Toxic shock syndrome (TSS) is caused by staphylococcal or streptococcal exotoxins. Symptoms include high fever, hypotension, diffuse erythematous rash, and multiple organ dysfunction, which may rapidly progress to severe and intractable shock. Diagnosis is made clinically and by isolating the organism. Treatment includes antibiotics, intensive support, and immune globulin.
TSS is caused by exotoxin-producing cocci. Strains of phage-group 1 Staphylococcus aureus elaborate the TSS toxin-1 (TSST-1) or related exotoxins; certain strains of Streptococcus pyogenes produce at least 2 exotoxins.
Staphylococcal toxic shock
Women who have preexisting staphylococcal colonization of the vagina and who use tampons or other devices inserted and left in the vagina (eg, contraceptive sponges, diaphragms) are at highest risk. Mechanical or chemical factors related to tampon use probably enhance production of the exotoxin or facilitate its entry into the bloodstream through a mucosal break or via the uterus. Estimates made from small series suggest about 3 cases/100,000 menstruating women still occur, and cases are still reported in women who do not use tampons and in women who have infection after childbirth, abortion, or surgery. About 15% of cases occur postpartum or as a complication of postoperative staphylococcal wound infections, which frequently appear insignificant. Cases have also been reported in both men and women with any type of S. aureus infection.
Mortality from staphylococcal TSS is < 3%. Recurrences are common among women who continue to use tampons during the first 4 mo after an episode.
Streptococcal toxic shock
The syndrome is similar to that caused by S. aureus, but mortality is higher (20 to 60%) despite aggressive therapy. In addition, about 50% of patients have S. pyogenes bacteremia, and 50% have necrotizing fasciitis (neither is common with staphylococcal TSS). Patients are usually otherwise healthy children or adults. Primary infections in skin and soft tissue are more common than in other sites. In contrast to staphylococcal TSS, streptococcal TSS is more likely to cause acute respiratory distress syndrome (ARDS) and less likely to cause a typical cutaneous reaction.
S. pyogenes TSS is defined as any group A β-hemolytic streptococci (GABHS) infection associated with shock and organ failure. Risk factors for GABHS TSS include minor trauma, surgical procedures, viral infections (eg, varicella), and use of NSAIDs.
Symptoms and Signs
Onset is sudden, with fever (39° to 40.5°C, which remains elevated), hypotension, a diffuse macular erythroderma, and involvement of at least 2 other organ systems.
Staphylococcal TSS is likely to cause vomiting, diarrhea, myalgia, elevated CK, mucositis, hepatic damage, thrombocytopenia, and confusion. The staphylococcal TSS rash is more likely to desquamate, particularly on the palms and soles, between 3 and 7 days after onset.
Streptococcal TSS commonly causes ARDS (in about 55% of patients), coagulopathy, and hepatic damage and is more likely to cause fever, malaise, and severe pain at the site of a soft-tissue infection.
Renal impairment is frequent and common to both. The syndrome may progress within 48 h to syncope, shock, and death. Less severe cases of staphylococcal TSS are fairly common.
Diagnosis is made clinically and by isolating the organism from blood cultures (for Streptococcus) or from the local site. TSS resembles Kawasaki disease, but Kawasaki disease usually occurs in children < 5 yr of age and does not cause shock, azotemia, or thrombocytopenia; the rash is maculopapular. Other disorders to be considered are scarlet fever, Reye syndrome, staphylococcal scalded skin syndrome, meningococcemia, Rocky Mountain spotted fever, leptospirosis, and viral exanthematous diseases. These disorders are ruled out by specific clinical differences, cultures, and serologic tests.
Specimens for culture should be taken from any lesions, the nose (for staphylococci), throat (for streptococci), vagina (for both), and blood. MRI or CT of soft tissue is helpful in localizing sites of infection. Continuous monitoring of renal, hepatic, bone marrow, and cardiopulmonary function is necessary.
Patients suspected of having TSS should be hospitalized immediately and treated intensively. Tampons, diaphragms, and other foreign bodies should be removed at once. Suspected primary sites should be decontaminated thoroughly. Decontamination includes reinspection and irrigation of surgical wounds, even if they appear healthy; repeated debridement of devitalized tissues; and irrigation of potential naturally colonized sites (sinuses, vagina). Fluids and electrolytes are replaced to prevent or treat hypovolemia, hypotension, and shock. Because fluid loss into tissues can occur throughout the body (because of systemic capillary leak syndrome and hypoalbuminemia), shock may be profound and resistant. Aggressive fluid resuscitation and circulatory, ventilatory, and/or hemodialysis support are sometimes required.
Obvious infections should be treated. Pending culture results, vancomycin, daptomycin, linezolid, or ceftaroline—empiric choices that cover the most likely etiologic organisms—should be used. If S. pyogenes is isolated, a β-lactam (eg, penicillin) plus clindamycin (900 mg IV q 8 h) continued for 14 days is the most effective antibiotic treatment. If methicillin-resistant S. aureus (MRSA) is suspected or confirmed, vancomycin, daptomycin, linezolid, or tigecycline is indicated (see Gram-Positive Cocci: Antibiotic resistance). Antibiotics given during the acute illness may eradicate pathogen foci and prevent recurrences. Passive immunization to TSS toxins with IV immune globulin (2 g/kg, followed by 0.4 g/kg daily for up to 5 days) has been helpful in severe cases of both types of TSS and lasts for weeks, but the disease may not induce active immunity, so recurrences are possible.
If a test for seroconversion of the serum antibody responses to TSST-1 in acute- and convalescent-phase paired sera is negative, women who have had staphylococcal TSS should probably refrain from using tampons and cervical caps, plugs, and diaphragms. Advising all women, regardless of TSST-1 antibody status, to change tampons frequently or use napkins instead and to avoid hyperabsorbent tampons seems prudent.
Last full review/revision April 2013 by Larry M. Bush, MD; Maria T. Perez, MD