Skip to main content

From Localized Angioedema to Generalized Capillary Leak Syndrome: Evidence for a Pivotal Role of C1-Inhibitor in Septic Shock-like Syndromes

  • Chapter
Sepsis

Part of the book series: Update in Intensive Care and Emergency Medicine ((UICM,volume 18))

Abstract

C1-inhibitor (C1-INH) is the only known inhibitor of the classical pathway of complement system and the most important inhibitor of factor XII-mediated contact activation. The genetic deficiency of C1-INH (hereditary angioedema) causes acute edema formation which is in most cases locally restricted to the upper extremities, neck, and face. Acute onset of an attack often results in life-threatening events such as laryngeal edema, which is resistant to treatment with corticosteroids.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 84.99
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 109.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. Bock SC, Skriver K, Nielsen E et al (1986) Human C1-inhibitor: primary structure, cDNA cloning and chromosomal localization. Biochemistry 25:4292–4301

    Article  PubMed  CAS  Google Scholar 

  2. Harrison RA (1983) Human C1-inhibitor: improved isolation and preliminary structural characterization. Biochemistry 22:5001

    Article  PubMed  CAS  Google Scholar 

  3. Nilsson T, Wiman B (1982) Purification and characterization of human C1-esterase inhibitor. Biochim Biophys 705:271

    Article  CAS  Google Scholar 

  4. Perkins SJ, Smith KF, Amatayakul S et al (1990) Two-domain structure of the native and reactive centre cleaved forms of C1-inhibitor of human complement by neutron scattering. J Mol Biol 214:751–756

    Article  PubMed  CAS  Google Scholar 

  5. Laskowski M Jr, Kato I (1980) Protein inhibitors of proteinases. Ann Rev Biochem 49:593–626

    Article  PubMed  CAS  Google Scholar 

  6. Ziccardi RJ, Cooper NR (1978) Modulation of the antigenicity of C1r and C1s inactivator. J Immunol 121:2148–2152

    PubMed  CAS  Google Scholar 

  7. Harpel PC, Cooper NR (1975) Studies on human plasma C1-inactivator enzyme interactions. I. Mechanisms of interaction with Cls, plasmin and trypsin. J Clin Invest 55:593–604

    CAS  Google Scholar 

  8. Ratnoff OD, Lepow IH (1957) Some properties of an esterase derived from preparations of the first component of complement. J Exp Med 106:327–343

    Article  PubMed  CAS  Google Scholar 

  9. Ratnoff OD, Pensky J, Ogston D, Naff GB (1969) The inhibition of plasmin, plasma kallikrein, plasma permeability factor, and the Clr subcomponent of the first component of complement by serum C1-esterase inhibitor. J Exp Med 129:315–331

    Article  PubMed  CAS  Google Scholar 

  10. Forbes CD, Pensky J. Ratnoff OD (1970) Inhibition of activated Hageman factor and activated plasma thromboplastin antecedent by purified C1-inactivator. J Lab Clin Med 76:809–815

    PubMed  CAS  Google Scholar 

  11. Schreiber AD, Kaplan AP, Austen KF (1973) Inhibition by C1-INH of Hageman factor fragment activation of coagulation, fibrinolysis, and kinin generation. J Clin Invest 52:1402–1409

    Article  PubMed  CAS  Google Scholar 

  12. De Agostini A, Lijnen HR, Pixley RA, Colman RW, Schapira M (1984) Inactivation of factor XII active fragment in normal plasma. J Clin Invest 73:1542–1549

    Article  PubMed  Google Scholar 

  13. Ghebrehiwet B, Siverberg M, Kaplan AP (1981) Activations of the classical pathway of complement by Hageman factor fragment. J Exp Med 153:665–676

    Article  PubMed  CAS  Google Scholar 

  14. Ghebrehiwet B, Randazzo BP, Dunn JT (1983) Mechanisms of activation of the classical pathway of complement by Hageman factor fragment. J Clin Invest 71:1450–1455

    Article  PubMed  CAS  Google Scholar 

  15. Bjork J, Hugli TE, Smedegård G (1985) Microvascular effects of anaphylatoxins C3a and C5a. J Immunol 134:1115–1119

    PubMed  CAS  Google Scholar 

  16. Vane JR, Anggard EE, Botting RM (1990) Regulatory functions of the vascular endothelium. N Engl J Med 323:27–36

    Article  PubMed  CAS  Google Scholar 

  17. Johnson AM, Alper CA, Rosen RS, Craig JM (1971) C1-inhibitor: evidence for decreased hepatic synthesis in hereditary angioneurotic oedema. Science 173:553–554

    Article  PubMed  CAS  Google Scholar 

  18. Schmaier AH, Smith PM, Colman RW (1985) Platelet C1 inhibitor. A secreted alphagranule protein. J Clin Invest 75:242–250

