The effect of C1-esterase inhibitor in definite and suspected streptococcal toxic shock syndrome. Report of seven patients

Objective: To evaluate the effect of adjunctive C1-esterase inhibitor subst itution therapy on clinical characteristics and outcome of patients with st reptococcal toxic shock syndrome (TSS). Design: Observational. Setting: Med izinische Poliklinik, University of Bonn, Germany. Patients: Seven patients with direct or indirect evidence of streptococcal TSS. Intervention: In ad dition to conventional and supportive therapy, all patients received 2-3 si ngle doses of C1-esterase inhibitor totaling 6,000-10,000 U within the firs t 24 h after admission. Measurements and results: All patients developed fu lminant septic shock, multiorgan failure and/or capillary leak syndrome and necrotizing fasciitis within 10-72 h following the onset of first symptoms . Between 1 and 4 days following administration of C1-esterase inhibitor, a marked shift of fluid from extravascular to intravascular compartments too k place in all but one patient, accompanied by a transient intra-alveolar l ung edema and rapidly decreasing need for adrenergic agents. Six of seven p atients survived. Conclusions: These clinical observations in a small serie s of patients and the favorable outcome point towards a positive effect of early and high-dose administration of C1-esterase inhibitor as adjunctive t herapy in streptococcal TSS. The possible mechanism involved may be the att enuation of capillary leak syndrome (CLS) via early inactivation of complem ent and contact systems. Controlled studies are needed to establish an impr ovement of the survival rates of patients with streptococcal TSS following administration of C1-esterase inhibitor.