Therapeutic options for patients with angioedema due to C1-inhibitor deficiencies: from pathophysiology to the clinic

References

• Longhurst, H., Cicardi, M. Hereditary angio-oedema. Lancet 2012, 379, 474–481.
   PubMed Web of Science ®Google Scholar
• Frank, M.M. Complement disorders and hereditary angioedema. J Allergy Clin Immunol 2010, 125, S262–S271.
   PubMed Web of Science ®Google Scholar
• Kaplan, A.P. C1 inhibitor deficiency: hereditary and acquired forms. J Investig Allergol Clin Immunol 2001, 11, 211–219.
   PubMed Web of Science ®Google Scholar
• Cugno, M., Zanichelli, A., Foieni, F., Caccia, S., Cicardi, M. C1-inhibitor deficiency and angioedema: molecular mechanisms and clinical progress. Trends Mol Med 2009, 15, 69–78.
   PubMed Web of Science ®Google Scholar
• Agostoni, A., Aygören-Pürsün, E., Binkley, K.E., Blanch, A., Bork, K., Bouillet, L., Bucher, C., Castaldo, A.J., Cicardi, M., Davis, A.E., De Carolis, C., Drouet, C., Duponchel, C., Farkas, H., Fáy, K., Fekete, B., Fischer, B., Fontana, L., Füst, G., Giacomelli, R., Gröner, A., Hack, C.E., Harmat, G., Jakenfelds, J., Juers, M., Kalmár, L., Kaposi, P.N., Karádi, I., Kitzinger, A., Kollár, T., Kreuz, W., Lakatos, P., Longhurst, H.J., Lopez-Trascasa, M., Martinez-Saguer, I., Monnier, N., Nagy, I., Németh, E., Nielsen, E.W., Nuijens, J.H., O’grady, C., Pappalardo, E., Penna, V., Perricone, C., Perricone, R., Rauch, U., Roche, O., Rusicke, E., Späth, P.J., Szendei, G., Takács, E., Tordai, A., Truedsson, L., Varga, L., Visy, B., Williams, K., Zanichelli, A., Zingale, L. Hereditary and acquired angioedema: problems and progress: proceedings of the third C1 esterase inhibitor deficiency workshop and beyond. J Allergy Clin Immunol 2004, 114, S51–131.
   PubMed Web of Science ®Google Scholar
• Kalmár, L., Hegedüs, T., Farkas, H., Nagy, M., Tordai, A. HAEdb: a novel interactive, locus-specific mutation database for the C1 inhibitor gene. Hum Mutat 2005, 25, 1–5.
   PubMed Web of Science ®Google Scholar
• Pappalardo, E., Caccia, S., Suffritti, C., Tordai, A., Zingale, L.C., Cicardi, M. Mutation screening of C1 inhibitor gene in 108 unrelated families with hereditary angioedema: functional and structural correlates. Mol Immunol 2008, 45, 3536–3544.
   PubMed Web of Science ®Google Scholar
• Cichon, S., Martin, L., Hennies, H.C., Müller, F., Van Driessche, K., Karpushova, A., Stevens, W., Colombo, R., Renné, T., Drouet, C., Bork, K., Nöthen, M.M. Increased activity of coagulation factor XII (Hageman factor) causes hereditary angioedema type III. Am J Hum Genet 2006, 79, 1098–1104.
   PubMed Web of Science ®Google Scholar
• Cicardi, M., Zingale, L.C., Pappalardo, E., Folcioni, A., Agostoni, A. Autoantibodies and C C1-inhibitor deficiencies. Medicine (Baltimore) 2003, 82, 274–281.
   PubMed Web of Science ®Google Scholar
• Caldwell, J.R., Ruddy, S., Schur, P.H., Austen, K.F. Acquired C1 inhibitor deficiency in lymphosarcoma. Clin Immunol Immunopathol 1972, 1, 39–52.
   Google Scholar
• Jackson, J., Sim, R.B., Whelan, A., Feighery, C. An IgG autoantibody which inactivates C1-inhibitor. Nature 1986, 323, 722–724.