    Article  CAS  Google Scholar 

  19. Lotz M, Zuraw BL (1987) Interferon gamma is a major regulator of C1-inhibitor synthesis by human blood monocytes. J Immunol 139:3382

    PubMed  CAS  Google Scholar 

  20. Lappin D, Hamilton AO, Morrison L, Aref M, Whaley K (1986) Synthesis of complement components (C3, C2, B and C1-inhibitor) and lysozyme by human monocytes and macrophages. J Clin Lab Immunol 20:101–105

    PubMed  CAS  Google Scholar 

  21. Gitlin D, Biasucci A (1969) Development of G, A, M, IC/IC, C1 esterase inhibitor, ceruloplasmin, transferrin, hemopexin, haptoglobin, fibrinogen, plasminogen, 1-antitrypsin, orosomucoid, lipoprotein, 2-macroglobulin, and prealbumin in the human conceptus. J Clin Invest 48:1433–1446

    Article  PubMed  CAS  Google Scholar 

  22. Heda GD, Mardente S, Weiner L, Schmaier AH (1990) Interferon gamma increases in vitro and in vivo expression of Cl-inhibitor. Blood 75:2401

    PubMed  CAS  Google Scholar 

  23. Falus A, Rokita H, Walcz E, Brozik M, Hidvegi T, Meretey K (1990) Hormonal regulation of complement biosynthesis in human cell lines. II. Upregulation of the biosynthesis of complement components C3, factor B and Cl-inhibitor by interleukin-6 and interleukin-1 in human hepatoma cell line. Mol Immunol 27:197

    CAS  Google Scholar 

  24. Zuraw BL, Lotz M (1990) Regulation of the hepatic synthesis of C1-inhibitor by the hepatocyte stimulating factors interleukin-6 and interferon gamma. J Biol Chem 165:12664

    Google Scholar 

  25. Donaldson VH (1966) Serum inhibitor of C1-esterase in health and disease. J Lab Clin Med 68:369–382

    PubMed  CAS  Google Scholar 

  26. Haupt H, Heimburger N, Kranz T, Schwick HG (1970) Ein Beitrag zur Isolierung und Charakterisierung des C1-Inaktivators aus Humanplasma. Eur J Biochem 17:254–261

    Article  PubMed  CAS  Google Scholar 

  27. Kress LF, Catanese J. Hirayama T (1983) Analysis of the effects of snake venom proteinases on the activity of human plasma C1-esterase inhibitor, α1-antichymotrypsin, and α2-antiplasmin. Biochim Biophys Acta 745:113–120

    Article  PubMed  CAS  Google Scholar 

  28. Catanese J, Kress LF (1984) Enzymatic inactivation of human plasma C1-inhibitor and 1-antichymotrypsin by Pseudomonas aeruginosa proteinase and elastase. Biochim Acta 789:37–43

    Article  CAS  Google Scholar 

  29. Brower MS, Harpel PC (1982) Proteolytic cleavage and inactivation of α2-plasmin inhibitor and C1-inactivator by human polymorphonuclear leukocyte elastase. J Biol Chem 257:9849–9854

    PubMed  CAS  Google Scholar 

  30. Pemberton PA, Harison RA, Lachmann PJ, Carrel RW (1989) The structural basis for neutrophil inactivation of Cl-inhibitor. Biochem J 258:193–198

    PubMed  CAS  Google Scholar 

  31. Reboul A, Prandini MH, Colomb MG (1987) Proteolysis and deglycosylation of human C1-inhibitor: effect on functional properties. Biochem J 244:117–119

    PubMed  CAS  Google Scholar 

  32. Quincke H (1882) Über akutes umschriebenes Hautoedem. Monatsschr Prakt Dermatol 1:129–131

    Google Scholar 

  33. Osier W (1888) Hereditary angio-neurotic edema. Am J Med Sci 95:362–367

    Article  Google Scholar 

  34. Crowder JR, Crowder TR (1917) Five generations of angioneurotic edema. Arch Intern Med 20:840–852

    Article  Google Scholar 

  35. Donaldson VH, Evans RR (1963) A biochemical abnormality in hereditary angioneurotic edema: absence of serum inhibitor of C1-esterase. Am J Med 31:37–44

    Article  Google Scholar 

  36. Agostoni A, Cicardi M (1992) Hereditary and acquired C1-inhibitor deficiency: biological and clinical characteristics in 235 patients. Medicine 71:206–215

    Article  PubMed  CAS  Google Scholar 

  37. Carreer FMJ (1992) The C1-inhibitor deficiency. A review. Eur J Clin Chem Clin Biochem 30:793–807

    CAS  Google Scholar 

  38. Colten HR (1987) Hereditary angioneurotic edema, 1887 to 1987. N Engl J Med 317:43–45

    Article  PubMed  CAS  Google Scholar 

  39. Davis AE (1988) C1-inhibitor and hereditary angioneurotic edema. Ann Rev Immunol 6:595–628

    Article  CAS  Google Scholar 

  40. Kodama J, Uchida K, Yoshimura S, Katayama Y, Kushiro H, Yutani C et al (1984) Studies of four Japanese families with hereditary angioneurotic edema. Simultaneous activation of plasma protease systems and exogenous triggering stimuli. Blut 49:405–418

