Grumpy-XL schreef op 24 augustus 2023 10:06:
angioedemanews.com/news/survey-us-doc..."The number of cases of hereditary angioedema with normal C1 inhibitor (HAE-nl-C1INH) within the U.S. was estimated to be between 1,230 and 1,331 from May 2019 and April 2020, according to a survey conducted among U.S. physicians."
"Commonly used medications
Physicians commonly used patients’ response to medication as a tool for diagnosis, particularly antihistamines (used by 73% of physicians), corticosteroids (57% of physicians), or HAE-specific medications (74% of physicians).
Among physicians who reported using patient responses to HAE-specific medications to inform HAE-nl-C1INH diagnosis, Firazyr (icatibant) was the most commonly used medication (87% of those physicians). Firazyr is a medicine normally used to treat acute angioedema attacks.
Genetic testing in the F12 gene was reported by 43% of physicians as a tool to diagnose HAE-nl-C1INH, while other types of genetic testing were reported by 5% or less.
“The development of more reliable, validated, and easily accessible methods to diagnose HAE-nl-C1INH accurately will also facilitate more rigorous evaluation of the efficacy and safety of available and future therapies,” the researchers wrote.
Regarding treatment for acute HAE-nl-C1INH symptoms, Firazyr was the most prescribed medication (73% of physicians), followed by high-dose antihistamines (15%). Other prescribed HAE-specific treatments included Ruconest (13%), Berinert (10%), and Kalbitor (8%).
Physicians reported 54% of the patients with HAE-nl-C1INH received long-term preventive treatment. Those patients experienced a mean of 16 attacks per year before starting treatment.
The main reasons for recommending initiating preventive treatment were the frequency, severity, and predictability of acute attacks, followed by patient request. The most commonly prescribed preventive treatment was Takhzyro (lanadelumab, 40% of physicians), followed by Haegarda (17% of physicians) and Cinryze (8% of physicians).
“To our knowledge, this is the first study surveying US HAE-treating physicians to estimate the prevalence of patients with HAE-nl-C1INH and to provide a comprehensive overview of how patients with HAE-nl-C1INH are given a diagnosis and treated in the United States,” the researchers wrote. “In addition, we were able to assess the most common management strategies and potential unmet needs associated with acute and preventive treatment.”"