemia. Eating undercooked, infected animal meat or drinking contaminated water may result in sore throat, nausea, vomiting, diarrhea, and abdominal pain. These cases are referred to as oropharyngeal tularemia. In rare cases, gastrointestinal bleeding may occur.
Tularemia can also affect the eyes resulting in inflammation of the delicate membrane that lines the eyes (conjunctiva), a condition called conjunctivitis. Conjunctivitis can cause pain, redness, and itching of the eyes. Nearby lymph nodes may be swollen and painful. These cases are referred to oculoglandular tularemia.
Some individuals may develop the typhoidal form of tularemia in which fever, myalgias, and general ill health (malaise) develop without accompanying skin lesions or lymphadenopathy. Because of the lack of obvious symptoms, this form of tularemia is difficult to diagnose.
Tularemia has the potential to affect various organ systems of the body including the central nervous system, heart, and liver resulting in inflammation of the membranes surrounding the brain and spinal cord (meningitis), inflammation of the lining of the heart (endocarditis), and inflammation of the liver (hepatitis). Additional serious complications potentially associated with tularemia include infection of the blood (sepsis) and infection of various bones (osteomyelitis).
Tularemia is caused by the bacterium Francisella tularensis. Most cases occur from being bitten by flies or ticks carrying the bacterium or from exposure to tissue from an animal infected with the bacteria.
Tularemia has been found in >100 animal species including rabbits, muskrats, squirrels and beavers. Handling infected animal tissue (e.g., skinning or dressing animal carcasses) or eating insufficiently cooked meat from an infected animal may result in tularemia. Domestic pets such as dogs and cats can be responsible for transmitting tularemia to humans as well. Dogs and cats may come in contact with infected animals or ticks and may spread the disease to humans through their saliva or claws.
Tularemia may be also transmitted through the air by breathing in (inhalation) of aerosolized bacteria. Common activities including cutting brush or mowing lawns can aerosolize the bacteria from the environment and cause infection in humans.
Francisella tularensis is an extremely resilient bacterium and able to survive in the environment (e.g., mud, water, or decaying animal carcasses) for weeks and, rarely, direct contact or ingestion of contaminated materials including water or soil may cause tularemia.
No human-to-human transmission has been documented in the medical literature.
Tularemia has gained more attention in recent years because of its potential for use in biological warfare.
Tularemia affects males and females, although the majority of cases are males, probably because of greater outdoor exposure opportunities. The disease is rare in the United States with approximately 100-200 new cases reported each year. Some researchers believe that many cases of tularemia often go unreported or misdiagnosed, making it difficult to determine the true frequency of this disease in the general population.
Although tularemia can occur anywhere in the United States, more than half of the cases reported each year occur in rural areas of Arkansas, Missouri, Oklahoma, and Kansas. Tularemia may be found in all parts of Europe and Asia with greater frequency in Siberia and Scandinavian countries.
In Japan, tularemia may be known as yato-byo or Oharaโs disease, after Dr. Ohara who first described a rabbit-associated febrile disease.
A diagnosis of tularemia is made by a thorough clinical evaluation, a detailed patient history, and a variety of tests that may include an attempt to isolate the bacteria from blood or body tissue or through specialized blood tests (serologic tests) that measure the bodyโs immune system response against the bacterium.
Treatment
Tularemia is treated with antibiotics. Streptomycin is the drug of choice, but gentamicin is an acceptable alternative. Other antibiotics such as doxycycline and ciprofloxacin may also be used and can be taken by mouth (orally).
Other treatment is symptomatic and supportive.
Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov
All studies receiving U.S. Government funding, and some supported by private industry, are posted on this government web site.
