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Validation of the Index for Facial Angiofibromas: Data analysis from a randomized controlled trial of sirolimus gel

Izumi Hamada¹, Yoshinori Yukutake¹, Yusuke Morita¹

¹Research & Development Division, Nobelpharma Co., Ltd, Tokyo, Japan.

The Journal of Dermatology

|April 15, 2024

Summary

The Index for Facial Angiofibromas (IFA) scoring system shows high reliability and validity for assessing angiofibromas in tuberous sclerosis complex (TSC) patients. A decrease of 1.667 or more in IFA score indicates a clinically meaningful improvement.

Keywords:

angiofibroma sirolimus topical administration tuberous sclerosis validation study

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Area of Science:

Dermatology
Clinical Trials
Biostatistics

Background:

Facial angiofibromas are a common manifestation of tuberous sclerosis complex (TSC).
Accurate assessment of treatment efficacy for facial angiofibromas is crucial.
The Index for Facial Angiofibromas (IFA) is a novel scoring system designed for this purpose.

Purpose of the Study:

To further validate the Index for Facial Angiofibromas (IFA) using data from a Phase III clinical trial.
To assess the reliability and clinical meaningfulness of the IFA score in patients with TSC treated with topical sirolimus.

Main Methods:

Analysis of photographs from a Phase III clinical trial (NCT02635789) involving 62 TSC patients randomized to topical sirolimus or placebo.
Independent assessment of angiofibromas using the primary composite endpoint (Facial Angiofibroma Severity Index - FASI) and the IFA.
Receiver operating characteristic (ROC) analysis to determine thresholds for clinically meaningful change in IFA score.

Main Results:

The IFA demonstrated good-to-excellent inter-assessor and very high intra-assessor reliability.
High correlations were found between categorized changes in IFA scores and the primary composite endpoint (W=0.8655, p<0.0001).
An optimal IFA cut-off of ≥1.667 was identified to signify clinically meaningful improvement (AUC=0.937).

Conclusions:

The Index for Facial Angiofibromas (IFA) is a valid and reliable tool for assessing angiofibromas in TSC patients.
A decrease in IFA score of ≥1.667 may represent a clinically meaningful treatment outcome.
The IFA can be utilized to evaluate disease severity distribution and treatment response in clinical trials.