Causes And Symptoms Of Quincke’s Edema
Quincke’s edema, angioneurotic edema or hereditary angioedema is the hereditary genetic disease that presents with painful inflammation and the appearance of edema in the deep dermis, in the subcutaneous tissue, in the mucous membranes and even in some viscera.
Sometimes this genetic disease determines a life-threatening picture if it affects the airways. Learn more about her in this article.
Types of hereditary angioedema
Hereditary angioedema can be of two types:
- Type 1 (most common): characterized by C1 inhibitor deficiency, which is a protein that regulates the classic pathway of complement activation of the immune system. It is a bridge between innate and acquired immunity that helps us defend ourselves against infections.
- Type 2: characterized by C1 inhibitor malfunction.
The first symptoms of hereditary angioedema usually appear during childhood or adolescence. In older ages, and when patients have an associated disorder, it may be the case that C1 inhibitor deficiency is acquired by the following causes:
- The complement system is depleted due to a neoplastic disorder (B-cell lymphoma).
- Antibodies are produced against the C1 inhibitor. This occurs in autoimmune diseases such as lupus erythematosus.
What can trigger Quincke’s edema?
The trigger for hereditary angioedema can be mild trauma, a viral illness, eating certain foods, stress, and even exposure to cold.
Symptoms associated with Quincke’s edema
The characteristic symptoms of Quincke’s edema are very similar to those that appear in other forms of bradykinin-mediated angioedema:
- Asymmetric painful swelling of the face, lips and tongue.
- Swelling on the back of the hands, feet and genitals.
- Intestinal obstruction.
- Nausea.
- Vomiting.
Hereditary angioedema does not present with hives or itching.
How is it diagnosed?
Bradykinin, which is a powerful vasodilator, is considered to be responsible for most of the symptoms of hereditary angioedema.
Diagnosis is made by laboratory tests through the detection of serum complement concentration. To do this, the concentrations of C4, inhibitor of C1 and C1q, which is a component of C1, are measured:
- Hereditary angioedema (types 1 and 2) is confirmed by low C4 levels.
- Hereditary angioedema type 1: low concentrations of C1 inhibitor and normal concentration of C1q.
- Hereditary angioedema type 2: normal or elevated C1 inhibitor level and normal C1q level.
- Acquired C1 inhibitor deficiency: low C1q levels.
Therefore, if angioedema is not accompanied by urticaria and recurs without clear cause, hereditary angioedema or acquired C1 inhibitor deficiency should be suspected.
Quincke’s edema treatment
Treatment of bradykinin-mediated angioedema, such as Quincke’s edema, is often resistant to “standard” therapies used to treat other types of angioedema. The use of epinephrine, glucocorticoid drugs, or antihistamines is not effective in these cases.
- For acute life-threatening attacks due to airway compromise: C1 inhibitor concentrate derived from human plasma is administered intravenously.
- Long-term prevention: treatment is based on a C1 inhibitor (ecallantide or icatibant). If none of these drugs were to be available, tranexamic acid is used in the European Union.
- Attenuated androgens (danazol) are also used for prevention or as a prophylactic measure. These stimulate the hepatic synthesis of inhibitor C1.
- In case of surgery, intubation or procedure that involves trauma to the cervico-facial region, a possible acute attack should be prevented in the short term. C1 esterase inhibitor concentrate is usually used thirty minutes before the invasive procedure.
- To relieve symptoms: pain relievers, anti-semen, and hydration can be used.
Conclution
Although it is a low prevalence pathology, Quincke’s edema or hereditary angioedema can compromise the life of the patient if it is not diagnosed in time.
The clinical signs and symptoms that occur with this disease may lead to suspect that it could be another pathology in the wrong way. Therefore, if there is a slight suspicion of the presence of angioedema, a specialist should be consulted.
