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• W2147726146 abstract "Acquired deficiency of C1 inhibitor is a relatively rare but serious disorder, causing frequently occurring, severe and sometimes life-threatening angioedema.1Agostoni A. Cicardi M. Hereditary and acquired C1-inhibitor deficiency biological and clinical characteristics in 235 patients.Medicine (Baltimore). 1992; 71: 206-215Crossref PubMed Scopus (559) Google Scholar, 2Markovic S.N. Inwards D.J. Frigas E.A. Phyliky R.P. Acquired C1 esterase inhibitor deficiency.Ann Intern Med. 2000; 132: 144-150Crossref PubMed Scopus (113) Google Scholar The deficiency of C1 inhibitor may be caused by the formation of auto-antibodies (type II) but may also occur in the setting of lymphoproliferative disorders (type I).3Jackson J. Sim R.B. Whelan A. Feighery C. An IgG autoantibody which inactivates C1-inhibitor.Nature. 1986; 323: 722-724Crossref PubMed Scopus (143) Google Scholar, 4Cicardi M. Zingale L.C. Pappalardo E. Folcioni A. Agostoni A. Autoantibodies and lymphoproliferative diseases in acquired C1-inhibitor deficiencies.Medicine (Baltimore). 2003; 82: 274-281PubMed Google Scholar In the latter case, at least 2 mechanisms have been proposed to explain the decrease of C1 inhibitor. One involves anti-C1-inhibitor specificity of the antibody produced by the malignant B-cell clone; the other assumes consumption of C1 inhibitor by excessive activation of C1, the first complement factor, by immune complexes consisting of the idiotype of the antibody produced by the malignant clone and anti-idiotype antibodies. The ongoing consumption of C1 inhibitor in the latter situation will cause a deficiency of this protease inhibitor.5Carugati A. Pappalardo E. Zingale L.C. Cicardi M. C1-inhibitor deficiency and angioedema.Mol Immunol. 2001; 38: 161-173Crossref PubMed Scopus (98) Google Scholar It is becoming increasingly clear that many of the patients with lymphoproliferative disorders have specific anti-C1 inhibitor antibodies, making the distinction between type I and II forms of acquired C1-inhibitor deficiency less explicit.6D’Incan M. Tridon A. Ponard D. et al.Acquired angioedema with C1 inhibitor deficiency is the distinction between type I and type II still relevant?.Dermatology. 1999; 199: 227-230Crossref PubMed Scopus (38) Google Scholar, 7Cicardi M. Beretta A. Colombo M. Gioffre D. Cugno M. Agostoni A. Relevance of lymphoproliferative disorders and of anti-C1 inhibitor autoantibodies in acquired angio-oedema.Clin Exp Immunol. 1996; 106: 475-480Crossref PubMed Scopus (90) Google Scholar Patients with acquired C1-inhibitor deficiency can sometimes be successfully managed by treatment of the underlying disorder, and patients in which no such underlying disease is diagnosed or who do not require specific treatment for this disease are treated with androgenic steroids, such as danazol (which stimulates hepatic production of C1-inhibitor)5Carugati A. Pappalardo E. Zingale L.C. Cicardi M. C1-inhibitor deficiency and angioedema.Mol Immunol. 2001; 38: 161-173Crossref PubMed Scopus (98) Google Scholar or with lysine analogues, such as epsilon caproic acid or tranexamic acid (which act by inhibition of plasmin formation, that is important in the pathogenesis of angioedema).4Cicardi M. Zingale L.C. Pappalardo E. Folcioni A. Agostoni A. Autoantibodies and lymphoproliferative diseases in acquired C1-inhibitor deficiencies.Medicine (Baltimore). 2003; 82: 274-281PubMed Google Scholar In case of severe angioedema (for example, located in the upper airways or severe abdominal attacks) administration of C1-inhibitor concentrate (or large amounts of plasma) may be considered.5Carugati A. Pappalardo E. Zingale L.C. Cicardi M. C1-inhibitor deficiency and angioedema.Mol Immunol. 2001; 38: 161-173Crossref PubMed Scopus (98) Google Scholar Treatment with immunosuppressive agents in patients with autoimmune C1 inhibitor deficiency is successful in some but not all cases.8Chevailler A. Arlaud G. Ponard D. et al.C-1-inhibitor binding monoclonal immunoglobins in three patients with acquired angioneurotic edema.J Allergy Clin Immunol. 1996; 97: 998-1008Abstract Full Text PDF PubMed Scopus (41) Google Scholar Although the majority of patients can be managed successfully, some patients are resistant to treatment, even despite frequent administration of C1-inhibitor concentrate, leading to very frequent attacks of severe angioedema.9Agostoni A. Aygoren-Pursun E. Binkley K.E. et al.