   PubMed Web of Science ®Google Scholar
• Schreiber, A.D., Zweiman, B., Atkins, P., Goldwein, F., Pietra, G., Atkinson, B., Abdou, N.I. Acquired angioedema with lymphoproliferative disorder: association of C1 inhibitor deficiency with cellular abnormality. Blood 1976, 48, 567–580.
   PubMed Web of Science ®Google Scholar
• Cicardi, M., Beretta, A., Colombo, M., Gioffré, D., Cugno, M., Agostoni, A. Relevance of lymphoproliferative disorders and of anti-C1 inhibitor autoantibodies in acquired angio-oedema. Clin Exp Immunol 1996, 106, 475–480.
   PubMed Web of Science ®Google Scholar
• Castelli, R., Deliliers, D.L., Zingale, L.C., Pogliani, E.M., Cicardi, M. Lymphoproliferative disease and acquired C1 inhibitor deficiency. Haematologica 2007, 92, 716–718.
   PubMed Web of Science ®Google Scholar
• Cugno, M., Castelli, R., Cicardi, M. Angioedema due to acquired C1-inhibitor deficiency: a bridging condition between autoimmunity and lymphoproliferation. Autoimmun Rev 2008, 8, 156–159.
   PubMed Web of Science ®Google Scholar
• Hauptmann, G., Petitjean, F., Lang, J.M., Oberling, F. Acquired C1 inhibitor deficiency in a case of lymphosarcoma of the spleen. Reversal of complement abnormalities after splenectomy. Clin Exp Immunol 1979, 37, 523–531.
   PubMed Web of Science ®Google Scholar
• Geha, R.S., Quinti, I., Austen, K.F., Cicardi, M., Sheffer, A., Rosen, F.S. Acquired C1-inhibitor deficiency associated with antiidiotypic antibody to monoclonal immunoglobulins. N Engl J Med 1985, 312, 534–540.
   PubMed Web of Science ®Google Scholar
• Jackson, J., Sim, R.B., Whaley, K., Feighery, C. Autoantibody facilitated cleavage of C1-inhibitor in autoimmune angioedema. J Clin Invest 1989, 83, 698–707.
   PubMed Web of Science ®Google Scholar
• He, S., Tsang, S., North, J., Chohan, N., Sim, R.B., Whaley, K. Epitope mapping of C1 inhibitor autoantibodies from patients with acquired C1 inhibitor deficiency. J Immunol 1996, 156, 2009–2013.
   PubMed Web of Science ®Google Scholar
• Alsenz, J., Bork, K., Loos, M. Autoantibody-mediated acquired deficiency of C1 inhibitor. N Engl J Med 1987, 316, 1360–1366.
   PubMed Web of Science ®Google Scholar
• Zuraw, B.L., Curd, J.G. Demonstration of modified inactive first component of complement (C1) inhibitor in the plasmas of C1 inhibitor-deficient patients. J Clin Invest 1986, 78, 567–575.
   PubMed Web of Science ®Google Scholar
• Whaley, K., Sim, R.B., He, S. Autoimmune C1-inhibitor deficiency. Clin Exp Immunol 1996, 106, 423–426.
   PubMed Web of Science ®Google Scholar
• D’Incan, M., Tridon, A., Ponard, D., Dumestre-Pérard, C., Ferrier-Le Bouedec, M., Bétail, G., Souteyrand, P., Caillaud, D. Acquired angioedema with C1 inhibitor deficiency: is the distinction between type I and type II still relevant? Dermatology (Basel) 1999, 199, 227–230.
   PubMed Web of Science ®Google Scholar
• Agostoni, A., Cicardi, M. Hereditary and acquired C1-inhibitor deficiency: biological and clinical characteristics in 235 patients. Medicine (Baltimore) 1992, 71, 206–215.
   PubMed Web of Science ®Google Scholar
• Cugno, M., Cicardi, M., Bottasso, B., Coppola, R., Paonessa, R., Mannucci, P.M., Agostoni, A. Activation of the coagulation cascade in C1-inhibitor deficiencies. Blood 1997, 89, 3213–3218.
