|55 Kenosia Avenue
Danbury, CT 06810
Toll Free: 1.800.999.6673
The National Organization for Rare Disorders (NORD) web site, its databases, and the contents thereof are copyrighted by NORD. No part of the NORD web site, databases, or the contents may be copied in any way, including but not limited to the following: electronically downloading, storing in a retrieval system, or redistributing for any commercial purposes without the express written permission of NORD. Permission is hereby granted to print one hard copy of the information on an individual disease for your personal use, provided that such content is in no way modified, and the credit for the source (NORD) and NORD’s copyright notice are included on the printed copy. Any other electronic reproduction or other printed versions is strictly prohibited.
The information in NORD’s Rare Disease Database is for educational purposes only. It should never be used for diagnostic or treatment purposes. If you have questions regarding a medical condition, always seek the advice of your physician or other qualified health professional. NORD’s reports provide a brief overview of rare diseases. For more specific information, we encourage you to contact your personal physician or the agencies listed as “Resources” on this report.
Copyright 1986, 1999, 2007, 2009
Legionnaires' Disease is recognized as an acute respiratory pneumonia caused by the aerobic gram-negative microorganism, Legionella pneumophila, and other species. This microorganism may also affect other body systems. Afflicted patients may have pulmonary (lung and bronchi), gastrointestinal tract, and central nervous system complications. Renal insufficiency may occur occasionally and can be severe enough to require dialysis.
The primary symptoms associated with Legionnaires' Disease appear to be pneumonia including a shaking chill, sharp pain in the involved side of the chest, cough with sputum or phlegm production, fever of up to 105º F, and, in some cases, rapid and painful respiration. Abdominal pain, diarrhea, neurological signs such as headache, confusion, lethargy or agitation may also be present. Laboratory data may include an abnormal liver function test, low phosphorus in the blood (hypophosphatemia), blood in the urine (hematuria), and low blood sodium (hyponatremia).
The term "legionellosis" refers to disease caused by infection with bacteria belonging to the Legionella family. Legionnaires' Disease is characterized by the development of pneumonia due to infection with the bacterium known as Legionella pneumophila. Another infectious disease, Pontiac Fever, is an acute, flulike (febrile), self-limited disease that is caused by infection with bacteria belonging to the same Legionella family. (For further information on Pontiac Fever, please see the "Related Disorders" section of this report below.)
Legionella pneumophila (L. pneumophila) is a gram-negative bacterium that thrives in the presence of oxygen (aerobic bacterium). Bacteria may be considered "gram negative" or "gram positive," depending upon the results of "Gram's stain," a testing method in which bacteria are stained with various solutions to help identify and classify the microorganisms. Such staining may be essential in identifying a specific bacterium responsible for an infectious disease and determining appropriate, effective treatments.
The L. pneumophila bacterium most easily reproduces in moist, warm areas. For example, in some outbreaks of Legionnaires' Disease, the source of infection was traced to air-conditioning or water systems. Infection with L. pneumophila results from inhalation or ingestion of contaminated water droplets.
Legionnaires' Disease was initially recognized in 1976, when an outbreak of a form of pneumonia affected members attending an American Legion Convention at a hotel in Philadelphia. The bacterium responsible for the disease was later identified and named Legionella pneumophila. Researchers subsequently determined that an earlier epidemic of the disease had occurred in Minnesota in 1957.
It is estimated that as many as 10,000 to 15,000 individuals are affected by Legionnaires' Disease each year in the United States. In addition, studies suggest that the disease may account for approximately three to 15 percent of all cases of community-acquired pneumonia. Researchers also indicate that an unknown number of individuals may be infected by the L. pneumophila bacterium yet experience mild illness or develop no apparent symptoms (asymptomatic).
Certain individuals may have an increased predisposition for the disease. Risk factors may include advanced age, cigarette smoking, chronic lung disease, and a suppressed immune system (immunosuppression). Reports suggest that elderly males may be most likely to develop the disease. In addition, certain individuals may be predisposed to acquiring the infection during hospitalization (nosocomial infection), such as those who undergo surgery, particularly transplants, or infants with lung disease or immunosuppression.
