What is Hyper-IgD Syndrome / Hyperimmunoglobulinemia D With Recurrent Fever (HIDS)

Hyper-IgD Syndrome (HIDS), also known as Hyperimmunoglobulinemia D With Recurrent Fever, is a rare genetic disorder that affects the immune system. It is characterized by recurrent episodes of fever accompanied by various symptoms such as abdominal pain, joint pain, headache, and skin rashes.

HIDS is caused by mutations in the mevalonate kinase gene, which leads to the overproduction of a protein called interleukin-1 beta. This excessive production triggers inflammation in the body, resulting in the recurrent fevers and other associated symptoms.

The fever episodes typically last for a few days and recur every few weeks or months. They can be triggered by various factors such as stress, infections, vaccinations, or changes in temperature. Between the episodes, individuals with HIDS usually have no symptoms and appear healthy.

Diagnosis of HIDS is based on clinical symptoms, genetic testing, and elevated levels of immunoglobulin D in the blood. Treatment aims to manage symptoms and prevent complications. This may involve the use of medications to reduce inflammation and control fever, as well as measures to prevent triggers and maintain overall health.

While HIDS can significantly impact an individual's quality of life, with proper management and support, most people with this condition can lead relatively normal lives.