A 25 year old women presents with a history of recurrent shortness of breath and severe wheezing. Laboratory studies demonstrate that she a has a deficiency of C1 inhibitor, an esterase inhibitor that regulates the activation of the classical complement pathway. What is the diagnosis?
Correct Answer: Hereditary angiodema
Description: Deficiency of C1 inhibitor, with excessive clearage of C4 and C2 by CLS, is associated with the syndrome of hereditory angioedema. This disease is characterised by episodic, painless, non pitting edema of soft tissue. It is the result of chronic complement activation, with the generation of a vasoactive peptide form C2 and may be life threatening because of the occurence of laryngeal edema.
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Category:
Pathology
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