Thromb Haemost 2006; 95: 898–9 Deficiency of the C1-inhibitor (C1-INH) is a very rare cause of angioedema. A hereditary (1) and an acquired form (2, 3) of C1-INH deficiency have been described. Two types of acquired angioedema (AAE) exist: type I is associated with lymphoproliferative disorders, whereas type II is characterized by autoantibodies against C1-INH (4). Clinically, AAE is indistingu...

The spirochete Borrelia recurrentis is the causal agent of louse-borne relapsing fever and is transmitted to humans by the infected body louse Pediculus humanus. We have recently demonstrated that the B. recurrentis surface receptor, HcpA, specifically binds factor H, the regulator of the alternative pathway of complement activation, thereby inhibiting complement mediated bacteriolysis. Here, w...

The StcE zinc metalloprotease is secreted by enterohemorrhagic Escherichia coli (EHEC) O157:H7 and contributes to intimate adherence of this bacterium to host cells, a process essential for mammalian colonization. StcE has also been shown to localize the inflammatory regulator C1 esterase inhibitor (C1-INH) to cell membranes. We tried to more fully characterize StcE activity to better understan...

Proteolytic inactivation of serine protease inhibitors (serpins) by neutrophil elastase (HNE) is presumed to contribute to the deregulation of plasma cascade systems in septic shock. Here, we report a supplementary approach to construct serpins, in our case C1 inhibitor, that are resistant to catalytic inactivation by HNE. Instead of shifting the specificity of alpha 1-antitrypsin towards the p...

Spondyloarthritis (SpA) encompasses a group of rheumatologic diseases, including axial spondyloarthritis (axSpA), psoriatic arthritis, arthritis with associated inflammatory bowel disease (i.e. Crohn’s and ulcerative colitis), reactive undifferentiated SpA, which all share certain clinical, biological genetic features. However, the pathogenesis remains largely unexplained. Recent evidence sugge...

Although both the complement and contact system are thought to contribute to the inflammatory reaction in arthritic joints, only activation of complement has so far been well established, whereas contact activation and its contribution to arthritis has not been systematically explored. Complement and contact activation were assessed in 71 patients with inflammatory arthropathies and 11 with ost...