Lanadelumab i l’inhibidor de la C1 esterasa d’administració subcutània per a la profilaxi a llarg termini de l’angioedema hereditari
Abstract
Hereditary angioedema (AEH) is a rare disease of autosomal dominant inheritance characterized by recurrent and unpredictable episodes of subcutaneous or submucosal edema, non-urticarial or pruritic, affecting the skin, upper airway, and / or gastrointestinal tract. The frequency of attacks can range from weekly to one or two episodes a year, and the presentation can be different between family members. Most attacks affect only one location, although they can affect multiple locations at once. Gastrointestinal attacks usually occur in most patients and are usually painful and debilitating. Its diagnosis can be complicated due to the similarity of the presentation with other gastrointestinal disorders. Laryngeal edemas usually appear in half of patients, but are rarely recurrent. These attacks can compromise the respiratory tract and life of patients, especially in children. In general, episodes are usually self-limiting and disappear after 48-72 hours, but can last up to 2-5 days.
Keywords
Lanadelumab; C1 Inhibitor; Hereditary Angioedema; Prophylaxis
Bibliographic citation
Programa d'Harmonització Farmacoterapèutica. Lanadelumab i l’inhibidor de la C1 esterasa d’administració subcutània per a la profilaxi a llarg termini de l’angioedema hereditari. Barcelona: Servei Català de la Salut; 2021.
Audience
Professionals
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