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Toxic Shock Syndrome, Emergency Medicine


Basics


Description


  • Toxic shock syndrome (TSS) is a severe, acute life-threatening illness
  • Etiologic organisms:
    • Staphylococcus aureus, more common (TSS)
    • Group A streptococcus or GAS, less common (Streptococcal TSS or STSS)
  • S. aureus produce structurally similar toxins:
    • Toxic shock syndrome toxin (TSST-1)
    • Enterotoxin B (SEB)
    • Enterotoxin C (SEC)
  • GAS pyrogenic exotoxins:
    • Exotoxin A (SPEA)
    • Exotoxin B (SPEB)
  • Exotoxins act as superantigens causing overwhelming immune response:
    • Massive cytokine production
    • Induce fever directly at the hypothalamus or indirectly via interleukin-1 (IL-1) and tumor necrosis factor (TNF) production
    • Enhance delayed hypersensitivity
    • Suppress neutrophil migration and immunoglobulin
    • Enhance host susceptibility to endotoxins
  • Massive vasodilation occurs
    • Serum protein and fluid shifts leading to hypotension

Etiology


  • Initial cases described in young healthy menstruating females due to highly absorbent tampons
    • Changes made in tampon composition to decrease incidence
  • Approximately one-half of reported TSS cases are nonmenstrual:
    • Surgical wounds
    • Postpartum wound infections
    • Mastitis
    • Septorhinoplasty
    • Sinusitis
    • Osteomyelitis
    • Arthritis
    • Burns
    • Nasal packing (nasal tampons)
    • Cutaneous and subcutaneous lesions
  • Nonmenstrual cases predominantly due to SEB and SEC producing S. aureus
  • 30 " “50% of healthy adults and children carry S. aureus in the nasal vestibule, vagina, rectum and/or on the skin
  • GAS infections often begin within 24 " “72 hr at the site of minor trauma, often without a visible in skin
  • Despite increased incidence of Methicillin-resistant S. aureus (MRSA) infections, a recent study reported MRSA only accounting for 7% of cases

Diagnosis


Signs and Symptoms


TSS Criteria for Diagnosis
  • CDC case definition:
    • Fever >38.9 ‚ °C (102 ‚ °F)
    • Hypotension (systolic BP <90 mm Hg) or shock
    • Diffuse, blanching nonpruritic macular erythroderma rash
    • Subsequent desquamation 1 " “2 wk after the onset of illness (particularly involving palms and soles)
    • Multisystem involvement " ”at least 3 of the following should be present:
      • GI: Profuse diarrhea or vomiting at onset of illness
      • Musculoskeletal: Severe myalgias or greater than a 2-fold increase in creatine phosphokinase (CPK)
      • Mucosal inflammation: Conjunctival, vaginal, or pharyngeal hyperemia
      • Renal: Increase in BUN or creatinine >2 times normal upper limit or sterile pyuria without evidence of infection
      • Hepatic: Total bilirubin or transaminases >2 times normal upper limit
      • Hematologic: Thrombocytopenia <100,000/mm3
      • CNS: Disorientation, confusion, or hallucinations
    • Negative results on the following tests, if obtained: Throat, or CSF cultures, rise in titer to Rocky Mountain spotted fever (RMSF), leptospirosis, or rubeola

Streptococcal TSS (STSS) Criteria for Diagnosis
  • CDC case definition:
    • Isolation of GAS from a normally sterile site
    • Hypotension
    • Plus 2 or more of the following:
      • Renal impairment (creatinine >2)
      • Coagulopathy
      • Liver involvement (>2 times the upper limit of normal for transaminases or bilirubin)
      • ARDS
      • Erythematous macular rash, may desquamate
      • Soft tissue necrosis

Other
  • Tachycardia frequently present
  • Can rapidly progress to multisystem dysfunction (ARDS or DIC)
  • STSS often presents with diffuse or localized pain " ”abrupt in onset and severe
  • Pain precedes physical findings
  • Nearly 80% of patients with STSS have clinical signs of soft tissue infection

