Based on drug class, the market has been classified into recombinant coagulation factors and human C1 esterase
Hereditary and acquired angioedema: problems and progress: proceedings of the Third C1 Esterase
Inhibitor Deficiency Workshop and beyond.
Protocolo Clinico e Diretrizes Tera peuticas do Angioedema associado a deficiencia de C1 esterase
Recurrent attacks are observed in hereditary angioneurotic edema, which is caused by deficiency of the enzyme C1 esterase
However, if C1 esterase
levels are normal or elevated (along with a low C4 level), functional C1-INH testing should be conducted to confirm type 2 HAE [6,8,11,12] (Table 2).
It was reported yesterday that the US Food and Drug Administration (USFDA) has approved the technology transfer of Ireland-based Shire's Cinryze - a C1 esterase
inhibitor - drug product manufacturing process to its Vienna, Austria manufacturing site.
Binkley et al., "Hereditary and acquired angioedema: problems and progress: proceedings of the third C1 esterase
inhibitor deficiency workshop and beyond," The Journal of Allergy and Clinical Immunology, vol.
Li et al., "Escalating doses of C1 esterase
inhibitor (CINRYZE) for prophylaxis in patients with hereditary angioedema," Journal of Allergy and Clinical Immunology: In Practice, vol.
Keywords: C1 esterase
inhibitor, hereditary angioedema, screening, questionnaires, C4
Angioedema can be classified into three forms: a) an idiopathic form with unknown pathogenetic mechanisms, b) an extrinsic factor-induced form (due to histamine [IgE- and non IgE- triggered] or kinin-mediated pathophysiology), and c) a hereditary/acquired form associated with C1 esterase
inhibitor deficiency or dysfunction (5).
We present a rare case of severe lingual angioedema attributed to acquired C1 esterase
inhibitor deficiency in the setting of systemic lupus erythematosus (SLE) which persisted despite utilization of previously described treatment strategies.