As discussed above, Legionnaires' Disease may occur in outbreaks. However, the disease usually occurs in single, isolated cases. Outbreaks, though typically recognized during the summer or fall, may develop at any time of the year.
Like Legionnaires' Disease, Pontiac Fever is caused by infection with bacteria belonging to the Legionella family. Associated symptoms usually include a general feeling of ill health (malaise), fatigue, fever, chills, muscle aches (myalgia), and headache. Some individuals with Pontiac Fever may also develop nausea, cough, joint aches (arthalgia), diarrhea, and abdominal pain. The pneumonia seen in Legionnaires' Disease is not present. Affected individuals usually recover without treatment (self-limiting illness) in a few days. In those who develop Pontiac Fever, the time between initial infection and symptom onset (incubation period) is typically about one to two days. In contrast, the incubation period for Legionnaires' Disease usually ranges from approximately two to 10 days.
Patients afflicted with Legionnaires' Disease may develop respiratory compromise requiring artificial ventilation and positive end expiratory pressure respirators to maintain adequate oxygenation. Erythromycin is the drug treatment of choice. In more severe cases, rifampin may be used in conjunction with erythromycin. Tetracycline may be substituted if the patient is allergic to erythromycin.
While the outbreak of Legionnaires' Disease has only occasionally been associated with a contaminated water system, appropriate treatment of the water system is recommended if this is the case.
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
For information about clinical trials sponsored by private sources, contact:
1600 Clifton Road NE
Atlanta, GA 30333
Phone #: 404-639-3534
800 #: 800-232-4636
Home page: http://www.cdc.gov/
PO Box 8126
Gaithersburg, MD 20898-8126
Phone #: 301-251-4925
800 #: 888-205-2311
Home page: http://rarediseases.info.nih.gov/GARD/
NIAID Office of Communications and Government Relations
5601 Fishers Lane, MSC 9806
Bethesda, MD 20892-9806
Phone #: 301-496-5717
800 #: 866-284-4107
Home page: http://www.niaid.nih.gov/
Avenue Appia 20
Geneva 27, 1211 Switzerland
Phone #: 412-279-12111
800 #: --
Home page: http://www.who.int/en/
Harrison's Principles of Internal Medicine, 14th Ed.: Anthony S. Fauci et al., Eds.: McGraw-Hill Companies, Inc., 1998. Pp. 928-33.
Cecil Textbook of Medicine, 18th ed.: James B. Wyngaarden, and Lloyd H. Smith, Jr., Eds.: W.B. Saunders Co., 1988. P. 1570.
The Merck Manual 15th ed: R. Berkow, et al: eds; Merck, Sharp & Dohme Research Laboratories, 1987. P. 665.
More Than 10 Years of Unrecognized Nososomial Transmission of Legionnaires' Disease Among Transplant Patients. J.L. Kool et al.; Infect Control Hosp Epidemiol (Dec 1998; 19(12)). Pp. 898-904. Comment in: Infect Control Hosp Epidemiol (Dec 1998; 19(12)). Pp. 893-97.
A Survey of Methods Used to Detect Nosocomial Legionellosis Among Participants in the National Nosocomial Infections Surveillance System. A.E. Fiore et al.; Infect Control Hosp Epidemiol (Jun 1999; 20(6)). Pp. 412-16.
Detecting Legionellosis by Unselected Culture of Respiratory Tract Secretions and Developing Links to Hospital Water Strains. J.R. Kohler et al.; J Hosp Infect (Apr 1999; 41(4)). Pp. 301-11.
Legionella Epidemic in the Netherlands. I.M. Hoepelman; Ned Tijdsch Geneeskd (Jun 5 1999; 143(23)). Pp. 1192-96.
Pontiac Fever at a Sewage Treatment Plant in the Food Industry. P. Gregersen et al.; Scand J Work Environ Health (Jun 1999; 25(3). Pp. 291-95.
FROM THE INTERNET
Report last updated: 2009/04/10 00:00:00 GMT+0