NORD gratefully acknowledges Pierre E. Rollin, MD, Viral Special Pathogens Branch, Centers for Disease Control and Prevention, for assistance in the preparation of this report.
Hantavirus pulmonary syndrome (HPS) is an infectious disease caused by hantaviruses (Sin Nombre hantavirus in most of the US). Transmission occurs when direct or indirect (airborne) contact is made with the saliva or waste products of rodents that carry the virus, most commonly the deer mouse (Peromyscus maniculatus). Initial symptoms may include fever, muscle aches (myalgias), headache, cough, and/or difficulty breathing. Symptoms progress rapidly, and abnormally low blood pressure (hypotension), shock, and/or respiratory failure may occur.
The initial symptoms of HPS most commonly include fever, muscle aches (myalgias), headache, and/or cough. Chills, abdominal pain, diarrhea, and/or a sense of overall discomfort (malaise) may be present. Other symptoms usually include shortness of breath, rapid breathing (tachypnea), rapid heartbeat (tachycardia), dizziness, and sometimes joint pain (arthralgia), back and/or chest pain, and/or sweating.
Shortly after the initial symptoms of HPS appear excess fluid may accumulate in the lungs (pulmonary edema), beginning in the air spaces in the lungs (interstitial edema). Fluid may then fill the pockets in the lung tissue (alveolar edema) of both lungs (bilaterally) and cause difficulty in breathing and abnormally low levels of oxygen in the blood (hypoxemia). Accumulation of tissue and cells not normally found in the lungs (infiltrates) may also occur. The disease progresses rapidly and may cause abnormally low blood pressure (hypotension), shock, and/or respiratory distress.
HPS in the US is mostly caused by Sin Nombre Hantavirus, a newly identified virus within the Bunyaviridae family. The virus is carried by the deer mouse (Peromyscus maniculatus). The deer mouse can be found in most parts of the United States, except the southeast. Not all deer mice are infected with Sin Nombre hantavirus, and those that do carry the virus do not appear to be affected by any associated disease.
Three other species of hantavirus are causing human disease in the US: New York Virus identified in the white-footed mouse (eastern US), Black Creek Canal virus in cotton rat in Florida, and Bayou virus in Rice Field Mice in Texas and Louisiana. In addition, many other hantaviruses have been found in other rodent species in several States but were never detected in humans. In South America, numerous hantaviruses have been found responsible of human diseases. Andes virus is the cause of very severe HPS in Argentina and Chile.
Sin Nombre hantavirus is transmitted to humans when they come in direct or indirect (airborne) contact with waste products or saliva from an infected rodent. Respiratory transmission, thought to be the most frequent mode of transmission of Sin Nombre hantavirus, occurs when an individual inhales airborne particles of dust or dried particles that carry saliva or waste products from an infected rodent. Infectious virus particles could also penetrate through the mucosa.
HPS appears to affect males and females in equal numbers. Approximately half of the cases reported in the medical literature have affected Native Americans, and the majority of the remaining reported cases affected Caucasians. The population affected by HPS appears to be related to geographic location and exposure to rodent droppings as opposed to ethnic background. Because many of the documented cases have occurred in the southwestern United States, a high percentage of the initially affected individuals were Native Americans. As the virus was found in others parts of the US, many others of varied ethnic backgrounds have been affected throughout the United States.
It is important to avoid areas where deer mice leave their droppings, such as storage sheds, basements, and wood piles. When exposed to mouse droppings, an individual should wear a face mask that covers both nose and mouth, as well as rubber gloves. The area should be sanitized with disinfectant to avoid aerosolization of potentially infected dust. People who exhibit flu-like symptoms after exposure to mouse droppings should be taken to a hospital immediately, because this disorder progresses over a matter of hours, and every hour is crucial.
The diagnosis of HPS depends on several factors, including the symptoms of the affected individual, a history of contact with rodents (especially deer mice) or exposure to areas where rodents may live, the lack of any alternative diagnosis, and/or laboratory tests that show characteristic changes. Because the symptoms of HPS are rapidly progressive, immediate aggressive care is indicated if HPS is suspected.
Characteristic laboratory blood test results for people with HPS may show abnormally enlarged white blood cells (atypical lymphocytes), a platelet count that is lower than normal (thrombocytopenia) or dropping, and/or a higher than normal white blood cell count. Oxygen levels in blood and/or tissue may be extremely low (hypoxemia).
The diagnosis of HPS is confirmed when laboratory tests reveal the presence and/or increased levels of certain proteins (Hantavirus IgM and/or a rising IgG titer) in blood samples from affected individuals. A process called polymerase chain reaction (PCR) may be used to detect a hantavirus and identify which strain has caused the infection.
Treatment of HPS involves intensive care, including the monitoring of fluid balances, electrolyte balances, and blood pressure. Abnormally low levels of oxygen in the blood (hypoxemia) may require the administration of oxygen. Shock and low blood pressure (hypotension) associated with HPS may be treated with drugs (i.e., dopamine and norepinephrine) to increase blood flow and thus improve blood and oxygen delivery to organs. In severe patients, Extra Corporeal Membrane Oxygenation (ECMO) therapy could be offered in specialized centers and may be the only chance of survival.
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: prpl@cc.nih.gov
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/
Knust, Barbara and Rollin, Pierre E. Twenty-year summary of surveillance for human hantavirus infections, United States. Emerging Infectious Diseases. 2013;19(12):1934-1937.
