C1-inhibitor (C1-Inh) is an important inhibitor of the inflammatory response and deficiency of this inhibitor, which may be hereditary or acquired, is associated with recurrent episodes of edema. Recently, an autoimmune form of angioedema has been described that is associated with functional deficiency of C1-Inh and an autoantibody that impedes C1-Inh function. In this report we describe the is...

Acute treatment of hereditary angioedema due to C1 inhibitor deficiency has become available in the last 10 years and has greatly improved patients' quality of life. Two plasma-derived C1 inhibitors (Berinert and Cinryze), a recombinant C1 inhibitor (Ruconest/Conestat alpha), a kallikrein inhibitor (Ecallantide), and a bradykinin B2 receptor inhibitor (Icatibant) are all effective. Durably good...

The reactive center of C1-inhibitor, a plasma protease inhibitor that belongs to the serpin superfamily, is located on a peptide loop which is highly susceptible to proteolytic cleavage. With plasma kallikrein, C1s and beta-Factor XIIa, this cleavage occurs at the reactive site residue P1 (Arg444); with neutrophil elastase, it takes place near P1, probably at residue P3 (Val442). After these cl...

Hereditary angioedema (HAE), is an autosomal immunological disease, that is characterized by repetitive diffuse non-pitting painless edema without urticaria or pruritus. HAE is a rare disease with wide range of manifestation, thus diagnosis can be easily missed. Screening for HAE should be warranted in any patient with following manifestations; recurrent self-limiting unexplained abdominal pain...

Angioedema due to acquired deficiency of the C1-inhibitor is a bridging condition between autoimmunity and lymphoproliferation. We report 32 patients with acquired C1 inhibitor deficiency: 23 have anti C1-inhibitor autoantibodies; 13 have monoclonal gammopathies of unknown significance and 9 have non-Hodgkin's lymphoma. Our series suggest that different forms of B cell disorders coexist and/or ...

Background Hereditary angioedema (HAE) is a rare disorder characterized by a congenital deficiency of the C1-inhibitor. It is a potentially fatal illness as laryngeal edema may lead to asphyxiation. Treatment of HAE is mainly performed with C1-inhibitor concentrate, icatibant, attenuated androgens, and antifibrinolytic agents. We conducted a systematic review to evaluate the efficacy and safety...

Background Hereditary angioedema due to C1 inhibitor deficiency (C1-INH-HAE) is a rare disease characterized by recurrent episodes of cutaneous, abdominal and laryngeal edema. Bradykinin is the mediator of increased vascular permeability and edema formation. Treatment of HAE attacks in Italy is based on the administration of human C1 inhibitor or bradykin receptor antagonist. A recombinant huma...

Activated complement contributes significantly to reperfusion injury after ischemia. This study explores functional consequences of C1-esterase inhibitor (C1-INH) treatment after superior mesenteric artery occlusion (SMAO)/reperfusion using intravital microscopy. Thirty anesthetized, spontaneously breathing, male Sprague-Dawley rats underwent SMAO for 60 min followed by reperfusion (4 h). C1-es...

Hereditary angioedema is characterized by sudden episodes of nonpitting edema that cause discomfort and pain. Typically the extremities, genitalia, trunk, gastrointestinal tract, face, and larynx are affected by attacks of swelling. Laryngeal swelling carries significant risk for asphyxiation. The disease results from mutations in the C1 esterase inhibitor gene that cause C1 esterase inhibitor ...

Complement 1 (C1) inhibitor is an acute phase protein with anti-inflammatory properties. The aim of the present study was to elucidate the relationship between brachial ankle pulse wave velocity (baPWV), the parameter of arterial stiffness, and C1 inhibitor. One hundred subjects were randomly enrolled in this study. Data about baPWV, age, gender, hypertension, smoking, and body mass index (BMI)...