Recurrent edema of the body, be alert to this rare disease

Recurring local subcutaneous or mucosal edema, need to be vigilant is a rare autosomal dominant genetic disease-hereditary angioedema. This disease can occur at any age. It is more common in childhood or adolescence. It is often accompanied by abdominal pain, nausea and vomiting, and decreased C1 esterase inhibitors in serum. There is often a family history. When the symptoms are swelling of the larynx, it is fatal.

According to reports in the literature, the global prevalence of hereditary angioedema is about one in 50,000. The main symptom of this disease is local subcutaneous or mucosal edema, often involving the face, limbs, genital area, gastrointestinal tract and throat. The onset of most patients is unpredictable, and once the onset occurs, the condition is usually moderate to severe. The most frightening thing is that when the disease is manifested as throat edema, if the rescue is not timely, the patient can suffocate and die within a few hours. According to statistics, 58.9% of patients with hereditary angioedema in my country have experienced laryngeal edema, which is also one of the main causes of death of patients.

Professor Zhi Yuxiang from the Allergy Department of Peking Union Medical College Hospital said: For a long time, there was a lack of targeted treatment drugs for acute episodes of hereditary angioedema in China, mainly relying on the infusion of freeze-dried fresh plasma to resolve the edema, but The bradykinin contained in the plasma itself may also cause the patient’s edema to increase. In 2018, hereditary angioedema was included in my country’s “First List of Rare Diseases”. At the same time, icatibant acetate injection, a therapeutic drug for the acute onset of the disease, was also included in the “First Batch of Urgent Need for Clinical Medicine”. List of Overseas New Drugs. A few days ago, icatibant acetate injection was approved by the State Food and Drug Administration, filling the gap in the field of targeted treatment of acute episodes of hereditary angioedema in my country.

In fact, icatibant acetate is a potent and selective bradykinin B2 receptor and antagonist. Its principle is to inhibit the mucosal edema of the skin, gastrointestinal tract and respiratory tract caused by the increase of bradykinin, thereby treating acute hereditary. Onset of angioedema. When used to treat acute attacks of hereditary angioedema in adults, adolescents, and children two years of age and older, it can quickly relieve symptoms, shorten the onset time, and reduce the risk and severity of the onset. For patients, treatment methods have also changed. After receiving subcutaneous injection technology training from medical staff, self-administration can be achieved like diabetic patients using insulin, instead of relying only on infusion of freeze-dried fresh plasma to reduce edema.