    CAS  Google Scholar 

  41. Sayama K, Shiraishi S, Miki Y (1985) Complement profile in a C1-inhibitor deficient family. Br J Dermatol 113:737–743

    Article  PubMed  CAS  Google Scholar 

  42. Widmer M, Späth P, Wüthrich B (1989) Hereditäres Angioödem (HAO): Substitutionstherapie mit C1-inhibitor Konzentrat zur Intervallbehandlung. Allergologie 12:432–439

    Google Scholar 

  43. Rosen FS; Charache P, Pensky J, Donaldson V (1965) Hereditary angioneurotic edema: two genetic variants. Science 148:957

    Article  PubMed  CAS  Google Scholar 

  44. Oltvai TN, Wong ECC, Atkinson JP, Tung KSK (1991) C1-inhibitor deficiency: molecular and immunologic basis of hereditary and acquired angioedema. Lab Invest 65:381–388

    PubMed  CAS  Google Scholar 

  45. Bork K, Kreuz W, Witzke G (1984) Hereditäres angioneurotisches Ödem. Dtsch Med Wochenschr 109:1331–1335

    Article  PubMed  CAS  Google Scholar 

  46. Huston DP, Bressler RB (1992) Urticaria and angioedema. Clin Allergy 76:805–840

    CAS  Google Scholar 

  47. Hentges F, Humbel R, Dicato M, Hemmer R, Kuntziger H (1986) Acquired C1-esterase inhibitor deficiency: case report with emphasis on complement and kallikrein activation during two patterns of clinical manifestations. J Allergy Clin Immunol 78:860–867

    Article  PubMed  CAS  Google Scholar 

  48. Nilsson T, Back O (1985) Elevated plasmin-α2-antiplasmin complex levels in hereditary angioedema. Evidence for the in vivo efficiency of the intrinsic fibrinolytic pathway. Thromb Res 40:817–824

    CAS  Google Scholar 

  49. Schapira M, Silver LD, Scott CF, Schmaier AH, Prograis LJ Jr, Curd JG, Colman RW (1983) Prekallikrein activation and high-molecular weight kininogen consumption in hereditary angioedema. N Engl J Med 308:1050–1053

    Article  PubMed  CAS  Google Scholar 

  50. Curd JG, Prograis LJ Jr, Cochrane CG (1980) Detection of active kallikrein in induced blister fluids of hereditary angioedema patients. J Exp Med 152:742

    Article  PubMed  CAS  Google Scholar 

  51. Agostoni A (1989) Inherited Cl-inhibitor deficiency. Complement Inflamm 6:112–118

    PubMed  CAS  Google Scholar 

  52. Melamed J, Alper CA, Cicardi M, Rosen FS (1986) The metabolism of C1-inhibitor and C1q in patients with acquired C1-inhibitor deficiency. J Allergy Clin Immunol 77:322–326

    Article  PubMed  CAS  Google Scholar 

  53. Geha RS, Quinti I, Austen KF, Cicardi M, Sheffer A, Rosen FS (1985) Acquired C1-inhibitor deficiency associated with antiidiotypic antibody to monoclonal immunoglobulins. N Engl J Med 312:534–540

    Article  PubMed  CAS  Google Scholar 

  54. Hauptmann G, Petitjean F, Lang JM, Oberling F (1979) Acquired C1-inhibitor deficiency in a case of lymphosarcoma of the spleen. Reversal of complement abnormalities after splenectomy. Clin Exp Immunol 37:523–531

    CAS  Google Scholar 

  55. Schreiber AD, Zweiman B, Atkins P, Goldwein F, Pietra G, Atkinson B, Abdou NI (1976) Acquired angioedema with lymphoproliferative disorder: association of Cl-inhibitor deficiency with cellular abnormality. Blood 48:567–580

    PubMed  CAS  Google Scholar 

  56. Alsenz J, Bork K, Loos M (1987) Autoantibody-mediated acquired deficiency of C1-inhibitor. N Engl J Med 316:1360–1366

    Article  PubMed  CAS  Google Scholar 

  57. Chin HL, Buchan DA (1990) Severe angioedema after long-term use of an angiotensin-converting enzyme inhibitor (letter). Ann Intern Med 112:312–313

    PubMed  CAS  Google Scholar 

  58. Agostoni A, Cicardi M (1991) Contraindications to the use of ACE-inhibitors in patients with C1-esterase inhibitor deficiency. Am J Med 90:278

