# Prediction of hereditary angioedema during attacks in patients with recurrent angioedema: Awareness at a glance with the hereditary angioedema prediction score

## TL;DR

This paper introduces a scoring system to help doctors quickly identify hereditary angioedema during attacks, improving diagnosis accuracy.

## Contribution

The novel contribution is the development of the Hereditary Angioedema Prediction Score (HAEps) for diagnosing HAE during acute attacks.

## Key findings

- The HAEps scoring system achieved 96.4% sensitivity and 96.1% specificity in predicting HAE.
- Key predictive items included absence of urticaria and unresponsiveness to antihistaminergic treatments.
- The scoring system was validated using 106 HAE and 155 mast cell-mediated angioedema patients.

## Abstract

Misdiagnosis of hereditary angioedema (HAE) leads to inappropriate management of the attacks. A scoring system that anticipates diagnosis can be beneficial for clinicians who are unfamiliar with angioedema. This study aims to develop a practical scoring system for use during acute attacks to predict HAE in patients with recurrent angioedema (RAE).

To predict HAE, nine HAE experts unanimously identified five predictive items (PIs); absence of urticaria, presence of abdominal pain episodes, family history, early onset of attacks and previous unresponsiveness to anti‐histaminergic treatments. The researchers questioned 106 patients with HAE and 155 patients with mast cell‐mediated angioedema (MMAE) about PIs. A score was attributed to each significant PI based on OR values obtained through logistic regression analysis. The cut‐off point for the prediction of HAE and its sensitivity and specificity were determined by ROC curve analysis.

In a univariate analysis, all items showed significant differences between HAE and MMAE patients. Regression analysis attributed scores as follows: 23 points for the absence of urticaria, 11 points for the abdominal pain episodes, 9 points for family history, and 53 points for unresponsiveness to antihistaminergic treatments. No score was attributed to early onset of age (p > 0.05). The ROC analysis revealed an area under the curve of 0.990, with a total score of ≥38 demonstrating the best sensitivity (96.4%) and specificity (96.1%).

HAEps is a valuable tool for diagnosing HAE in patients with RAE. A score of 38 or more indicates the possible presence of HAE with substantial sensitivity and specificity.