C1 inhibitors are protease inhibitors whose main function is inhibition of the complement system to prevent spontaneous activation. These belong to the superfamily of proteins known as serpin. The inhibitors nearly double due to increase in inflammation, but its normal level in blood is around 0.25g/L.
These are known as the most essential physiological inhibitors of plasma kallikrein, fXIa, and fXIIa. If the level of C1 inhibitors is not normal and is lower or higher in the blood, it leads to hereditary or acquired angioedema. C1-INH is a protein available freely in the bloodstream and certain tests are done to track the deficiency of these group of proteins. Both the conditions are life threatening when the larynx is affected, leading to fatal laryngeal attack and mortality. C1 inhibitors are also known as C1 esterase inhibitors and unlike other serpin family members, it has two domains: C-terminal and N-terminal.
The global C1 inhibitors market is projected to grow at a rapid pace due to usage in hereditary angioedema (HAE) treatment and more preferred use as prophylactic treatment. C1 inhibitors are heavy glycosylated proteins and the human C1-inhibitor gene is found on the eleventh chromosome. C1 inhibitors are used to treat acute attacks and as routine prophylaxis against HAE. Increase in level of bradykinin caused by C1 deficiency leads to HAE and acquired C1 inhibitor deficiency. HAE is classified into two primary types: type 1 and type 2. Type 1 is the most common form, accounting for 80% to 85% of HAE and is caused by deficiency of C1. Type 2 accounts for 15% to 20% of HAE and is caused by C1 dysfunction. Type 3 is a rare form and its occurrence is unknown and primarily affects women. Acquired deficiency is the result of complement system consumed in B-cell lymphoma and autoimmune disorders. Common symptoms of C1 deficiency leading to hereditary or acquired angioedema are painful swelling on face, lips, tongue, hands, feet, and others. It also affects GI tract by causing nausea and vomiting. Laryngeal edema is also one of the symptoms.
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The global C1 inhibitors market is driven by rise in awareness about the effective treatment for HAE diseases. C1 inhibitors are the most effective and feasible treatment for HAE and acquired angioedema. Increase in investments is also one of the factors driving the market. Rise in prevalence of HAE also fuels the growth of the global C1 inhibitors market. Novel drugs and formulations are the key focus areas for the companies in this market. C1 inhibitor drugs are also used as short term prophylactic medication. Increase in demand for such treatment is another factor that propels the market. Acceptance of subcutaneous route of administration by specialists is a major benefit. In emerging economies, availability of Ruconest acts as a driver of the market. In some regions, slow approvals and lack of awareness act as restraints of the global C1 inhibitors market. In few countries, delay in reimbursement process also slowdown the ongoing demand, in turn negatively affecting the overall growth of the global market. Expensive medicines and misdiagnosis are the factors hampering the growth of the market.
Based on source, the global C1 inhibitors market can be bifurcated into human derived and recombinant derived. Currently available human derived products in the global market are CINRYZE, Berinert, and HAEGARDA. Recombinant derived products in the global market include Ruconest. In terms of route of administration, the market can be classified into intravenous and subcutaneous. Based on end-user, the global C1 inhibitors market can be divided into hospitals and pharmacies. In terms of region, the global market can be segmented into North America, Europe, Asia Pacific, the Middle East, and Latin America. North America is the leading C1 inhibitors market, driven by developed health care infrastructure, awareness related to the treatment options for HAE, and strong reimbursement support. North America was followed by Europe and Asia Pacific.
Major players leading the global C1 inhibitors market include Shire, CSL Behring, Lev Pharmaceuticals, Pharming Group, Salix Pharmaceuticals, Viropharma Biologics, Inc., Ionis Pharmaceuticals, Inc., iBio, Inc., Sanquin, and BioCryst Pharmaceuticals, Inc.
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