    PubMed  CAS  Google Scholar 

  59. Seidman MD, Lewandowski CA, Sarpa JR, Potesta E, Schweitzer VG (1990) Angioedema related to angiotensin-converting enzyme inhibitors. Otolaryngol Head Neck Surg 102:727–731

    PubMed  CAS  Google Scholar 

  60. Caspritz G (1986) The blocking by human C1-inactivator of both carrageenin paw edema and its potentiation by the angiotensin converting enzyme-inhibitor ramipril. IRCS Med Sci 14:568–569

    CAS  Google Scholar 

  61. Van der Starre P, Sinclair D, Damen J. Brummelhuis H (1980) Inhibition of the hypotensive effect of plasma protein solutions by C1-esterase inhibitor. J Thorac Cardiovasc Surg 79:738–740

    PubMed  Google Scholar 

  62. Wernze H (1988) Angioneurotisches Ödem unter ACE-Hemmern: Häufigkeit, klinische Charakteristik, Auslösemechamismen. Z Kardiol 77:61–64

    PubMed  Google Scholar 

  63. (1991) Angioneurotische Ödeme und ACE-Hemmer. Bundesgesundheitsbl 7:353

    Google Scholar 

  64. Diehl KL, Wernze H (1992) Angioneurotisches Ödem durch Angiotensin-Konversionsenzym-Hemmer. Dtsch Med Wochenschr 117:727–732

    Article  PubMed  CAS  Google Scholar 

  65. Clarkson B, Thompson D, Horwith M, Luckey EH (1960) Cyclical edema and shock due to increased capillary permeability. Am J Med 29:193–216

    Article  PubMed  CAS  Google Scholar 

  66. Luke IW, Rubenstein E (1962) Fatal postpartum shock due to massive angioneurotic edema. Am J Obst Gynecol 83:322–327

    CAS  Google Scholar 

  67. George C, Regnier B, Le Gall JR, Gastinne H, Carlet J, Rain M (1978) Hypovolemic shock with oedema due to increased capillary permeability. Intensive Care Med 4:159–163

    Article  PubMed  CAS  Google Scholar 

  68. Teelucksingh S, Padfield PL (1989) Systemic capillary leak syndrome. Lancet 1:330–331

    Article  PubMed  CAS  Google Scholar 

  69. Cain JR, Bourgoignie JJ, Ahn YS, Schulz DR (1986) Systemic capillary leak syndrome (CLS): a rare cause of systemic edema. Kidney Int 29:181–186

    Google Scholar 

  70. Johansson BR, Löfdahl CG (1979) Ultrastructure of the microvessels in skeletal muscle in a case of systemic capillary leak syndrome. Acta Med Scand 206:413–416

    Article  PubMed  CAS  Google Scholar 

  71. Löfdahl CG, Sölvell L, Laurell AB, Johansson BR (1979) Systemic capillary leak syndrome with monoclonal IgG and complement alterations. Acta Med Scand 206:405–412

    Article  PubMed  Google Scholar 

  72. Atkinson JP, Waldmann TA, Stein SF et al (1977) Systemic capillary leak syndrome and monoclonal gamopathy. Medicine (Baltimore) 56:225–239

    CAS  Google Scholar 

  73. Knaus WA, Wagner DP (1989) Multiple systems organ failure: epidemiology and prognosis. Crit Care Clin 5:221

    PubMed  CAS  Google Scholar 

  74. Nuytinck HKS, Offermans KK, Goris RJA (1987) Whole body inflammation in trauma patients, an autopsy study. First Vienna Shock Forum, Part A: pathophysiological role of mediators and mediator inhibitors in shock, pp 55-61

    Google Scholar 

  75. Williams JG, Maier RV (1992) The inflammatory response. Intensive Care Med 7:53–66

    Google Scholar 

  76. Bone RC (1992) Sepsis and multiple organ failure: consensus and controversy. In: Lamy M, Thijs LG (eds) Mediators of sepsis. Springer, Berlin Heidelberg New York pp 3–12

    Chapter  Google Scholar 

  77. Hack CE, De Groot ER; Felt-Bersma RJF et al (1989) Increased plasma levels of interleukin-6 in sepsis. Blood 74:1704–1710

    PubMed  CAS  Google Scholar 

  78. Hesse DG, Tracey KJ, Fong Y et al (1988) Cytokine appearance in human endotoxemia and primate bacteremia. Surg Gynecol Obstet 166:147–153

    PubMed  CAS  Google Scholar 

  79. Martich GD, Danner RL, Ceska M. Suffredini AF (1991) Detection of IL-8 and TNF in normal humans after intravenous endotoxin: the effect of antiinflammatory agents. J Exp Med 173:1021–1024

    Article  PubMed  CAS  Google Scholar 

  80. Michie HR, Manogue KR, Spriggs DR et al (1988) Detection of circulating tumor necrosis factor after endotoxin administration. N Engl J Med 318:1481–1486