Essential Workup


  • Clinical diagnosis using diagnostic criteria in the absence of other attributable illness
  • Thorough history and physical exam

Diagnosis Tests & Interpretation


Lab
  • CBC:
    • Leukocytosis or leukopenia, marked bandemia common
  • Electrolytes, BUN, creatinine, glucose:
    • Elevated BUN and creatinine common
  • Calcium, magnesium:
    • Hypocalcemia/hypomagnesemia often present
  • Urinalysis:
    • Normal or sterile pyuria without evidence of infection
  • CPK:
    • 2-fold increase
  • Hepatic function:
    • Elevated total bilirubin, AST, ALT
  • Prothrombin time (PT), partial thromboplastin time (PTT), platelets:
    • Thrombocytopenia <100,000 platelets/mm3
  • Culture the site of injury/infection if possible
  • Blood, urine, throat, and CSF cultures as indicated:
    • The case definition does not require a positive blood culture for S. aureus, but does for Streptococcus organisms.
  • Serology for RMSF, rubeola, and leptospirosis
  • Hepatitis B surface antigen

Imaging
  • Chest x-ray " “ to rule out other sources of systemic illness
  • Consider x-ray or CT scan if localized pain is concerning for abscess or necrotizing infection

Differential Diagnosis


  • Staphylococcal scalded skin syndrome:
    • In children <5 yr of age
    • Initial macular rash followed by the formation of ill-defined bullae that can be rubbed off revealing a shiny, moist epidermis (positive Nikolsky sign)
  • Scarlet fever:
    • Preceding streptococcal pharyngitis
    • Rash begins on the upper chest, neck, and back spreading to the remainder of the trunk, sparing the palms and soles
    • Hypotension absent
  • Kawasaki disease:
    • Fever, conjunctival hyperemia, and erythema of the mucous membranes
    • Not associated with renal failure, hypotension, or thrombocytopenia
  • Stevens " “Johnson syndrome:
    • Severe multisystem involvement
    • Mucosal involvement of the mouth, conjunctivae, vagina, anus, and urethral meatus
  • Leptospirosis:
    • Transmitted through contact with infected animals
    • Fever, headache, severe myalgias, and conjunctival suffusion
    • Truncal rash that only desquamates in children
  • RMSF:
    • Rash is pink and macular, beginning on the wrists, palms, ankles, and soles spreading to the trunk and face
    • Petechiae appear after 4 days
  • Meningococcemia:
    • Meningismus present
    • Rash is petechial

Treatment


Pre-Hospital


  • ABCs
  • IV access
  • IV fluids for hypotension

Initial Stabilization


  • Again, ABCs
  • Aggressive management of circulatory shock
    • IV fluids
    • Pressors

Ed Treatment/Procedures


Hypotension
  • Aggressive fluid replacement
    • The 1st 24 hr may require 4 " “20 L of crystalloid and/or fresh frozen plasma (colloid)
    • Caution: Large amounts of IV fluids and pressors used to treat refractory hypotension can result in rapid onset pulmonary edema
    • Pressors (dopamine/norepinephrine) if fluid correction fails to restore normal arterial pressure

Infection Management
  • Search for and treat the focus of infection
  • Remove the source of infection (e.g., tampon, nasal or wound packing)
  • Early surgical/gynecologic consultation if drainage or debridement of infectious sites necessary
  • Antibiotics
    • Recommended to reduce recurrence, but have not been shown to alter the course of the initial infection
    • Clindamycin and linezolid are potent suppressers of bacterial toxin synthesis
    • Clindamycin or linezolid+vancomycin for TSS
    • Linezolid+vancomycin for TSS with extensive infection
    • If TSS due to known methicillin-susceptible S. aureus then clindamycin +oxacillin or nafcillin
    • Clindamycin + imipenem or meropenem or ticarcillin " “clavulanate or piperacillin " “tazobactam for STSS
  • IV immunoglobulin (IVIG) treatment:
    • May be efficacious in streptococcal toxic shock, but no controlled trials have proven efficacy in staphylococcal TSS.
    • May initiate if no response to fluids, pressors, and antibiotics in patients with pulmonary edema and hypotension