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-S131Abstract Full Text Full Text PDF PubMed Scopus (562) Google Scholar These attacks may be life-threatening and obviously have a major impact on morbidity and quality of life. Here we describe 3 patients with such a therapy-resistant severe acquired deficiency of C1 inhibitor, who show a normalization of C1-inhibitor plasma levels and a complete remission of angioedema attacks after treatment with anti-CD20 monoclonal antibodies. Three patients with acquired angioedema had very frequent (>1/10 days) angioedema attacks despite treatment with danazol (150-300 mg/day), alone or in combination with tranexamic acid (2-3 g daily). Severe angioedema attacks (defined as an attack in the upper airway or a serious abdominal attack) were treated with intravenous administration of 1000 U of plasma-derived C1-inhibitor concentrate (Sanquin, Amsterdam, the Netherlands). The 3 patients had high requirements for C1-inhibitor concentrate administration (mean consumption 2500-9000 U/month). The diagnostic criteria for acquired angioedema were based on the clinical presentation with recurrent attacks of angioedema, the absence of a family history of C1-inhibitor deficiency, onset of the angioedema attacks at age >25 years, a low level of C1-inhibitor (<0.5 U/mL) and C4 (<100 mg/L) in plasma, low levels of C1q (<80 IU/mL), the presence of anti-C1-inhibitor antibodies (optionally), and a diagnosis of a lymphoproliferative disorder (optionally). One of the patients (patient A, female, age 45 years) had positive anti-C1-inhibitor IgG antibodies. One patient (patient B, female, age 70 years) was recently diagnosed with an indolent follicular B-cell lymphoma, stage IV, with localization in spleen, bone marrow and peripheral blood, for which no specific treatment was given so far, and in 1 patient (patient C, male, age 68 years) no underlying cause for the C1-inhibitor deficiency was found. Patient A and patient C were diagnosed with acquired C1-inhibitor deficiency for 12 and 7 years, respectively. In an attempt to reduce the frequency and severity of the angioedema attacks, patient A and C were treated with prednisolone (50 mg/day) in combination with cyclophosphamide (150 mg/day). However, these treatment regimens had no effect at all on the clinical course or C1-inhibitor levels in plasma during 3-6 months follow-up. Hereupon and after provision of informed consent by the patients, all 3 patients were treated with anti-CD20 monoclonal antibodies (Rituximab, Roche, the Netherlands, 375 mg/m2, once every week for a total of 4 doses). After 3-4 months following this treatment, in all 3 patients the frequency of angioedema attacks was drastically reduced, along with a marked reduction in C1-inhibitor concentrate requirement, and after 8-10 months, patients were completely free from any angioedema attack (Figure). The clinical improvement was associated with increasing levels of C1-inhibitor in plasma to (near) normal levels at 10-12 months after treatment and a normalization of C4 levels in 2 of 3 patients (Figure). No adverse effects of the treatment were noted in the patients, except for some mild diffuse muscle and joint pain in 1 patient starting after the fourth administration of anti-CD20 antibodies and persisting for 4-5 months. We here show successful treatment of acquired angioedema with administration of anti-CD20 antibodies in 3 patients. Patients with very frequent attacks of severe angioedema requiring high doses of C1-inhibitor concentrate became asymptomatic 3-4 months after anti-CD20 treatment. Although we realize that this type of case series with successful outcome does not provide the ultimate proof of efficacy of this treatment for patients with acquired C1-inhibitor deficiency, we feel the results are rather compelling and suggest that further study is warranted. It is not very likely that spontaneous remission of acquired C1-inhibitor deficiency would have occurred in the 2 patients with a very long history of this disorder or in the untreated patient with acquired angioedema and the indolent B-cell lymphoma. Another limitation of this observation is that it is not yet clear