Koster, Frederick and Mackow, Erich. Pathogenesis of the hantavirus pulmonary syndrome. Future Virology. 2012;7(1):41-51.
MacNeil, Adam; Ksiazek, Thomas G., and Rollin, Pierre E. Hantavirus pulmonary syndrome, United States, 1993-2009. Emerging Infectious Diseases. 2011;17(7):1195-1201
Wernly, J. A.; Dietl, C. A.; Tabe, C. E.; Pett, S. B.; Crandall, C.; Milligan, K., and Crowley, M. R. Extracorporeal membrane oxygenation support improves survival of patients with Hantavirus cardiopulmonary syndrome refractory to medical treatment. European Journal of Cardio-Thoracic Surgery. 2011;40(6):1334-1340.
Mills, James N.; Amman, Brian R., and Glass, Gregory E. Ecology of hantaviruses and their hosts in North America. Vector-Borne and Zoonotic Diseases. 2010;10(6):563-574.
Jonsson, Colleen B.; Hooper, Jay, and Mertz, Gregory. Treatment of hantavirus pulmonary syndrome. Antiviral Research. 2008;78 (1):162-169.
Mertz, Gregory J.; Hjelle, Brian; Crowley, Mark; Iwamoto, Gary; Tomicic, Vinko, and Vial, Pablo A. Diagnosis and treatment of new world hantavirus infections. Current Opinion in Infectious Diseases. 2006;19(5):437-442.
Koster, Frederick; Foucar, Kathryn; Hjelle, Brian; Scott, Amy; Chong, Yap-Yee; Larson, Richard, and McCabe, Melvina. Rapid presumptive diagnosis of hantavirus cardiopulmonary syndrome by peripheral blood smear review. American Journal of Clinical Pathology. 2001;116(5):665-672.
Ketai, Loren H.; Kelsey, Charles A.; Jordan, Kirk; Levin, David L.; Sullivan, Lisa M. ; Williamson, Michael R.; Wiest, Philip W., and Sell, James J. Distinguishing hantavirus pulmonary syndrome from acute respiratory distress syndrome by chest radiography: are there different radiographic manifestations of increased alveolar permeability? Journal of Thoracic Imaging. 1998;13(3):172-177.
Hjelle, Brian; Jenison, Steven; Torrez-Martinez, Norah; Herring, Bruce; Quan, Stella; Polito, Alan; Pichuantes, Sergio; Yamada, Takashi; Morris, Carol; Elgh, Fredrik; Lee, Ho-Wang; Artsob, Hartsob, and Dinello, Robert. Rapid and specific detection of Sin Nombre virus antibodies in patients with hantavirus pulmonary syndrome by a strip immunoblot assay suitable for field diagnosis. Journal of Clinical Microbiology. 1997;35(3):600-608.
Hallin, Gustav W.; Simpson, Steven Q.; Crowell, Richard E.; James, David S.; Koster, Frederick T.; Mertz, Gregory J., and Levy, Howard. Cardiopulmonary manifestations of hantavirus pulmonary symptoms. Critical Care Medicine. 1996;24(2):252-258.
Ksiazek, Thomas G.; Peters, Clarence J.; Rollin, Pierre E.; Zaki, Sherif; Nichol, Stuart T.; Spiropoulou, Christina; Morzunov, Sergey; Feldmann, Heinz; Sanchez, Anthony; Khan, Ali S.; Mahy, Brian W. J.; Wachsmuth, Kaye, and Butler, Jay C. Identification of a new North American hantavirus that causes acute pulmonary insufficiency. American Journal of Tropical Medicine and Hygiene. 1995;52(2):1017-1023.
Mills, James N.; Yates, Terry L.; Childs, James E.; Parmenter, Robert R.; Ksiazek, Thomas G.; Rollin, Pierre E., and Peters, Clarence J. Guidelines for working with rodents potentially infected with hantaviruses. Journal of Mammalogy. 1995;76(3):716-722.
Hjelle, Brian; Spiropoulou, Christina F.; Torrez-Martinez, Norah; Morzunov, Sergey; Peters, Clarence J., and Nichol, Stuart T. Detection of Muerto Canyon virus RNA in peripheral blood mononuclear cells from patients with hantavirus pulmonary syndrome. Journal of Infectious Diseases. 1994;170:1013-1017.
INTERNET
Hantavirus pulmonary syndrome. Mayo Clinic. http://www.mayoclinic.com/health/hantavirus-pulmonary-syndrome/DS00900. Updated January 2, 2014. Accessed June 20, 2016.
Cunha BA. Hantavirus Pulmonary Syndrome. Medscape. http://emedicine.medscape.com/article/236425-overview . Updated October 27, 2015. Accessed June 20, 2016.
Centers for Disease Control and Prevention. Hantavirus Pulmonary Syndrome (HPS). Last Update February 6, 2013. http://www.cdc.gov/hantavirus/hps/. Accessed June 20, 2016.
The information in NORD’s Rare Disease Database is for educational purposes only and is not intended to replace the advice of a physician or other qualified medical professional.
The content of the website and databases of the National Organization for Rare Disorders (NORD) is copyrighted and may not be reproduced, copied, downloaded or disseminated, in any way, for any commercial or public purpose, without prior written authorization and approval from NORD. Individuals may print one hard copy of an individual disease for personal use, provided that content is unmodified and includes NORD’s copyright.
National Organization for Rare Disorders (NORD)
55 Kenosia Ave., Danbury CT 06810 • (203)744-0100