For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:
Tollfree: (800) 411-1222
TTY: (866) 411-1010
Email: [email protected]
Some current clinical trials also are posted on the following page on the NORD website:
https://rarediseases.org/living-with-a-rare-disease/find-clinical-trials/
For information about clinical trials sponsored by private sources, contact:
www.centerwatch.com
For information about clinical trials conducted in Europe, contact:
https://www.clinicaltrialsregister.eu/
Research on infectious diseases such as tularemia is ongoing. For more information about these disorders, contact the World Health Organization (WHO) listed in the Resources section below.
TEXTBOOKS
Longo DL, et al. eds. Harrisonโs Principles of Internal Medicine, 18th e. ed. New York, NY: McGraw-Hill. 2012: retrieved May 2016.
JOURNAL ARTICLES
Kwit NA, et al. Human tularaemia associated with exposure to domestic dogs-United States, 2006-2016. Zoonoses and Public Health. 2019; 66(4):417-421.
Nelson C, Kugeler K, Petersen J. Mead P. Tularemia โ United States, 2001โ2010. MMWR. 2013: 62(47): 963-966.
World Health Organization. WHO Guidelines on Tularemia. Geneva: WHO Press. 2007.
Oyston PC, Sjostedt A, Titball RW. Tularemia: bioterrorism defence renews interest in Francisella tularensis. Nat Rev Microbiol. 2004;2:967-78.
Goethert HK, Shani I, Telford SR 3rd. Genotypic diversity of Francisella tularensis infecting Dermacentor variabilis ticks on Marthaโs Vineyard, Massachusetts. J Clin Microbiol. 2004;42:4968-73.
Tarnvik A, Priebe HS, Grunow R. Tularaemia in Europe: an epidemiological review. Scad J Infect Dis. 2004:36:350-5.
Cronquist SD. Tularemia: the disease and the weapon. Dermatol Clin. 2004;22:313-20.
Conlan JW. Vaccines against Francisella tularensis โ past, present and future. Expert Rev Vaccines. 2004;3:307-14.
Avashia SB, et al., First reported prairie dog-to-human tularemia transmission, Texas, 2002. Emerg Infect Dis. 2004;10:483-6.
Gallagher-Smith M, Kim J, Al-Bawardy R, Josko D. Francisella tularensis: possible agent in bioterrorism. Clin Lab Sci. 2004;17:35-9.
Tarnvik A, Berglund L. Tularemia. Eur Respir J. 2003;21:361-73.
Jensen WA, Kirsch CM. Tularemia. Semin Respir Infect. 2003;18:146-58.
Dennis DT, Inglesby TV, Henderson DA, et al. Tularemia as a Biological Weapon: medical and public health management. JAMA 2001; 285:2763-73.
Hornick R. Tularemia Revisited. N Engl J Med. 2001;345:1637-9.
Feldman KA, et al., An outbreak of primary pneumonic tularemia on Marthaโs Vineyard. N Engl J Med. 2001;345:1601-6.
INTERNET
Maurelius, K.Tularemia in Emergency Medicine. Medscape. Updated: Sep 05, 2018. Available at: https://www.emedicine.com/emerg/topic591.htm Accessed Oct 2, 2019.
Centers for Disease Control (CDC) Website. Last Update December 13, 2018 www.cdc.gov/tularemia Accessed Oct 2, 2019.
Center for Biosecurity. University of Pittsburgh Website. Tularemia Fact Sheet. October 13, 2011. Available at: https://www.upmc-biosecurity.org/website/our_work/biological-threats-and-epidemics/fact_sheets/tularemia.html Accessed Oct 2, 2019.
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The Genetic and Rare Diseases Information Center (GARD) has information and resources for patients, caregivers, and families that may be helpful before and after diagnosis of this condition. GARD is a program of the National Center for Advancing Translational Sciences (NCATS), part of the National Institutes of Health (NIH).
View reportOrphanet has a summary about this condition that may include information on the diagnosis, care, and treatment as well as other resources. Some of the information and resources are available in languages other than English. The summary may include medical terms, so we encourage you to share and discuss this information with your doctor. Orphanet is the French National Institute for Health and Medical Research and the Health Programme of the European Union.
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