   PubMed Web of Science ®Google Scholar
• Nussberger, J., Cugno, M., Amstutz, C., Cicardi, M., Pellacani, A., Agostoni, A. Plasma bradykinin in angio-oedema. Lancet 1998, 351, 1693–1697.
   PubMed Web of Science ®Google Scholar
• Davis III, AE. C1-inhibitor and hereditary angioneurotic edema. Annu Rev Immunol 1988, 6, 595–628.
   PubMed Web of Science ®Google Scholar
• Cugno, M., Cicardi, M., Agostoni, A. Activation of the contact system and fibrinolysis in autoimmune acquired angioedema: a rationale for prophylactic use of tranexamic acid. J Allergy Clin Immunol 1994, 93, 870–876.
   PubMed Web of Science ®Google Scholar
• Kaplan, A.P., Joseph, K. The bradykinin-forming cascade and its role in hereditary angioedema. Ann Allergy Asthma Immunol 2010, 104, 193–204.
   PubMed Web of Science ®Google Scholar
• Schapira, M., Silver, L.D., Scott, C.F., Schmaier, A.H., Prograis, L.J., Curd, J.G., Colman, R.W. Prekallikrein activation and high-molecular-weight kininogen consumption in hereditary angioedema. N Engl J Med 1983, 308, 1050–1053.
   PubMed Web of Science ®Google Scholar
• Fields, T., Ghebrehiwet, B., Kaplan, A.P. Kinin formation in hereditary angioedema plasma: evidence against kinin derivation from C2 and in support of “spontaneous” formation of bradykinin. J Allergy Clin Immunol 1983, 72, 54–60.
   PubMed Web of Science ®Google Scholar
• Shoemaker, L.R., Schurman, S.J., Donaldson, V.H., Davis, A.E. Hereditary angioneurotic oedema: characterization of plasma kinin and vascular permeability-enhancing activities. Clin Exp Immunol 1994, 95, 22–28.
   PubMed Web of Science ®Google Scholar
• Han, E.D., MacFarlane, R.C., Mulligan, A.N., Scafidi, J., Davis, A.E. Increased vascular permeability in C1 inhibitor-deficient mice mediated by the bradykinin type 2 receptor. J Clin Invest 2002, 109, 1057–1063.
   PubMed Web of Science ®Google Scholar
• Cugno, M., Hack, C.E., de Boer, J.P., Eerenberg, A.J., Agostoni, A., Cicardi, M. Generation of plasmin during acute attacks of hereditary angioedema. J Lab Clin Med 1993, 121, 38–43.
   PubMed Web of Science ®Google Scholar
• Kleniewski, J., Blankenship, D.T., Cardin, A.D., Donaldson, V. Mechanism of enhanced kinin release from high molecular weight kininogen by plasma kallikrein after its exposure to plasmin. J Lab Clin Med 1992, 120, 129–139.
   PubMed Web of Science ®Google Scholar
• Cicardi, M., Zingale, L.C., Zanichelli, A., Deliliers, D.L., Caccia, S. The use of plasma-derived C1 inhibitor in the treatment of hereditary angioedema. Expert Opin Pharmacother 2007, 8, 3173–3181.
   PubMed Web of Science ®Google Scholar
• Bork, K., Meng, G., Staubach, P., Hardt, J. Treatment with C1 inhibitor concentrate in abdominal pain attacks of patients with hereditary angioedema. Transfusion 2005, 45, 1774–1784.
   PubMed Web of Science ®Google Scholar
• Waytes, A.T., Rosen, F.S., Frank, M.M. Treatment of hereditary angioedema with a vapor-heated C1 inhibitor concentrate. N Engl J Med 1996, 334, 1630–1634.
   PubMed Web of Science ®Google Scholar
• Bork, K. Pasteurized C1 inhibitor concentrate in hereditary angioedema: pharmacology, safety, efficacy and future directions. Expert Rev Clin Immunol 2008, 4, 13–20.
