Language
Country/Area
No result found
The mysterious source of edema in the body: What exactly is acquired angioedema?
Acquired C1 esterase inhibitor deficiency, also known as acquired angioedema (AAE), is a rare medical condition characterized by edema in the body and can be life-threatening. The condition is often caused by other underlying medical conditions.
Acquired angioedema usually develops in early adulthood or middle age, in contrast to familial angioedema, which usually develops in early childhood. It usually takes several months to diagnose the disease because the symptoms often resemble those of other illnesses. Determining the cause of acquired angioedema is an important step in providing appropriate treatment.
"The global prevalence of this disorder may be as high as 1 in 10,000 to 1 in 150,000 people, but the actual number is likely higher due to gaps in diagnosis."
Epidemiology
Based on current research, the prevalence of acquired angioedema is estimated to be between one per 10,000 and one per 150,000 people. However, many experts believe that the actual condition may be more serious due to a lack of awareness of the symptom among the medical community. The condition affects both men and women equally, with symptoms usually appearing in the fourth decade or older.
Cause
The causes of acquired C1 esterase inhibitor deficiency are related to a variety of diseases, including lymphoproliferative disorders and immune system disorders. For example, monoclonal plasma proteinopathies (MGUS) and non-Hodgkin's lymphoma are closely associated with acquired angioedema.
Lymphoproliferative diseases and lymphoid malignancies
Many studies have pointed out that MGUS is one of the most common diseases related to acquired angioedema. In addition to these, certain lymphoid neoplasms are also associated with AAE.
Autoimmune diseases
Systemic lupus erythematosus (SLE) is a common autoimmune disease associated with the development of acquired angioedema. Certain other vasculitic diseases such as eosinophilic vasculitis (EGPA) have also been associated with this disease.
Infection
Human immunodeficiency virus (HIV) and hepatitis B virus (HBV) are infections known to cause acquired angioedema.
Metabolic diseases
Hypercholesterolemia-related liposis diseases (eg, jaundice) may also be associated with the condition.
“When known causes have been ruled out, conditions with unknown causes are considered idiopathic.”
Clinical manifestations
Acquired angioedema is mainly characterized by mucosal swelling, which may extend to the face, limbs, and even the upper respiratory tract. However, abdominal or gastrointestinal symptoms are relatively uncommon compared with familial angioedema.
Diagnosis
Diagnosis usually requires detailed clinical examination and laboratory evaluation. Laboratory tests include testing of complement levels, typically low C4 levels, low C1q levels, and normal C3 levels.
Management
The treatment of acquired angioedema is mainly divided into acute symptom control and chronic management. In acute situations, missing enzymes often need to be replaced to reduce life threats. In terms of chronic management, it is important to control the underlying cause.
Prognosis
The prognosis for acquired angioedema often depends on the underlying cause. Patients who successfully treat the underlying cause sometimes experience partial to complete remission.
Our understanding of acquired angioedema is still limited, so will we find more effective diagnosis and treatment methods in the future to improve patients' quality of life?