    Article  PubMed  CAS  Google Scholar 

  81. Van Deventer SJH, Büller HR, ten Cate JW, Aarden LA, Hack CE, Sturk A (1990) Experimental endotoxaemia in humans: analysis of cytokine release and coagulation, fibrinolytic, and complement pathway. Blood 76:2520–2526

    PubMed  Google Scholar 

  82. Van Zee KJ, DeForge LE, Fischer E, Marano MA, Kenney JS, Remick DG Lowry SF, Moldawer LL (1991) IL-8 in septic shock, endotoxemia, and after IL-1 administration. J Immunol 146:3478–3482

    PubMed  Google Scholar 

  83. Waage A, Brandtzaeg P, Halstensen A, Kierulf P, Espevik T (1989) The complex pattern of cytokines in serum from patients with meningococcal septic shock. Association between interleukin-6, interleukin-1 and fatal outcome. J Exp Med 169:333–338

    CAS  Google Scholar 

  84. Alexander HR, Doherty GM, Buresh CM, Venzon DJ, Norton JA (1991) A recombinant human receptor antagonist to IL-1 improves survival after lethal endotoxemia in mice. J Exp Med 173:1029–1032

    Article  PubMed  CAS  Google Scholar 

  85. Ashkenazi A, Marsters SA, Capon DJ et al (1991) Protection against endotoxic shock by tumor necrosis factor receptor immunoadhesion. Proc Natl Acad Sci USA 88:10535–10539

    Article  PubMed  CAS  Google Scholar 

  86. Beutler B, Milsark IW, Cerami A (1985) Passive immunization against cachectin/ tumor necrosis factor protects mice from lethal effect of endotoxin. Science 229:869–871

    Article  PubMed  CAS  Google Scholar 

  87. Heremans H, Van Damme J, Dillen C, Dijkmans R, Billiau A (1990) Interferon gamma, a mediator of lethal lipopolysaccharide-induced Schwartzman-like shock reactions in mice. J Exp Med 171:1853–1869

    Article  PubMed  CAS  Google Scholar 

  88. Ohlsson K, Björk P, Bergenfeldt M, Hageman R, Thompson RC (1990) Interleukin-1 receptor antagonist reduces mortality from endotoxin shock. Nature 348:550–552

    Article  PubMed  CAS  Google Scholar 

  89. Starnes HF Jr, Pearce MK, Tewari A, Yim JH, Zou JC, Abrams JS (1990) Anti-IL-6 monoclonal antibodies protect against lethal Escherichia coli infection and lethal tumor necrosis factor-alpha challenge in mice. J Immunol 145:4185–4191

    PubMed  CAS  Google Scholar 

  90. Baars JWS, Hack CE, Wagstaff J et al (1992) The activation of polymorphonuclear neutrophils and the complement system during immunotherapy with recombinant interleukin-2. Br J Cancer 65:96–101

    Article  PubMed  CAS  Google Scholar 

  91. Suffredini AF, Harpel P, Parillo JE (1989) Promotion and subsequent inhibition of plasminogen activator after administration of intravenous endotoxin to normal subjects. N Engl J Med 18:1165–1171

    Article  Google Scholar 

  92. Thijs LG, Hack CE, Strack van Schijndel RJM et al (1990) Activation of the complement system during immunotherapy with recombinant interleukin-2: relation to the development of side effects. J Immunol 144:2419–2424

    PubMed  CAS  Google Scholar 

  93. Vachino G, Gelfand JA, Atkins MB, Tamerius JD, Demchak P, Mier JW (1991) Complement activation in cancer patients undergoing immunotherapy with interleukin-2 (IL-2): binding of complement and C-reactive protein by IL-2-activated lymphocytes. Blood 78:2505–2513

    PubMed  CAS  Google Scholar 

  94. Gaynor ER, Vitek L, Sticklin L et al (1988) The hemodynamic effects of treatment with interleukin-2 and lymphokine activated killer cells. Ann Intern Med 109:953–958

    PubMed  CAS  Google Scholar 

  95. Natanson C, Eichenholz PW, Danner RL et al (1989) Endotoxin and tumor necrosis factor challenges in dogs simulate the cardiovascular profile of human septic shock. J Exp Med 169:823–832

    Article  PubMed  CAS  Google Scholar 

  96. Ognibene FP, Parker MM, Burch-Whitman C et al (1988) Neutrophil aggregating activity and septic shock in humans: neutrophil aggregation by C5a-like material occurs more frequently than complement component depletion and correlates with depression of systemic vascular resistance. Crit Care Med 3:103–111

    Google Scholar 

  97. Okusawa S, Dinarello CA, Yancey KB et al (1987) C5a induction of human interleu-kin 1. Synergistic effect with endotoxin or interferon-gamma. J Immunol 139:2635–2640

    PubMed  CAS  Google Scholar 

  98. Okusawa S, Gelfland JA, Ikejima T, Connolly RJ, Dinarello CA (1988) Interleukin 1 induces a shock-like state in rabbits. J Clin Invest 81:1162–1172