   PubMed Web of Science ®Google Scholar
• De Filippi, F., Castelli, R., Cicardi, M., Soffredini, R., Rumi, M.G., Silini, E., Mannucci, P.M., Colombo, M. Transmission of hepatitis G virus in patients with angioedema treated with steam-heated plasma concentrates of C1 inhibitor. Transfusion 1998, 38, 307–311.
   PubMed Web of Science ®Google Scholar
• Cicardi, M., Mannucci, P.M., Castelli, R., Rumi, M.G., Agostoni, A. Reduction in transmission of hepatitis C after the introduction of a heat-treatment step in the production of C1-inhibitor concentrate. Transfusion 1995, 35, 209–212.
   PubMed Web of Science ®Google Scholar
• Zuraw, B.L., Busse, P.J., White, M., Jacobs, J., Lumry, W., Baker, J., Craig, T., Grant, J.A., Hurewitz, D., Bielory, L., Cartwright, W.E., Koleilat, M., Ryan, W., Schaefer, O., Manning, M., Patel, P., Bernstein, J.A., Friedman, R.A., Wilkinson, R., Tanner, D., Kohler, G., Gunther, G., Levy, R., McClellan, J., Redhead, J., Guss, D., Heyman, E., Blumenstein, B.A., Kalfus, I., Frank, M.M. Nanofiltered C1 inhibitor concentrate for treatment of hereditary angioedema. N Engl J Med 2010, 363, 513–522.
   PubMed Web of Science ®Google Scholar
• Hofstra, J.J., Kleine Budde, I., van Twuyver, E., Choi, G., Levi, M., Leebeek, F.W., de Monchy, J.G., Ypma, P.F., Keizer, R.J., Huitema, A.D., Strengers, P.F. Treatment of hereditary angioedema with nanofiltered C1-esterase inhibitor concentrate (Cetor®): multi-center phase II and III studies to assess pharmacokinetics, clinical efficacy and safety. Clin Immunol 2012, 142, 280–290.
   PubMed Web of Science ®Google Scholar
• Longhurst, H.J., Carr, S., Khair, K. C1-inhibitor concentrate home therapy for hereditary angioedema: a viable, effective treatment option. Clin Exp Immunol 2007, 147, 11–17.
   PubMed Web of Science ®Google Scholar
• Wahn, V., Aberer, W., Eberl, W., Faßhauer, M., Kühne, T., Kurnik, K., Magerl, M., Meyer-Olson, D., Martinez-Saguer, I., Späth, P., Staubach-Renz, P., Kreuz, W. Hereditary angioedema (HAE) in children and adolescents-a consensus on therapeutic strategies. Eur J Pediatr 2012, 171, 1339–1348.
   PubMed Web of Science ®Google Scholar
• Caballero, T., Farkas, H., Bouillet, L., Bowen, T., Gompel, A., Fagerberg, C., Bjökander, J., Bork, K., Bygum, A., Cicardi, M., de Carolis, C., Frank, M., Gooi, J.H., Longhurst, H., Martínez-Saguer, I., Nielsen, E.W., Obtulowitz, K., Perricone, R., Prior, N.; C-1-INH Deficiency Working Group. International consensus and practical guidelines on the gynecologic and obstetric management of female patients with hereditary angioedema caused by C1 inhibitor deficiency. J Allergy Clin Immunol 2012, 129, 308–320.
   PubMed Web of Science ®Google Scholar
• Varga, L., Farkas, H. Treatment of type I and II hereditary angioedema with Rhucin, a recombinant human C1 inhibitor. Expert Rev Clin Immunol 2008, 4, 653–661.
   PubMed Web of Science ®Google Scholar
• Zuraw, B., Cicardi, M., Levy, R.J., Nuijens, J.H., Relan, A., Visscher, S., Haase, G., Kaufman, L., Hack, C.E. Recombinant human C1-inhibitor for the treatment of acute angioedema attacks in patients with hereditary angioedema. J Allergy Clin Immunol 2010, 126, 821–827.e14.
