About Acquired angioedema with C1Inh deficiency

What is Acquired angioedema with C1Inh deficiency?

Acquired angioedema with C1Inh deficiency is a rare condition in which the body does not produce enough of a protein called C1 inhibitor (C1Inh). This protein helps regulate the body's immune system and is important for controlling inflammation. Without enough of this protein, the body can experience episodes of swelling, known as angioedema, which can affect the face, tongue, throat, and other parts of the body. Treatment for this condition typically involves replacing the missing C1Inh protein with a medication called C1 esterase inhibitor (C1Inh-EI).

What are the symptoms of Acquired angioedema with C1Inh deficiency?

The symptoms of Acquired angioedema with C1Inh deficiency can include:

-Swelling of the face, lips, tongue, and throat
-Difficulty breathing
-Abdominal pain
-Nausea and vomiting
-Diarrhea
-Hives
-Itching
-Swelling of the hands and feet
-Joint pain
-Fatigue
-Fever

What are the causes of Acquired angioedema with C1Inh deficiency?

The most common cause of acquired angioedema with C1Inh deficiency is an autoimmune disorder, in which the body's immune system mistakenly attacks and destroys the C1Inh protein. Other causes include certain medications, infections, and lymphoproliferative disorders.

What are the treatments for Acquired angioedema with C1Inh deficiency?

1. Replacement therapy with C1-inhibitor concentrate: This is the mainstay of treatment for acquired angioedema with C1-inhibitor deficiency. It is a purified form of C1-inhibitor that is administered intravenously or subcutaneously.

2. Antifibrinolytic agents: These agents, such as tranexamic acid, inhibit the breakdown of fibrin, which is a protein involved in blood clotting. They can be used to reduce the severity and duration of attacks.

3. Corticosteroids: These medications, such as prednisone, can be used to reduce inflammation and swelling.

4. Antihistamines: These medications, such as diphenhydramine, can be used to reduce itching and hives associated with angioedema.

What are the risk factors for Acquired angioedema with C1Inh deficiency?

1. Family history of C1Inh deficiency
2. Genetic mutations
3. Certain medications, such as ACE inhibitors
4. Autoimmune diseases, such as lupus
5. Infections, such as hepatitis C
6. Exposure to certain toxins
7. Certain cancers, such as lymphoma
8. Surgery or trauma
9. Pregnancy

Is there a cure/medications for Acquired angioedema with C1Inh deficiency?

Yes, there are treatments available for Acquired angioedema with C1Inh deficiency. These include medications such as C1-inhibitor concentrate, antifibrinolytic agents, and corticosteroids. Additionally, immunosuppressive agents such as cyclophosphamide and rituximab may be used in some cases.