# Tumor Necrosis Factor Receptor-Associated Periodic Syndrome: Genetics, Autoinflammation, and Recurrent Pericarditis

## TL;DR

This paper discusses a rare genetic disorder called TRAPS that causes recurring inflammation and how it can lead to pericarditis, which was successfully treated with a specific drug.

## Contribution

The paper presents a case where TRAPS was identified as the cause of idiopathic recurrent pericarditis and highlights the effectiveness of rilonacept treatment.

## Key findings

- TRAPS can manifest as recurrent pericarditis and should be considered in diagnostic evaluations.
- Rilonacept was more effective than canakinumab in treating RP in this patient.
- Interleukin-1 inhibitors are promising treatments for RP linked to autoinflammatory diseases.

## Abstract

Tumor necrosis factor receptor–associated periodic syndrome (TRAPS) is a rare hereditary autoinflammatory disorder caused by mutations in the tumor necrosis factor superfamily member 1A gene, which encodes tumor necrosis factor receptor 1. It typically presents with recurrent fever and other signs of inflammation.

A 26-year-old Hispanic woman presented with recurrent fever, tachycardia, and pleuritic chest pain. She was diagnosed with recurrent pericarditis (RP) secondary to TRAPS. She showed an excellent clinical response upon switching from canakinumab to rilonacept.

RP is often labeled idiopathic, but autoinflammatory diseases such as TRAPS should be considered in such cases on the basis of the clinical presentation. Interleukin-1 inhibitors have emerged as promising treatments for RP, with radical pericardiectomy being considered as a last resort.