   PubMed Web of Science ®Google Scholar
• Caballero, T., Baeza, M.L., Cabañas, R., Campos, A., Cimbollek, S., Gómez-Traseira, C., González-Quevedo, T., Guilarte, M., Jurado-Palomo, J., Larco, J.I., López-Serrano, M.C., López-Trascasa, M., Marcos, C., Muñoz-Caro, J.M., Pedrosa, M., Prior, N., Rubio, M., Sala-Cunill, A.; Spanish Study Group on Bradykinin-Induced Angioedema (SGBA). Consensus statement on the diagnosis, management, and treatment of angioedema mediated by bradykinin. Part II. Treatment, follow-up, and special situations. J Investig Allergol Clin Immunol 2011, 21, 422–41; quiz 442.
   PubMed Web of Science ®Google Scholar
• Pappalardo, E., Zingale, L.C., Cicardi, M. Increased expression of C1-inhibitor mRNA in patients with hereditary angioedema treated with Danazol. Immunol Lett 2003, 86, 271–276.
   PubMed Web of Science ®Google Scholar
• Bork, K., Bygum, A., Hardt, J. Benefits and risks of danazol in hereditary angioedema: a long-term survey of 118 patients. Ann Allergy Asthma Immunol 2008, 100, 153–161.
   PubMed Web of Science ®Google Scholar
• Cicardi, M., Castelli, R., Zingale, L.C., Agostoni, A. Side effects of long-term prophylaxis with attenuated androgens in hereditary angioedema: comparison of treated and untreated patients. J Allergy Clin Immunol 1997, 99, 194–196.
   PubMed Web of Science ®Google Scholar
• Helsing, P., Nielsen, E.W. Hepatocellular focal nodular hyperplasia after danazol treatment for hereditary angio-oedema. Acta Derm Venereol 2006, 86, 272–273.
   PubMed Web of Science ®Google Scholar
• Bork, K., Pitton, M., Harten, P., Koch, P. Hepatocellular adenomas in patients taking danazol for hereditary angio-oedema. Lancet 1999, 353, 1066–1067.
   PubMed Web of Science ®Google Scholar
• Cicardi, M., Bork, K., Caballero, T., Craig, T., Li, H.H., Longhurst, H., Reshef, A., Zuraw, B.; HAWK (Hereditary Angioedema International Working Group). Evidence-based recommendations for the therapeutic management of angioedema owing to hereditary C1 inhibitor deficiency: consensus report of an International Working Group. Allergy 2012, 67, 147–157.
   PubMed Web of Science ®Google Scholar
• Williams, A., Baird, L.G. DX-88 and HAE: a developmental perspective. Transfus Apher Sci 2003, 29, 255–258.
   PubMed Web of Science ®Google Scholar
• Czaller, I., Visy, B., Csuka, D., Füst, G., Tóth, F., Farkas, H. The natural history of hereditary angioedema and the impact of treatment with human C1-inhibitor concentrate during pregnancy: a long-term survey. Eur J Obstet Gynecol Reprod Biol 2010, 152, 44–49.
   PubMed Web of Science ®Google Scholar
• Cicardi, M., González-Quivedo, T., Caballero, T., Bork, K., Williams, A. DX-88 a recombinant inhibitor of human plasma kallikrein: efficacy and safety in hereditary and acquired angioedema. Mol Immunol 2003, 40, 197–198.
   Web of Science ®Google Scholar
• Cugno, M., Nussberger, J., Biglioli, P., Alamanni, F., Coppola, R., Agostoni, A. Increase of bradykinin in plasma of patients undergoing cardiopulmonary bypass: the importance of lung exclusion. Chest 2001, 120, 1776–1782.
   PubMed Web of Science ®Google Scholar
• Fincham, C.I., Bressan, A., Paris, M., Rossi, C., Fattori, D. Bradykinin receptor antagonists–a review of the patent literature 2005–2008. Expert Opin Ther Pat 2009, 19, 919–941.
