Summary Hereditary angioedema due to C1 inhibitor de-ciency (HAE-C1-INH) is characterized by recur-rent skin swellings, abdominal pain attacks, and – more rarely – potentially life-threatening laryngeal attacks.  us, HAE-C1-INH may be associated with a signi
cant morbidity and mortality. Over the last years, the eld of research in the pathogen-esis of HAE-C1-INH has greatly expanded.  is has led to new clinical trials with new therapeutic options. Strategies for managing HAE-C1-INH are aimed at treating acute attacks or preventing attacks through the use of prophylactic treatment. Agents available for treating acute attacks include
plasma-derived C1-INH concentrates, a bradyki-nin B2 receptor antagonist, and a recombinant human C1-INH. In the USA, a plasma kallikrein inhibitor has additionally been approved. Long-term prophylactic treatments include attenuated androgens, a plasma-derived C1-INH concentrate, and antibrinolytics. Plasma-derived C1-INH and a bradykinin B2-receptor antagonist are approved for self-administration at home.  e number of management options for HAE-C1-INH has in-creased considerably within the last few years, thus
helping to alleviate the burden of disease.