Based on drug class, the market has been classified into recombinant coagulation factors and human C1 esterase inhibitor
. The recombinant coagulation factors sub-segment has been further sub-segmented into recombinant coagulation factor VIII, recombinant coagulation factor IX, recombinant coagulation factor VIIa, and others.
Hereditary and acquired angioedema: problems and progress: proceedings of the Third C1 Esterase Inhibitor
Deficiency Workshop and beyond.
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).
with Jordan's group (Cedars-Sinai Medical Center, Los Angeles, CA, USA) reviewed the agents, IVIg, anti-CD20 antibodies (rituximab, obinutuzumab), proteasome inhibitors (bortezomib, carfilzomib), anti-IL-6R blocker (tocilizumab), IgG endopeptidase (Ides[R] produced by Streptococcus pyogenes), blockers of B cell stimulator protein to B cell receptor (belimumab) blockers of C5 to inhibit the complement sequence (eculizumab), C1 esterase inhibitor
, and belatacept (CTLA-Ig that can inhibit plasma cells and DSA generation) used in desensitization.
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.
Angiotensin-converting enzyme inhibitors-induced angioedema treated by C1 esterase inhibitor
concentrate (Berinert[R]): About one case and review of the therapeutic arsenal.
Patients have low levels or activity of C1 esterase inhibitor
(C1INH), C4, and C1q.