   PubMed Web of Science ®Google Scholar
• Bork, K., Frank, J., Grundt, B., Schlattmann, P., Nussberger, J., Kreuz, W. Treatment of acute edema attacks in hereditary angioedema with a bradykinin receptor-2 antagonist (Icatibant). J Allergy Clin Immunol 2007, 119, 1497–1503.
   PubMed Web of Science ®Google Scholar
• Cicardi, M., Banerji, A., Bracho, F., Malbrán, A., Rosenkranz, B., Riedl, M., Bork, K., Lumry, W., Aberer, W., Bier, H., Bas, M., Greve, J., Hoffmann, T.K., Farkas, H., Reshef, A., Ritchie, B., Yang, W., Grabbe, J., Kivity, S., Kreuz, W., Levy, R.J., Luger, T., Obtulowicz, K., Schmid-Grendelmeier, P., Bull, C., Sitkauskiene, B., Smith, W.B., Toubi, E., Werner, S., Anné, S., Björkander, J., Bouillet, L., Cillari, E., Hurewitz, D., Jacobson, K.W., Katelaris, C.H., Maurer, M., Merk, H., Bernstein, J.A., Feighery, C., Floccard, B., Gleich, G., Hébert, J., Kaatz, M., Keith, P., Kirkpatrick, C.H., Langton, D., Martin, L., Pichler, C., Resnick, D., Wombolt, D., Fernández Romero, D.S., Zanichelli, A., Arcoleo, F., Knolle, J., Kravec, I., Dong, L., Zimmermann, J., Rosen, K., Fan, W.T. Icatibant, a new bradykininreceptor antagonist, in hereditary angioedema. N Engl J Med 2010, 363, 532–541.
   PubMed Web of Science ®Google Scholar
• Agostoni, A., Cicardi, M. Drug-induced angioedema without urticaria. Drug Saf 2001, 24, 599–606.
   PubMed Web of Science ®Google Scholar
• Bork, K., Dewald, G. Hereditary angioedema type III, angioedema associated with angiotensin II receptor antagonists, and female sex. Am J Med 2004, 116, 644–645.
   PubMed Web of Science ®Google Scholar
• Bork, K., Fischer, B., Dewald, G. Recurrent episodes of skin angioedema and severe attacks of abdominal pain induced by oral contraceptives or hormone replacement therapy. Am J Med 2003, 114, 294–298.
   PubMed Web of Science ®Google Scholar
• Yip, J., Cunliffe, W.J. Hormonally exacerbated hereditary angioedema. Australas J Dermatol 1992, 33, 35–38.
   PubMedGoogle Scholar
• Borradori, L., Marie, O., Rybojad, M., Vexiau, P., Morel, P., Späth, P. Hereditary angioedema and oral contraception. Dermatologica 1990, 181, 78–79.
   PubMedGoogle Scholar
• Pence, H.L., Evans, R., Guernsey, L.H., Gerhard, R.C. Prophylactic use of epsilon aminocaproic acid for oral surgery in a patient with hereditary angioneurotic edema. J Allergy Clin Immunol 1974, 53, 298–302.
   PubMed Web of Science ®Google Scholar
• Sheffer, A.L., Fearon, D.T., Austen, K.F., Rosen, F.S. Tranexamic acid: preoperative prophylactic therapy for patients with hereditary angioneurotic edema. J Allergy Clin Immunol 1977, 60, 38–40.
   PubMed Web of Science ®Google Scholar
• Crosher, R. Intravenous tranexamic acid in the management of hereditary angio-oedema. Br J Oral Maxillofac Surg 1987, 25, 500–506.
   PubMed Web of Science ®Google Scholar
• Atkinson, J.C., Frank, M.M. Oral manifestations and dental management of patients with hereditary angioedema. J Oral Pathol Med 1991, 20, 139–142.
   PubMed Web of Science ®Google Scholar
• Farkas, H., Gyeney, L., Gidófalvy, E., Füst, G., Varga, L. The efficacy of short-term danazol prophylaxis in hereditary angioedema patients undergoing maxillofacial and dental procedures. J Oral Maxillofac Surg 1999, 57, 404–408.