    Article  PubMed  CAS  Google Scholar 

  99. Tracey KJ, Beutler B, Lowry SF et al (1986) Shock and tissue injury induced by human recombinant cachectin. Science 234:470–474

    Article  PubMed  CAS  Google Scholar 

  100. Piguet PF, Grau GE, Vassalli P (1990) Subcutaneous perfusion of tumor necrosis factor induces local proliferation of fibroblasts, capillaries, and epidermal cells, or massive tissue necrosis. Am J Pathol 136:103–110

    PubMed  CAS  Google Scholar 

  101. Remick DG, Kunkel RG, Larric JW, Kunkel SL (1987) Acute in vivo effects of human recombinant tumor necrosis factor. Lab Invest 56:583–590

    PubMed  CAS  Google Scholar 

  102. Colman RW (1992) The role of the kallikrein-kinin system in septic shock. In: Lamy M, Thijs LG (eds) Mediators in sepsis. Springer, Berlin Heidelberg New York, pp 99–112

    Chapter  Google Scholar 

  103. Thijs LG, Hack CE (1992) Role of the complement cascade in severe sepsis. In: Lamy M, Thijs LG (eds) Mediators of sepsis. Springer, Berlin Heidelberg New York, pp 78–98

    Chapter  Google Scholar 

  104. Nürnberger W, Göbel U, Stannigel H, Eisele B, Janssen A, Delvos U (1992) C1-inhibitor concentrate for sepsis-related capillary leak syndrome. Lancet 339:990

    Article  PubMed  Google Scholar 

  105. Beutler B, Cerami A (1987) Cachectin: more than a tumor necrosis factor. N Engl J Med 316:379–385

    Article  PubMed  CAS  Google Scholar 

  106. Bone RC (1991) The pathogenesis of sepsis. Ann Intern Med 115:457–469

    PubMed  CAS  Google Scholar 

  107. Cohen J, Glauser MP (1991) Septic shock: treatment, Lancet 338:736–739

    Article  PubMed  CAS  Google Scholar 

  108. DeForge LE, Nguyen DT, Kunkel SL, Remick DG (1990) Regulation of the pathophysiology of tumor necrosis factor. J Lab Clin Med 116:429–438

    PubMed  CAS  Google Scholar 

  109. Fong Y, Lowry SF (1990) Tumor necrosis factor in the pathophysiology of infection and sepsis. Clin Immunol Immunopathol 55:157–170

    Article  PubMed  CAS  Google Scholar 

  110. McCabe WR, Treadwell TL, De Maria A Jr (1983) Pathophysiology of bacteremia. Am J Med 75:7–18

    Article  PubMed  CAS  Google Scholar 

  111. Morrison DC, Ulevitch RJ (1978) The effects of bacterial endotoxins on host mediation systems. Am J Pathol 93:527–617

    Google Scholar 

  112. Parillo JE, Parker MM, Natanson C, Suffredini AF, Danner RL. Cunnion RE, Ognibene FP (1990) Septic shock in humans. Advances in the understanding of pathogenesis, cardiovascular dyscfunction, and therapy. Ann Intern Med 113:227–242

    Google Scholar 

  113. Parker MM, Parillo JE (1983) Septic shock. Hemodynamics and pathogenesis. JAMA 250:3324–3327

    CAS  Google Scholar 

  114. Fearon DT, Ruddy S, Schur PH, McCabe WR (1975) Activation of the properdin pathway of complement in patients with gram-negative bacteremia. N Engl J Med 292:937–940

    Article  PubMed  CAS  Google Scholar 

  115. Füst G, Petras G, Ujhelyi E (1976) Activation of the complement system during infections due to gram-negative bacteria. Clin Immunol Immunopathol 5:293–302

    Article  PubMed  Google Scholar 

  116. León C, Rodrigo MJ, Tomasa A et al (1982) Complement activation in septic shock due to gram-negative and gram-positive bacteria. Crit Care Med 10:308–310

    Article  PubMed  Google Scholar 

  117. Slotman GJ, Burchard KW, Williams JJ, D’Arezzo A, Yellin SA (1986) Interaction of prostaglandins, activated complement, and granulocytes in clinical sepsis and hypotension. Surgery 99:744–751

    PubMed  CAS  Google Scholar 

  118. Brandtzaeg P, Mollnes TE, Kierulf P (1989) Complement activation and endotoxin levels in systemic meningococcal disease. J Infect Dis 16:58–65

    Article  Google Scholar 

  119. Hack CE, Nuijens JH, Felt-Bersma RJF et al (1989) Elevated plasma levels of the anaphylatoxins C3a and C4a are associated with a fatal outcome in sepsis. Am J Med 86:20–26

