Skip to main content
  • Meeting abstract
  • Published:

C1-Inhibitor substitution as ultima ratio therapy in septic shock: experience with 15 patients


Plasma C1-Inhibitor (C1-INH) is the major inhibitor of both the classical pathway of complement system and contact activation. A relative deficiency of C1-INH has been proposed as an important contributor to the development of shock and organ failure [1,2]. Among others, our working group has published two cases of successful C1-INH substitution in septic patients recently [3].


In a retrospective study we investigated data of 15 patients with septic shock who received C1-INH concentrate as ultima ratio therapy. The mean substitutional dosage of C1-INH concentrate (Berinert HS', Centeon) was 10.300 units. Mean values for C1-INH activity, antigenic C1-INH level, fluid balance, hemodynamic and respiratory parameters were compared 24 h prior to 96 h after C1-INH administration.


After substitutional therapy the antigenic C1-INH level increased significantly (from 159 to 228%) while the functional C1-INH activity did not change statistically. The daily fluid balance decreased from +2695 to -186 ml/24 h (P = 0.05), hemodynamic parameters were not affected. There was a good tendency towards higher arterial-to-inspired oxygen ratios (P = 0.55) after C1-INH administration.


Our data show significantly higher antigenic C1-INH levels after substitutional therapy but no difference in C1-INH functional activity. This may be due to complex formation of C1-INH with its target proteases. Prior to C1-INH administration the patients had positive fluid balances and impaired arterial-to-inspired oxygen ratios. If This is looked upon as an equivalent to capillary leakage these results encourage to perform a randomised controlled study on C1-INH substitution in septic patients.


  1. Nuijens JH, et al: Proteolytic inactivation of plasma C1 inhibitor in sepsis. J Clin Invest. 1989, 84: 443-10.1172/JCI114185.

    Article  PubMed  CAS  PubMed Central  Google Scholar 

  2. Hack CE, et al: C1 -inhibitor substitution therapy in septic shock and in vascular leak syndrome induced by high doses of interleukin-2. Intensive Care Med. 1993, 19: 19-28. 10.1007/BF01738946.

    Article  Google Scholar 

  3. Vangerow B, et al: Clinical improvement after administration of C1 -Inhibitor in two patients with SIRS and capillary leak syndrome. Intensive Care Med. 1996, 22 (suppl 3): 367-

    Google Scholar 

Download references

Author information

Authors and Affiliations


Rights and permissions

Reprints and permissions

About this article

Cite this article

Vangerow, B., Marx, G., Leuwer, M. et al. C1-Inhibitor substitution as ultima ratio therapy in septic shock: experience with 15 patients. Crit Care 2 (Suppl 1), P019 (1998).

Download citation

  • Published:

  • DOI: