Acquired Angioedema

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Acquired Angioedema
Overview

What Is Acquired Angioedema

What disease is acquired angioedema?

Acquired angioedema is a rare disease. Some diseases (such as lymphoma and autoimmune diseases) result in the reduction of serum "C1 esterase inhibitor", causing repeated swelling of the skin, respiratory tract or gastrointestinal mucosa, manifested as local skin swelling, dyspnea, abdominal pain, diarrhea, etc., without urticaria.

Acquired angioedema occurs mostly in older adults with underlying disease, which is neither hereditary nor contagious.

Is acquired angioedema common?

Acquired angioedema is rare.

Cause

What Is The Cause Of Acquired Angioedema

What causes acquired angioedema?

According to the different etiology, acquired angioedema is divided into two types.

  • Type I: It is caused by lymphoma, leucocyte and lymphocyte proliferative disease, and malignant tumor.
  • Type II: Caused by autoimmune disease or without underlying disease. Angioedema is divided into two types.

What are the common causes of acquired angioedema?

Patients with lymphoma, leucocyte and lymphocyte proliferative disease, malignant tumor and autoimmune disease usually have angioedema under certain precipitating factors, including:

  • Emotional disorders: such as excessive sadness, anxiety, stress, etc.
  • Minor trauma: such as extractions and oral surgery.
  • Infection: diseases such as cold or influenza, helicobacter pylori infection, etc.
  • Long periods of physical activity, such as typing, writing, or other physical activity.
  • Others: such as estrogen drugs, angiotensin converting enzyme inhibitor, analgesic drugs, menstruation, pregnancy, etc.

Acquired angioedema can be infected?

It's not contagious.

Will acquired angioedema be inherited?

Not hereditary. This is also one of the main differences between acquired angioedema and hereditary angioedema.

Symptom

What Are The Symptoms Of Acquired Angioedema

What are the common manifestations of acquired angioedema?

Patients' symptoms vary from person to person and may vary from person to person. Edema can occur in one site, or in multiple sites (such as skin, airway, and gastrointestinal tract) at the same time, and can last for 2–5 days. Without urticaria and fever.

  • If the edema occurs in the skin: skin edema often occurs in the face, limbs or genitalia and other parts. It can manifest as tingling or swelling of the skin, or an itchy rash followed by swelling and tightness, and may be accompanied by pain and dysfunction. Edema usually resolves within a few days or spreads to other parts of the body.
  • If edema occurs in airway, it can be manifested as respiratory obstruction or tightness, dysphagia, and even obstructed breathing, chest stuffiness, suffocation and faint due to further aggravation.
  • If edema occurs in the gastrointestinal tract: abdominal pain (colic), nausea, vomiting, abdominal distension, diarrhea, etc. will occur to varying degrees.

Where does acquired angioedema often occur?

  • Skin of the lips, face, limbs and genitals;
  • Respiratory tract such as throat and trachea;
  • Tongue, gastrointestinal tract.

What are the serious consequences of acquired angioedema?

  • Edema that occurs near or in the airways (e.g., the face, neck, tongue, and throat) can cause dyspnea and even suffocation to death.
  • Edema occurring in the digestive tract is often misdiagnosed as appendicitis, acute pancreatitis and other diseases, and even leads to unnecessary laparotomy.
Detect

How To Check For Acquired Angioedema

How is acquired angioedema diagnosed?

Doctors need to combine the patient's history, physical examination and laboratory tests to make a comprehensive judgment on whether hereditary angioedema exists.

  • History: The patient often had underlying conditions such as lymphoma, leukocytic and lymphoproliferative disorders, malignancy, and autoimmune disease, but there was no family history of angioedema. Symptoms often start after the age of 30, with repeated episodes of skin, airway, digestive tract edema, specific symptoms see the previous content. Patients may have tried general antihistamine and glucocorticoid therapy, but it is usually ineffective.
  • Physical examination: It is performed by a doctor and local subcutaneous (or submucous) swelling is visible, usually in the eyelids, lips, face, throat, limbs, and external genitalia. The edema is asymmetric and non-pitting, and the skin may be normal in color or slightly red.
  • Laboratory tests: Complement test results indicated low level of C4, low level of C1 inhibitor antigen or decreased function of C1 inhibitor, positive anti-C1 inhibitor antibody, and low level of C1q.

What does acquired angioedema need to do?

