CLINICAL DESCRIPTION
Streptococcal toxic-shock syndrome (STSS) is a severe illness associated with invasive or noninvasive group A streptococcal (Streptococcus pyogenes) infection. Streptococcal toxic-shock syndrome may occur with infection at any site, but most often occurs in association with infection of a cutaneous lesion. Signs of toxicity and a rapidly progressive clinical course are characteristic, and the case-fatality rate may exceed 50%. See also Streptococcal Disease, Invasive, Group A.

CASE CLASSIFICATION

1. A. CONFIRMED
   
   An illness with the following clinical manifestations occurring within the first 48 hours of illness:
   
   
• Hypotension (systolic blood pressure less than or equal to 90 mmHg for adults or less than the fifth percentile by age for children aged less than 16 years)
  
  
• Multi-organ involvement characterized by two or more of the following:
  
  
  1. Renal impairment: Creatinine greater than or equal to 2 mg/ml (177 umol/L) for adults or greater than or equal to twice the upper limit of normal for age. In patients with preexisting renal disease, a greater then twofold elevation over the baseline level.
     
  2. Coagulopathy: Platelets less than or equal to 100,000/mm3 (less than or equal to 100 x 106/L) or disseminated intravascular coagulation, defined by prolonged clotting times, low fibrinogen level, and the presence of fibrin degradation products.
     
  3. Liver involvement: Alanine aminotransferase, aspartate aminotransferase, or total bilirubin levels greater than or equal to twice the upper limit of normal for the patient's age. In patients with preexisting liver disease, a greater than twofold increase over the baseline level.
     
  4. Acute respiratory distress syndrome: defined by acute onset of diffuse pulmonary infiltrates and hypoxemia in the absence of cardiac failure or by evidence of diffuse capillary leak manifested by acute onset of generalized edema, or pleural or peritoneal effusions with hypoalbuminemia.
     
  5. A generalized erythematous macular rash that may desquamate.
     
  6. Soft-tissue necrosis, including necrotizing fasciitis or myositis or gangrene.
  
  AND
  
  Isolation of Group A Streptococcus from a normally sterile site (e.g., blood or cerebrospinal fluid or, joint, pleural or pericardial fluid).
  
  
2. PROBABLE
   
   
• A case that meets clinical case definition criteria in the absence of another identified etiology for the illness and with isolation of group A Streptococcus from a nonsterile site.
  
  
3. POSSIBLE
   
   Not used

NOTE Isolates of Streptococcus pyogenes from blood or cerebrospinal fluid are to be submitted to the New Jersey Department of Health and Senior Services, Division of Public Health and Environmental Laboratories, P.O. Box 361, John Fitch Plaza, Trenton, New Jersey 08625-0361.