Toxic Shock Syndrome

Toxic Shock Syndrome

National Organization for Rare Disorders, Inc.

Important

It is possible that the main title of the report Toxic Shock Syndrome is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

  • TSS

Disorder Subdivisions

  • None

General Discussion

Toxic Shock Syndrome is a rare multisystem disease with many widespread symptoms. It is caused by a toxin that is produced and secreted by the bacterium Staphylococcus aureus. The symptoms of Toxic Shock Syndrome may include a sudden high fever, nausea, vomiting, diarrhea, abnormally low blood pressure (hypotension), and a characteristic skin rash that resemble a bad sunburn. Most cases of Toxic Shock Syndrome occur in menstruating females in association with the use of tampons. Other cases may occur in association with postoperative wound infections, nasal packing, or other factors.

Symptoms

The symptoms of Toxic Shock Syndrome begin suddenly and usually include high fever, headache, sore throat (pharyngitis), inflammation of the whites of the eyes (conjunctivitis), muscle aches and pain (myalgia), and/or certain digestive symptoms, such as nausea, vomiting, and profuse watery diarrhea. Involvement of the central nervous system is also common and may be characterized by listlessness, dizziness, confusion, and/or disorientation. A characteristic "sunburn-like" skin rash typically develops within a few hours of onset, with later scaling and peeling (desquamation) of skin, particularly of the palms and soles.



In severe cases, blood pressure may fall dangerously low (hypotension), with an inadequate blood supply to body tissues (shock). Additional complications may include kidney and/or liver failure, heart dysfunction, adult respiratory distress syndrome, and/or other abnormalities. Adult respiratory distress syndrome is characterized by shortness of breath (dyspnea), abnormally rapid breathing, and insufficient levels of oxygen in the circulating blood. Without early diagnosis and appropriate treatment of Toxic Shock Syndrome, potentially life-threatening complications may result.

Causes

Toxic Shock Syndrome (TSS) is a rare acute multisystemic disease caused by toxins (such as "toxic shock syndrome toxin-1" [TSST-1], enterotoxin B, enterotoxin C) produced by certain strains (particularly phage group I) of the bacterium Staphylococcus aureus (S. aureus). TSS is most common in menstruating women who use highly absorbent tampons. Evidence suggests that the prolonged use of tampons in the presence (i.e., colonization) of toxin-secreting S. aureus strains may promote increased production of the toxin, which may enter the bloodstream through the uterus or tiny cuts within the vaginal lining.



In addition, TSS may also occur in nonmenstruating women or those who do not use tampons. Such "nonmenstrual TSS" may occur due to vaginal colonization of toxin-secreting S. aureus strains and certain associated factors, such as the use of vaginal contraceptive devices (e.g., diaphragms, contraceptive sponges); vaginal infection; childbirth or the period (e.g., hours to several weeks) following delivery (postpartum state); or other factors. Nonmenstrual TSS may also occur in women, men, or children in association with post-surgical wounds; nasal packing; burns; certain skin infections; other inflammatory conditions, such as of the windpipe (tracheitis), the sinuses (sinusitis), the lungs (pneumonia), or the bone or bone marrow (osteomyelitis); the "flu" (influenza); or other factors.

Affected Populations

Toxic Shock Syndrome (TSS) was originally described as a disease entity in 1978, when the condition was reported in seven children from age eight to 17 years. In 1980 and 1981, a large number of TSS cases were recognized, primarily in young menstruating women who used tampons. This sudden increase was thought to be due to the introduction and availability of new, highly absorbent tampons.



Since the removal of super absorbent tampon varieties from the market and the introduction of federal regulations, the incidence of TSS has declined in women in the United States. (Incidence refers to the number of new cases of a particular disorder or condition during a specific period.) For example, in 1980 in the U.S., the incidence of TSS was approximately six per 100,000 women aged 19 to 44 years. In 1986 and later, the incidence decreased among this age group to one per 100,000. Other estimates suggest that TSS may currently occur in up to three per 100,000 menstruating women. Investigators indicate that menstruation-associated TSS most commonly occurs in young women aged 15 to 25 years who are using tampons.



As noted above, TSS may also occur in nonmenstruating women and in women who do not use tampons as well as in men and children. Nonmenstrual TSS more commonly affects females than males by a ratio of about three to one. (For further information, please see the "Causes" section above.)

Standard Therapies

Diagnosis

The diagnosis of Toxic Shock Syndrome (TSS) has been defined by the Centers for Disease Control clinical and laboratory criteria. TSS is considered probable if three or more criteria are met in association with peeling (desquamation) of affected skin or if five or more criteria are met in the absence of desquamation. The criteria include the following: fever; rash, with possible, subsequent peeling (desquamation), particularly on the palms and soles; low blood pressure (hypotension); the involvement of three or more organ systems (i.e., digestive [gastrointestinal], muscular, mucous membranes, kidneys, liver, blood, and/or brain and spinal cord [central nervous system]), and negative results of blood tests for the infectious diseases Rocky Mountain Spotted Fever, Leptospirosis, and Measles. Sometimes S. aureus may be isolated from the vagina or from localized (focal) wound sites and identified with the use of various laboratory techniques (e.g., bacterial cultures).