    Article  PubMed  CAS  Google Scholar 

  120. Weinberg PF, Matthay MA, Webster RO, Roskos KV, Goldstein IM, Murray JF (1984) Biologically active products of complement and acute lung injury in patients with the sepsis syndrome. Am Rev Respir Dis 130:791–796

    PubMed  CAS  Google Scholar 

  121. Bodammer G, Vogt W (1967) Actions of anaphylatoxin on circulation and respiration in the guinea pig. Int Arch Allergy 32:417–428

    Article  PubMed  CAS  Google Scholar 

  122. Lundberg C, Marceau F, Hugli TE (1987) C5a-induced hemodynamic and hematologic changes in the rabbit. Role of cyclooxygenase products and polymorphonuclear leukocytes. Am J Pathol 128:471–483

    CAS  Google Scholar 

  123. Pavel K, Piper PJ, Smedegård G (1979) Anaphylatoxin-induced shock and two patterns of anaphylactic shock: hemodynamics and mediators. Acta Physiol Scand 105:393–403

    Article  Google Scholar 

  124. Smedegard G, Cui L, Hugli TE (1989) Endotoxin-induced shock in the rat. A role for C5a. Am J Pathol 135:489–497

    CAS  Google Scholar 

  125. Garner R, Chater BV, Brown DL (1974) The role of complement in endotoxin shock and disseminated intravascular coagulation. Experimental observations in the dog. Br J Haematol 28:393–401

    CAS  Google Scholar 

  126. Hachfeld del Balzo U, Levi R, Polley MJ (1985) Cardiac dysfunction caused by purified human C3a anaphylatoxin. Proc Natl Acad Sci 82:886–890

    Article  Google Scholar 

  127. Thijs LG, Hack CE, Strack van Schijndel RJM et al (1989) Complement activation and high-dose of interleukin-2. Lancet II: 395

    Article  Google Scholar 

  128. Colman RW, Edelman R, Scott CF, Gilman RM (1978) Plasma kallikrein activation and inhibition during typhoid fever. J Clin Invest 61:287–296

    Article  PubMed  CAS  Google Scholar 

  129. Kalter ES; Daha MR, ten Cate JW, Verhoef J, Bouma BN (1985) Activation and inhibition of Hageman factor-dependent pathways and the complement system in uncomplicated bacteremia or bacterial shock. J Infect Dis 151:1019–1027

    Article  PubMed  CAS  Google Scholar 

  130. Mason JM, Kleeberg V, Dolan P, Colman RW (1970) Plasma kallikrein and Hageman factor in gram-negative bacteremia. Ann Intern Med 73:545–551

    PubMed  CAS  Google Scholar 

  131. Nuijens JH, Huijbregts CC, Eerenberg-Belmer AJ et al (1988) Quantification of plasma factor Xlla-C1-inhibitor and kallikrein-C1-inhibitor complexes in sepsis. Blood 72:1841–1848

    PubMed  CAS  Google Scholar 

  132. Robinson JA, Kloduycky ML, Lock HH, Racic MR, Gunner RM (1975) Endotoxin prekallikrein, complement and systemic vascular resistance sequential measurements in man. Am J Med 59:61–67

    Article  PubMed  CAS  Google Scholar 

  133. Hack CE, Nuijens JH, Strack van Schijndel RJM, Abink JJ, Eerenberg AJM, Thijs LG (1990) A model for the interplay of inflammatory mediators in sepsis — a study in 48 patients. Intensive Care Med 16:187–191

    Article  Google Scholar 

  134. Pixley RA, De La Cadena R, Page JD et al (1993) The contact system contributes to hypotension but not disseminated intravascular coagulation in lethal bacteremia: in vivo use of a monoclonal antifactor XII antibody to block contact activation in baboons. J Clin Invest (in press)

    Google Scholar 

  135. Hack CE, Ogilvie AC, Eisele B, Eerenberg AJM, Wagstaff J, Thijs LG (1993) C1-inhibitor substitution therapy in septic shock and in the vascular leak syndrome induced by high doses of interleukin-2. Intensive Care Med 19:(in press)

    Google Scholar 

  136. Nuijens JH, Eerenberg-Belmer AJM, Huijbregts CCM et al (1989) Proteolytic inactivation of plasma C1-inhibitor in sepsis. J Clin Invest 84:443

    Article  PubMed  CAS  Google Scholar 

  137. Rosenberg SA, Lotze MT, Muul LM et al (1987) A progress report on the treatment of 157 patients with advanced cancer using lymphokine-activated killer cells and interleukin-2 or high-dose interleukin-2 alone. N Engl J Med 316:889–897

    Article  PubMed  CAS  Google Scholar 

  138. Rosenberg SA, Lotze MT, Mule JJ (1988) New approaches to the immunotherapy of cancer using interleukin-2. Ann Intern Med 108:853–864

