(Vienna, 16 May 2025) Hereditary angioedema (HAE) is a rare, genetic disorder characterised by recurrent episodes of painful and potentially life-threatening swelling of the skin and mucous membranes. Treatment has improved significantly in recent years. Newly approved drugs for the long-term prevention of swelling attacks offer hope to patients who have not responded to previously available treatment options. In order to provide all those affected with the best possible individual care and enable them to lead a near-normal life, an expert forum has recently been established under the leadership of Tamar Kinaciyan from the Department of Dermatology at MedUni Vienna. 16 May is World Hereditary Angioedema Day.
The enormous progress in recent years has made it necessary to pool specialist expertise in order to provide both patients and doctors with specialists´ knowledge who are located in HAE centers. Until 2010, the primary focus of medical care for hereditary angioedema was on saving the lives of those affected when a swelling attack occurred, especially in the larynx area. It is only since 2018 that drugs (such as Lanadelumab pre-filled SC injection, Berotralstat oral and human C1INH SC) have gradually become available on the market for the prophylaxis of swelling attacks. A few days ago, another long-term prophylaxis (Garadacimab) became available in Austria, which patients can administer themselves using a pen. In a few months, the approval of the active ingredient donidalorsen will provide another treatment option. "All of these modern therapies reduce both the number and severity of swelling episodes and contribute significantly to improving the quality of life of patients," explains HAE specialist Tamar Kinaciyan.
Hereditary angioedema is a rare, genetic disorder characterised by recurrent episodes of painful and potentially life-threatening swelling of the skin and mucous membranes. The face, extremities and gastrointestinal tract are often affected. Swelling episodes in the larynx are particularly feared, as they can lead to acute airway obstruction and death by suffocation within 20 minutes. The often-unexpected swelling attacks and the associated pain and disfigurement lead to a massive reduction in quality of life. "Thanks to significant recent medical advances, we can now enable most HAE patients to lead a near-normal life," says Tamar Kinaciyan from the Department of Dermatology at MedUni Vienna, who has been instrumental in researching the disease and developing therapies. "The constantly expanding range of therapies makes close interdisciplinary care in specialised centers essential in order to find the most suitable treatment option for each individual patient." The newly established HAE Expert Forum, which includes all Austrian HAE centers and specialists, aims to support this effort.