Treatment

Individuals with Toxic Shock Syndrome (TSS) should immediately be hospitalized for appropriate intensive treatment. Disease management requires aggressive intravenous fluid and electrolyte replacement and other supportive care measures as appropriate. Vaginal examination is also essential in women to remove a tampon or vaginal contraceptive device and to collect small tissue samples (cultures) from the vagina and neck of the uterus (cervix) for possible isolation of the S. aureus bacterium. In nonmenstrual TSS, measures may include obtaining cultures from and appropriately cleaning and treating focal wound sites.



In addition, in individuals with TSS, treatment typically includes the intravenous administration of beta-lactamase resistant antistaphylococcal antibiotics, such as nafcillin or oxacillin, possibly in combination with clindamycin. Other appropriate antibiotic regimens may be required in some cases. Such antibiotic therapy is important in preventing recurrences of TSS. In extremely severe cases, treatment may include infusion of concentrated preparations of certain antibodies (intravenous immune globulins).



To prevent menstrual TSS, tampons should only be used intermittently and replaced frequently. Super absorbent brands should also be avoided. Women who use tampons should be aware of the symptoms of TSS and advised to seek immediate medical attention if they occur. In addition, avoidance of tampons is important in helping to prevent a recurrence of menstrual TSS.



As noted above (see "Causes"), TSS has also been reported in association with the use of certain vaginal contraceptive devices. Women who use such contraceptive methods are cautioned to carefully follow all package instructions and to share any questions and concerns with their physicians and pharmacists to help minimize risk and ensure proper use.



Other treatment for this disorder is symptomatic and supportive.

Investigational Therapies

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

References

TEXTBOOKS

Mandell GL, et al., eds. Mandell, Douglas, and Bennett's Principles and Practice of Infectious Diseases. 5th ed. Philadelphia, PA: Churchill Livingstone; 2000:2080-82, 2110-13.



Wyngaarden JB, et al., eds. Cecil Textbook of Medicine. 19th ed. Philadelphia, PA: W.B. Saunders Company; 1992:780, 1629, 1650, 2301-02.



Beers MH, et al., eds. The Merck Manual. 17th ed. Whitehouse Station, NJ: Merck Research Laboratories; 1999:1149-50, 1152.



Behrman RE, et al., eds. Nelson Textbook of Pediatrics. 15th ed. Philadelphia, PA: W.B. Saunders Company; 1996:749-50.



JOURNAL ARTICLES

Baxter F, et al. Severe group A streptococcal infection and streptococcal toxic shock syndrome. Can J Anaesth. 2000;47:1129-40.



Givner LB. Invasive disease due to group A beta-hemolytic streptococci: continued occurrence in children in North Carolina. South Med J. 1998;91:333-37.



Fishman G, et al. Toxic shock syndrome. Harefuah. 1997;132:622-24, 679.



Fichtenbaum CJ, et al. Ehrlichiosis presenting as a life-threatening illness with features of the toxic shock syndrome. Am J Med. 1993;95:351-57.



Strausbaugh LJ. Toxic shock syndrome. Are you recognizing its changing presentations? Postgrad Med. 1993;94:107-08, 111-13, 117-18.



Kain KC, et al. Clinical spectrum of nonmenstrual toxic shock syndrome (TSS): comparison with menstrual TSS by multivariate discriminant analyses. Clin Infect Dis. 1993;16:100-06.



Panina-Bordignon P, et al. Identification of HLA-DR alpha chain residues critical for binding of the toxic shock syndrome toxin superantigen. J Exp Med. 1992;176:1779-84.



McCahill PD, et al. Toxic shock syndrome: a complication of continent urinary diversion. J Urol. 1992;147:681-82.



Schuchat A, et al. Toxic shock syndrome and tampons. Epidemiol Rev. 1991;13:99-112.



Givner LB, et al. Apparent increase in the incidence of invasive group A beta- hemolytic streptococcal disease in children. J Pediatr. 1991;18:341-46.



Todd JK. Therapy of toxic shock syndrome. Drugs. 1990;39:856-61.



Allen ST, et al. Toxic shock syndrome associated with use of latex nasal packing. Arch Intern Med. 1990;150:2587-88.



Bryner CL, et al. Recurrent toxic shock syndrome. Am Fam Physician. 1989;39:157-64.



Rizkallah MF, et al. Toxic shock syndrome caused by a strain of staphylococcus aureus that produces enterotoxin C but not toxic shock syndrome toxin-1. Am J Dis Child. 1989; 143:848-49.



Prechter GC, et al. Postinfluenza toxic shock syndrome. Chest. 1989;95:1153-54.



Kass EH, et al. On the pathogenesis of toxic shock syndrome. Rev Infect Dis. 1987;9:S482-89.



Osterholm, MT, et al. Tri-state toxic-shock syndrome study. I. Epidemiologic findings. J Infect Dis. 1982;145:431-40.



Davis JP, et al. Toxic-shock syndrome: epidemiologic features, recurrence, risk factors, and prevention. New Engl J Med. 1980;303:1429-35.



Todd J, et al. Toxic-shock syndrome associated with phage-group-I staphylococci. Lancet. 1978;2:1116-18.

Resources

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