  • Routine blood test: it is used to judge whether there is infection.
  • Complement tests including C4 level, C1 inhibitor antigen level, C1 inhibitor function test, anti-C1 inhibitor antibody test, and C1q level.
  • Genetic examination: differentiation between hereditary and acquired angioedema.
  • Abdominal ultrasound or CT examination: to assist in the diagnosis of acquired angioedema of the intestine.

What diseases are acquired angioedema easily confused with? How?

  • Hereditary angioedema: Similar to acquired angioedema, it is difficult to differentiate. However, hereditary angioedema generally has a family history, and occurs at a relatively young age. Generally, the first attack occurs before the age of 20 years old. There is generally no basic disease such as lymphoproliferative disease, malignant tumor or autoimmune disease. The C1q level is normal.
  • Angioedema caused by anaphylaxis: Angioedema caused by anaphylaxis is much more common than acquired angioedema, and generally occurs after contact with a definite allergen (such as eating, insect bites, or drug use). It can be accompanied by urticaria and pruritus, and develops more rapidly with normal complement C4 and C1 inhibitors.
  • Drug-induced angioedema: Medical history, especially antihypertensive angiotensin converting enzyme inhibitor or non-steroidal anti-inflammatory drug, oral cavity and throat edema most frequently occurred, and complement C4 and C1 inhibitors were normal.
  • Contact dermatitis: It is characterized by skin swelling, erythema, papules, and blisters at the site of contact with foreign substances, with severe itching. A patch test was positive and acquired angioedema was not.
  • Autoimmune diseases such as systemic lupus erythematosus, polymyositis, dermatomyositis and Sjogren's syndrome can cause edema around the face and eyelids, and sometimes hand edema, which usually lasts for a long time and does not suddenly appear and disappear. In addition, autoimmune diseases often include joint pain, fever and other accompanying symptoms. Complement testing in patients with autoimmune diseases is normal and can be distinguished from acquired angioedema on this basis.
  • Hypothyroidism: Severe hypothyroidism can cause facial and lip edema, which lasts for a long time and does not suddenly appear and suddenly disappear. Thyroid function tests can identify.
  • Superior vena cava syndrome and tumors: the local swelling they cause is persistent and gradual, while the angioedematous swelling spontaneously resolves. Complement workup is normal and can be distinguished from hereditary angioedema.
Prevention

How To Prevent Acquired Angioedema

Can acquired angioedema be prevented? How to prevent?

The frequency of acute episodes of angioedema can be reduced by avoiding associated precipitating factors. Specific practices include:

  • Avoid emotional tension, anxiety and stress.
  • Pay attention to oral health and care, try to avoid tooth extraction and oral surgery.
  • Eat a balanced diet, strengthen exercise, and get vaccinations to avoid catching cold, flu and other infectious diseases.
  • Avoid long-term physical activity or other sports activities, so as to avoid overwork.
  • Drugs such as estrogen and angiotensin-converting enzyme inhibitors are best avoided if possible.
Treatment

How To Treat Acquired Angioedema

Acquired angioedema to see which department?

Prior to the diagnosis, depending on the location of the angioedema, patients may visit dermatology, emergency, digestive, ophthalmology, otorhinolaryngology, and other departments. However, patients should generally visit the Allergic Reaction Department after confirmed diagnosis.

Acquired angioedema can oneself good?

Acquired angioedema with only mild skin manifestations resolves spontaneously within a few days without leaving a trace, even if untreated, but recurs later.

Acquired angioedema with severe symptoms requires medical treatment to improve.

How to treat acquired angioedema?

  • Treatment during acute attack: currently, no effective drugs are available in China, and only drugs that can relieve the symptoms can be given. If dyspnea is caused by laryngeal edema, immediate emergency treatment should be provided.
  • Preventive treatment: Preventive treatment can be taken in the space between the episodes of angioedema to prevent repeated episodes of angioedema. At present, the most commonly used drugs are refined C1 inhibitor concentrate, ikaran peptide, and atenolol. The specific dose depends on the condition.

Does acquired angioedema require hospitalization?

Acquired angioedema requires hospitalization for complete investigation and treatment.

Can acquired angioedema be cured radically?

Acquired angioedema is often caused by lymphoma, leucocyte and lymphocyte proliferative diseases, malignant tumor, and autoimmune disease. These diseases are usually difficult to cure, so acquired edema can hardly be cured.

Life

What Should Patients With Acquired Angioedema Pay Attention To In Life

Does acquired angioedema need reexamination?

Repeat studies are required, as directed by your physician, and may include dose adjustments and a review of complement C4 levels.

Does acquired angioedema affect fertility?

No effect.

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