[Lethal, non-menstrual toxic shock syndrome associated with Staphylococcus aureus sepsis]

We report a rare case of non-menstrual toxic shock syndrome (TSS) in the course of Staphylococcus aureus sepsis in a 31-year-old primigravida who developed high fever and severe pulmonary and cardiovascular failure within a few hours at the end of the 29th week of a twin pregnancy. Mechanical ventilation was necessary due to signs of adult respiratory distress syndrome (ARDS) and catecholamines were needed to maintain a somewhat adequate blood pressure. A forceps delivery was performed immediately. Postoperatively, the patient was brought to the intensive care unit (ICU) due to the suspicion of severe septic shock. In addition to the extreme cardiovascular instability and massive disturbance of pulmonary gas exchange, the clinical picture was characterised by a disseminated intravascular coagulopathy (DIC) with marked petechial bleeding and ecchymoses on all extremities. Moreover, a confluent, spotty exanthem of the trunk and extremities could be seen. Despite all therapeutic efforts, the patient died within a few hours after admission to the ICU with signs of multiorgan failure. Post-mortem, multiple staphylococcal abscesses were found in the kidneys, liver, and uterus. Moreover, acute ulcerous endocarditis of the mitral valve and septic myocardial foci with myocarditis were seen. The Staph. aureus strain isolated from the blood cultures was shown to produce TSS toxin 1 (TSST-1) and enterotoxin B. In summary, the clinical picture can be interpreted as severe staphylococcal sepsis complicated by TSS. TSS is a specific type of infectious disease, occurring mainly in young women during the menstrual period (80%-90%), but it has also been reported in non-menstrual cases (10%-20%). It is characterised by sudden-onset high fever, hypotension, rash, mucosal hyperaemia, and various additional symptoms such as myalgia, vomiting, and diarrhoea. The clinical course depends on the extent of the organ failure due to decreased tissue perfusion during hypotension. Severe cases are accompanied by multiple organ-system failure including impaired renal function, which is reversible in nearly all cases. Respiratory failure ranges from interstitial and alveolar aedema to ARDS in 10% of cases; severe DIC is seen in 10%-15%. Another severe clinical complication is cardiac insufficiency. The etiology of TSS is based on a localized or, rarely, systemic infection with certain Staph. aureus strains that are capable of producing toxins, the most important one being TSST-1. Staph. aureus strains can also produce various other enterotoxins that may be involved in the pathogenesis of TSS. The pathogenetic importance of the toxins is supported by the antibody titers in TSS patients: more than 80% of healthy adults show high levels of antibody titers, whereas 90% of TSS patients exhibit low levels in the acute phase followed by a significant increase during convalescence. It is not clear whether the toxins cause TSS by a direct effect or by release of mediators due to their function as superantigens. The clinical characteristics of non-menstrual TSS are identical to those of menstrual TSS, but it can occur in many clinical settings in both sexes at any age. Severe clinical courses are more frequent in non-menstrual TSS: the mortality is about 8%-11% in non-menstrual TSS compared to 2%-5% in menstrual TSS. The diagnosis is based mainly on clinical signs and the isolation of toxin-producing Staph. aureus strains. Besides antibiotic therapy, treatment is primarily directed to the correction of hypotension and additional organ-system failure. Other therapeutic measures such as the elimination of toxins by plasma separation or the administration of antibodies or gamma-globulins are subjects of investigation with no general recommendations at this time.