whether the effect of CD20 antibody treatment is definitive or that relapse of the disease will occur. So far, patients have been followed for a mean period of 12 months without signs of recurrence of angioedema, but we realize that a longer follow-up period may be required. Nevertheless, when a relapse occurs, repeat treatment with anti-CD20 antibodies is an option to be considered. Rituximab is a chimeric antibody directed toward human CD20, and its administration leads to a transient elimination of CD20-positive B-cells by thus far not completely understood mechanisms, including antibody-dependent cellular cytotoxicity, complement activation, and induction of B-cel apoptosis.10Smith M.R. Rituximab (monoclonal anti-CD20 antibody) mechanisms of action and resistance.Oncogene. 2003; 22: 7359-7368Crossref PubMed Scopus (627) Google Scholar, 11Silverman G.J. Weisman S. Rituximab therapy and autoimmune disorders prospects for anti-B cell therapy.Arthritis Rheum. 2003; 48: 1484-1492Crossref PubMed Scopus (342) Google Scholar This CD20-directed treatment has been shown to be highly effective in the (adjunctive) treatment of lymphoproliferative disorders, in particular B-cell lymphomas.12McLaughlin P. Grillo-Lopez A.J. Link B.K. et al.Rituximab chimeric anti-CD20 monoclonal antibody therapy for relapsed indolent lymphoma half of patients respond to a four-dose treatment program.J Clin Oncol. 1998; 16: 2825-2833Crossref PubMed Scopus (2566) Google Scholar, 13Coiffier B. Lepage E. Briere J. et al.CHOP chemotherapy plus rituximab compared with CHOP alone in elderly patients with diffuse large-B-cell lymphoma.N Engl J Med. 2002; 346: 235-242Crossref PubMed Scopus (4448) Google Scholar In addition, rituximab also has been shown to be effective in therapy-resistant autoimmune diseases,14Edwards J.C. Cambridge G. Prospects for B-cell-targeted therapy in autoimmune disease.Rheumatology (Oxford). 2005; 44: 151-156Crossref PubMed Scopus (71) Google Scholar such as autoimmune thrombocytopenia,15Stasi R. Pagano A. Stipa E. Amadori S. Rituximab chimeric anti-CD20 monoclonal antibody treatment for adults with chronic idiopathic thrombocytopenic purpura.Blood. 2001; 98: 952-957Crossref PubMed Scopus (510) Google Scholar systemic lupus erythematosus (SLE),16Looney R.J. Anolik J.H. Campbell D. et al.B cell depletion as a novel treatment for systemic lupus erythematosus a phase I/II dose-escalation trial of rituximab.Arthritis Rheum. 2004; 50: 2580-2589Crossref PubMed Scopus (695) Google Scholar or rheumathoid arthritis.17Edwards J.C. Szczepanski L. Szechinski J. et al.Efficacy of B-cell-targeted therapy with rituximab in patients with rheumatoid arthritis.N Engl J Med. 2004; 350: 2572-2581Crossref PubMed Scopus (2164) Google Scholar A recent report suggested that administration of Rituximab in a patient with diffuse B-cell lymphoma also affected the occurrence of angioedema attacks in this patient.18Ziakas P.D. Giannouli S. Psimenou E. Evangelia K. Tzioufas A.G. Voulgarelis M. Acquired angioedema a new target for rituximab.Haematologica. 2004; 89: ELT13PubMed Google Scholar In our experience presented here, anti-CD20 treatment was effective in both auto-antibody-associated C1-inhibitor deficiency and in the patients with C1-inhibitor deficiency in the framework of a lymphoproliferative disorder. It is likely that in both situations the point of impact of anti-CD20 antibodies must be the antibody producing B-cell clone. Importantly, as mentioned before, the distinction between the 2 types of acquired angioedema is rather unclear and many overlap situations may occur. In conclusion, we here demonstrate successful treatment of severe and conventional treatment-resistant acquired angioedema due to deficiency of C1 inhibitor with the administration of anti-CD20 antibodies. Additional experience, and preferably, a controlled trial of anti-CD20 antibody treatment in patients with acquired C1 inhibitor deficiency, is required to confirm these observations, although we realize that execution of a clinical trial may be difficult in view of the limited number of patients." @default.
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• W2147726146 title "Rituximab-induced Elimination of Acquired Angioedema Due to C1-Inhibitor Deficiency" @default.
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