Ultrasound findings in an abdominal crisis of a patient with hereditary angioedema.

ereditary Angioedema (HAE) is a rare autosomal-dominant disease caused by serum C1 inhibitor deficiency. This deficiency leads to an up-regulation of complement, activating the bradykinin pathway and causing vascular permeability and subsequent mucosal edema. Abdominal angioedema is a less recognized type of angioedema and the clinical signs may range from subtle, diffuse abdominal pain and nausea, to overt peritonitis.

[Algorithm for diagnosis and treatment of hereditary angioedema as a tool for management].

Introduction: Hereditary angioedema is a disease with low prevalence and high heterogeneity with regards to the severity of the clinical picture, which makes the diagnosis difficult and requires the need for early start of specific treatment in order to prevent complications.

Objective: To propose a decision algorithm for hereditary angioedema (HAE), based on the evidence available on the diagnosis, clinical assessment, and treatment. The aim is to present the available therapeutic options as well as a decision algorithm to select the most efficient therapy at each time.