   PubMed Web of Science ®Google Scholar
• Peled, M., Ardekian, L., Schnarch, A., Laufer, D. Preoperative prophylaxis for C1 esterase-inhibitor deficiency in patients undergoing oral surgery: a report of three cases. Quintessence Int 1997, 28, 169–171.
   PubMedGoogle Scholar
• Lehmann, A., Lang, J., Boldt, J., Saggau, W. Successful off-pump coronary artery bypass graft surgery in a patient with hereditary angioedema. J Cardiothorac Vasc Anesth 2002, 16, 473–476.
   PubMed Web of Science ®Google Scholar
• Castelli, R., Cicardi, M., Gardinali, M., Zingale, L.C., Savi, C., Munari, M., Agostoni, A. Cardiopulmonary by-pass in a patient with acquired C1 inhibitor deficiency. Int J Artif Organs 1997, 20, 175–177.
   PubMed Web of Science ®Google Scholar
• Donaldson, V.H., Bernstein, D.I., Wagner, C.J., Mitchell, B.H., Scinto, J., Bernstein, I.L. Angioneurotic edema with acquired C1- inhibitor deficiency and autoantibody to C1- inhibitor: response to plasmapheresis and cytotoxic therapy. J Lab Clin Med 1992, 119, 397–406.
   PubMed Web of Science ®Google Scholar
• Chevailler, A., Arlaud, G., Ponard, D., Pernollet, M., Carrère, F., Renier, G., Drouet, M., Hurez, D., Gardais, J. C-1-inhibitor binding monoclonal immunoglobins in three patients with acquired angioneurotic edema. J Allergy Clin Immunol 1996, 97, 998–1008.
   PubMed Web of Science ®Google Scholar
• Wasserfallen, J.B., Spaeth, P., Guillou, L., Pécoud, A.R. Acquired deficiency in C1-inhibitor associated with signet ring cell gastric adenocarcinoma: a probable connection of antitumor-associated antibodies, hemolytic anemia, and complement turnover. J Allergy Clin Immunol 1995, 95, 124–131.
   PubMed Web of Science ®Google Scholar
• Zingale, L.C., Castelli, R., Zanichelli, A., Cicardi, M. Acquired deficiency of the inhibitor of the first complement component: presentation, diagnosis, course, and conventional management. Immunol Allergy Clin North Am 2006, 26, 669–690.
   PubMed Web of Science ®Google Scholar
• Cicardi, M., Bisiani, G., Cugno, M., Späth, P., Agostoni, A. Autoimmune C1 inhibitor deficiency: report of eight patients. Am J Med 1993, 95, 169–175.
   PubMed Web of Science ®Google Scholar
• Alsenz, J., Lambris, J.D., Bork, K., Loos, M. Acquired C1 inhibitor (C1-INH) deficiency type II. Replacement therapy with C1-INH and analysis of patients’ C1-INH and anti-C1-INH autoantibodies. J Clin Invest 1989, 83, 1794–1799.
   PubMed Web of Science ®Google Scholar
• Zanichelli, A., Badini, M., Nataloni, I., Montano, N., Cicardi, M. Treatment of acquired angioedema with icatibant: a case report. Intern Emerg Med 2011, 6, 279–280.
   PubMed Web of Science ®Google Scholar
• Branellec, A., Bouillet, L., Javaud, N., Mekinian, A., Boccon-Gibod, I., Blanchard-Delaunay, C., Oksenhendler, E., Ollivier, Y., Dunogué, B., Amarger, S., Ponard, D., Drouet, C., Mouthon, L., Thomas, M., Fain, O.; on behalf of the French National Reference Center for Angioedema (CREAK). Acquired C1-Inhibitor Deficiency: 7 Patients Treated with Rituximab. J Clin Immunol 2012, 32, 936–941.
   PubMed Web of Science ®Google Scholar
• Lam, D.H., Levy, N.B., Nickerson, J.M., Gruenberg, D.A., Lansigan, F. Acquired angioedema and marginal zone lymphoma. J Clin Oncol 2012, 30, e151–e153.
   PubMed Web of Science ®Google Scholar