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Epidemiology, clinical manifestations, and diagnosis of streptococcal toxic shock syndrome

Dennis L Stevens, MD, PhD
Section Editor
Daniel J Sexton, MD
Deputy Editor
Elinor L Baron, MD, DTMH


Streptococcal toxic shock syndrome (TSS) is a clinical illness characterized by shock and multiorgan failure. Streptococcal TSS consists of isolation of group A Streptococcus (GAS) from a normally sterile body site, together with hypotension, tachycardia, and evidence of organ failure, such as acute respiratory distress syndrome, coagulopathy, liver failure, or renal failure [1]. It occurs as a result of capillary leak and tissue damage due to release of inflammatory cytokines induced by streptococcal toxins.

Streptococcal TSS occurs most frequently in the setting of invasive infection due to group A Streptococcus (Streptococcus pyogenes). GAS typically causes pharyngitis or skin and soft tissue infection; these are generally responsive to appropriate antibiotic therapy, and patients do not develop streptococcal TSS [2]. Less commonly, GAS causes invasive disease such as necrotizing infection of the skin and fascia, gangrenous myositis, bacteremia, or pneumonia [3], and these types of infection are complicated by TSS in approximately one-third of cases [4].

The epidemiology, clinical manifestations, and diagnosis of streptococcal toxic shock syndrome will be reviewed here. The treatment of streptococcal toxic shock syndrome is discussed separately. (See "Treatment of streptococcal toxic shock syndrome".)


Invasive infections associated with group A streptococcal (GAS) toxic shock syndrome (TSS) have been reported with increasing frequency, predominantly from North America and Europe [1,4-13]. There are an estimated 3.5 cases of invasive GAS per 100,000 persons, with a case-fatality rate of 30 to 60 percent [3,13-16]. One study noted an increase in the incidence of invasive group A streptococcal disease in Utah between 2002 and 2010, from 3.5 to 9.8 cases per 100,000 [17]. Up to one-third of patients with invasive GAS disease developed TSS in reported case series [15,18]. The rate of TSS among patients with necrotizing fasciitis is approximately 50 percent [3,19].

Risk factors — GAS TSS occurs among all age groups. Most patients are not immunosuppressed; diabetes and alcoholism are risk factors described in some studies [7,20-25]. Among reports of invasive GAS infections associated with bacteremia in the late 1980s, most patients were either <10 or >60 years of age and had underlying diseases, such as cancer, renal failure, leukemia, and severe burns, or were receiving corticosteroids or other immunosuppressive drugs [22-24]. Invasive infection remains highest in patients >50 years of age.

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Literature review current through: Nov 2017. | This topic last updated: Dec 05, 2017.
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