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Complement is part of the innate immune response, and dysfunctional complement regulation leads to a number of serious systemic disease conditions. Endogenous regulators that protect host tissue from damage include the soluble serum complement regulatory proteins Factor H (FH), FHL1/2, FHR1, and CD46/MCP. CD46 is a highly expressed extracellular glycoprotein present on all human cells with the exception of red blood cells. The main ligand for CD46 is C3b, and CD46 regulates complement activity mainly by serving as a co-factor for Factor I which cleaves C3b and inactivates complement. Here we present two distinct patients with a new CD46 mutation.

Patients and methods
====================

Two patients presented with onset of severe pain and inflammation during their fifth decade. The differential diagnoses were Wegener’s granulomatosis and bacterial infection of the joint. At the age of 50 years, both patients developed hemoptysis that only partially responded to methylprednisolone treatment. Family members of these patients were examined, and the diagnosis of hereditary angioedema (HAE) was made. Because of the relatively low

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