Medication


  • Clindamycin: 600 " “900 mg (peds: 20 " “40 mg/kg/24 h) IV q6 " “8h
  • Dopamine: 2 " “20 Ž ¼g/kg/min IV, titrate to BP
  • Linezolid: 600 mg (peds: 10 mg/kg/12 h) IV q12h
  • Meropenem: 1 g IV q8h
  • Nafcillin: 1.5 g (peds: 100 mg/kg/24 h) IV q4h
  • Norepinephrine: 0.01 " “3 mcg/kg/min IV, titrate to BP
  • Oxacillin: 1 " “2 g (peds: 50 " “100 mg/kg/24 h) IV q4h
  • Piperacillin " “tazobactam: 4.5 g q6h
  • Ticarcillin " “clavulanate: 3.1 g q4h
  • Vancomycin: 30 mg/kg QD IV div. in 2 doses (peds: 40 mg/kg QD IV div. in 4 doses)
  • Staphylococcal TSS: IVIG, 400 mg/kg over several hours
  • Streptococcal TSS: IVIG 1 g/kg on day 1 then 0.5 g/kg on days 2 and 3

Follow-Up


Disposition


Admission Criteria
  • Cases necessitateadmission
  • ICU admission for critically ill or those in shock

Discharge Criteria
None ‚  
Issues for Referral
Early surgical/gynecologic consultation if drainage or debridement is needed ‚  

Follow-Up


Follow-Up Recommendations


  • Patients who are bacteremic are treated for a minimum of 14 days:
    • Depending on the clinical course
    • Continue treatment for 14 days from the last positive culture.
  • Screening for S. aureus nasal carriage in patient with S. aureus TSS and eradication of the carrier state with mupirocin

Pearls and Pitfalls


  • Consider the diagnoses of staphylococcal TSS and GAS TSS
  • Ensure adequate supportive care for hypotension in TSS
  • Prompt and aggressive exploration and debridement of suspected deep-seated infection
  • Empiric broad-spectrum antibiotics including clindamycin or linezolid is recommended

Additional Reading


  • DeVries ‚  AS, Lesher ‚  L, Schlievert ‚  PM, et al. Staphylococcal toxic shock syndrome 2000 " “2006: Epidemiology, clinical features, and molecular characteristics. PLoS One.  2011;6(8):e22997.
  • Darenberg ‚  J, Ihendyane ‚  N, Sj ƒ ¶lin ‚  J, et al. Intravenous immunoglobulin G therapy in streptococcal toxic shock syndrome: A European randomized, double-blind, placebo-controlled trial. Clin Infect Dis.  2003;37:333 " “340.
  • Lappin ‚  E, Ferguson ‚  AJ: Gram-positive toxic shock syndromes. Lancet Infect Dis.  2009;281 " “290.
  • O 'Brien ‚  KL, Beall ‚  B, Barrett ‚  NL, et al. Epidemiology of invasive group A streptococcus disease in the United States, 1995 " “1999. Clin Infect Dis.  2002;35:268 " “276.
  • Stevens ‚  DL, Wallace ‚  RJ, Hamilton ‚  SM, et al. Successful treatment of staphylococcal toxic shock syndrome with linezolid: A case report and in vitro evaluation of the production of toxic shock syndrome toxin type 1 in the presence of antibiotics. Clin Infect Dis.  2006;42:729 " “730.

See Also (Topic, Algorithm, Electronic Media Element)


  • Streptococcal Infections
  • Kawasaki Disease
  • Meningococcemia
  • Leptospirosis

Codes


ICD9


040.82 Toxic shock syndrome ‚  

ICD10


A48.3 Toxic shock syndrome ‚  

SNOMED


  • 18504008 Toxic shock syndrome (disorder)
  • 240450004 Staphylococcal toxic shock syndrome (disorder)
  • 240451000 Streptococcal toxic shock syndrome (disorder)
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