    PubMed  CAS  Google Scholar 

  139. Ognibene FP, Rosenberg SA, Lotze M, Skibber J, Parker MM, Shelhamer JH, Parillo JE (1988) Interleukin-2 administration causes reversible hemodynamic changes and left ventricular dysfunction similar to those seen in septic shock. Chest 94:750–754

    Article  PubMed  CAS  Google Scholar 

  140. Boccoli G, Masciulli R, Ruggeri EM et al (1990) Adaptive immunotherapy of human cancer: the cytokine cascade and monocyte activation following high-dose interleukin-2 bolus treatment. Cancer Res 50:5795–5800

    PubMed  CAS  Google Scholar 

  141. Jablons DM, Mul’ JJ, Mcintosh JK et al (1989) IL-6/IFN-β-2 as a circulating hormone. Induction by cytokine administration in humans. J Immunol 142:1542–1547

    CAS  Google Scholar 

  142. Mier JW, Vachino G, Van der Meer JW et al (1988) Induction of circulating tumor necrosis factor (TNF alpha) as the mechanism for the febrile response to interleukin-2 (IL-2) in cancer patients. J Clin Immunol 8:426–436

    Article  PubMed  CAS  Google Scholar 

  143. Hack CE, Wagstaff J, Strack van Schijndel RJM, Eerenberg AJM, Pinedo HM, Thijs LG, Nuijens JH (1991) Studies on the contact system of coagulation during therapy with high doses of recombinant IL-2: implications for septic shock. Thromb Haemost 65:497–503

    PubMed  CAS  Google Scholar 

  144. Nuytinck HKS, Offermans KK, Goris RJA (1988) Whole body inflammation in trauma patients. Arch Surg 123:1519

    Article  PubMed  CAS  Google Scholar 

  145. Oldham K, Guice K, Till G, Ward P (1988) Activation of complement by hydroxyl radical thermal injury. Surgery 104:272–277

    PubMed  CAS  Google Scholar 

  146. Nerlich M, Flynn J, Demling R (1983) Effect of thermal injury on endotoxin induced lung injury. Surgery 92:289

    Google Scholar 

  147. Wilmore D, Orcutt T, Mason A (1975) Alterations in hypothalamic function following thermal injury. J Trauma 15:697

    Article  PubMed  CAS  Google Scholar 

  148. Youn YK, LaLonde C, Demling R (1992) The role of mediators in the response to thermal injury. World J Surg 16:30–36

    Article  PubMed  CAS  Google Scholar 

  149. Demling R, LaLonde C (1993) Early burn excision attenuates the post burn lung and systemic response to endotoxin. Surgery (in press)

    Google Scholar 

  150. Adam A, Damas J, Albert A, Ers P, Marichi J, Calay G, Laurent P (1986) Plasma prokallikrein and kininogens in burned patients. Thromb Res 41:537–543

    Article  PubMed  CAS  Google Scholar 

  151. Scott-Conner CEH, Meydrech E (1988) Quantitation of rate of wound closure and the prediction of death following major burns. Burns 14:373–378

    Article  CAS  Google Scholar 

  152. Nürnberger W, Michelmann I, Petrik K et al (1993) Activity of C1-esterase inhibitor in patients with vascular leak syndrome after bone marrow transplantation. Ann Hematol 67:17–21

    Article  PubMed  Google Scholar 

  153. Nürnberger W, Petrik K, Kurre P, Lauermann G, Michelmann I, Burdach S, Göbel U (1993) Effect of Cl-esterase inhibitor (C1-INH) on the activation of complement and contact system in capillary leakage syndrome (CLS) after bone marrow transplantation (BMT). Klin Paediatr 205:309

    Google Scholar 

  154. Martinez-Brotóns F, Oncins JR, Mestress J, Amargós V, Reynaldo C (1987) Plasma kallikrein-kinin system in patients with uncomplicated sepsis and septic shock — comparison with cardiogenic shock. Thromb Haemost 58:709–713

    PubMed  Google Scholar 

  155. Stevens JH, O’Hanley P, Shapiro JM et al (1986) Effects of anti-C5a antibodies on the adult respiratory distress syndrome in septic primates. J Clin Invest 77:1816–1818

    Article  Google Scholar 

Download references

Authors

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 1994 Springer-Verlag Berlin Heidelberg

About this chapter

Cite this chapter

Eisele, B., Delvos, U. (1994). From Localized Angioedema to Generalized Capillary Leak Syndrome: Evidence for a Pivotal Role of C1-Inhibitor in Septic Shock-like Syndromes. In: Reinhart, K., Eyrich, K., Sprung, C. (eds) Sepsis. Update in Intensive Care and Emergency Medicine, vol 18. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-85036-3_39

Download citation

  • DOI: https://doi.org/10.1007/978-3-642-85036-3_39

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-642-85038-7

  • Online ISBN: 978-3-642-85036-3

  • eBook Packages: Springer Book Archive

Publish with us

Policies and ethics