immune disorders
Hereditary and Acquired Angioedema
Treatment
Drugs such asecallantide,icatibant, or C1 inhibitor Fresh frozen plasma Drugs to prevent future attacks Drugs that can sometimes relieve the swelling includeecallantide,icatibant, purified C1 inhibitor (which is derived from human blood), and recombinant C1 inhibitor (which is obtained from the milk of genetically modified rabbits). Giving C1 inhibitor replenishes the missing or malfunctioning C1 inhibitor. These drugs are given by vein or injection under the skin. Because hereditary angioedema is rare, the person's doctor usually checks whether the local health care facility has one of these necessary drugs. If the person has had a severe reaction, the doctor may give the person one of these drugs to keep at home and use when an attack starts. The person or family members are taught how to inject the drugs themselves. The earlier treatment begins, the better. When these drugs are not available, Plasma or, in the European Union, Pain relievers, drugs to relieve nausea (antiemetic drugs), and fluids may help relieve symptoms. Sometimes if the airway suddenly swells and people have difficulty breathing, doctors must open the airway. To do so, they may injectepinephrineunder the skin or into the muscle to reduce the swelling. However,epinephrinemay not reduce the swelling quickly or long enough. Then doctors insert a breathing tube in the windpipe through the person’s mouth or nose (intubation). Sometimes doctors have to make a small incision in the skin over the windpipe (trachea) to insert the breathing tube. Several treatments can be used to help prevent episodes of angioedema in people with hereditary angioedema. They include C1 inhibitor derived from human blood Lanadelumab Berotralstat Synthetic male hormones Antifibrinolytics (eg,tranexamic acid) C1 inhibitor derived from human blood can be used to prevent attacks. However, recombinant C1 inhibitor cannot. is a Monoclonal Antibodies (a manufactured antibody) that targets and suppresses one of the substances involved in causing angioedema. Berotralstatsuppresses the same substance aslanadelumaband is taken by mouth 3 times a day. Stanozolol anddanazol(which are synthetic male hormones) may help prevent subsequent attacks. These drugs, taken by mouth, can stimulate the body to produce more C1 inhibitor, but they may be less effective for acquired angioedema. Because these drugs can have masculinizing side effects, the dose is reduced as soon and as much as possible when these drugs are given to women for a long time. Stanozolol ordanazolmay be given 5 days before until 2 days after a dental or surgical procedure. Or C1 inhibitor, if available, may be given 1 hour before dental or surgical procedures instead of stanozolol ordanazol. 