Interferons-alpha, -beta and -gamma (IFNs-alpha, -beta and -gamma) stimulated the synthesis of the second complement component (C2), Factor B (B) and C1 inhibitor (C1-inh) by human monocytes in vitro. The degree of increase of the secretion rates of C2, B and C1-inh was dose-dependent and proportional to increases in the abundances of their respective mRNAs. IFN-gamma was the most effective at ...

Hereditary angioedema (HAE), a rare autosomal dominant disorder, was first described in the late 19th century. The disease remained poorly understood and without therapeutic options until the latter half of the 20th century. Advances in the understanding of immunologic and hematologic pathways have shed light on HAE, a disease characterized by painful and unpredictable recurrent attacks of nonp...

INTRODUCTION Administration of human C1 esterase inhibitor (Berinert(®) P) from target import is the most widespread treatment strategy for patients with hereditary angioedema (HAE). However, a therapeutic health program including Ruconest(®) (conestat alfa) could shorten a patient's expectancy for a life-saving treatment. AIM To evaluate the cost-utility of Ruconest(®) (conestat alfa) financ...

Heparin is a naturally occurring polysaccharide known to interact with complement proteins and regulate multiple steps in the complement cascade. Quantitative information, in the form of affinity constants for heparin-complement interactions, is not generally available and there are no reports of a comprehensive analysis using the same interaction method. Such information should improve our und...

Almost two decades ago, a new superfamily of proteins was proposed, based on the similarity between the primary structure of ovalbumin, a1-antitrypsin (also known as a1-proteinase inhibitor) and antithrombin III (1). Even though the acronym “serpins” (serine proteinase inhibitors) was given to these proteins (2), it became soon apparent that a variety of non-inhibitory serpins [e.g. ovalbumin (...

INTRODUCTION We report angioedema as a rare presentation leading to a diagnosis of systemic lupus erythematosus (SLE). CLINICAL PICTURE A diagnosis of angioedema was delayed in a patient presenting with limb and facial swelling until she developed acute upper airway compromise. After excluding allergic and hereditary angioedema, acquired angioedema (AAE) was suspected, possibly precipitated b...

The activated first component of complement (CI) possesses an esterase activity in vitro which will hydrolyse an ester of tyrosine to release H+. The activity of the serum inhibitor of C1 esterase may be measured by monitoring its ability to prevent H+ release under standard conditions. This paper describes a method of measuring such activity, monitoring H+ release by the use of either an acid ...