What does C1 esterase inhibitor test for?

Your doctor may order a C1-INH test if you have unexplained inflammation or swelling, known as edema. Your doctor may also order a C1-INH test to assess you for hereditary angioedema (HAE). Symptoms of HAE are: swelling in feet, face, hands, airway, and gastrointestinal wall.

What does the C1 inhibitor do?

C1-inhibitor (serpin G1) is a 105 kDa inhibitor which functions as a major antiinflammatory protein in the body. It has its effects via inhibition of the proteases of the complement system and contact system of coagulation, as well as several direct effects mediated by its unique highly glycosylated N-terminal domain.

What is HAE disease?

Hereditary angioedema (HAE) is caused by a low level or improper function of a protein called the C1 inhibitor. It affects the blood vessels. An HAE attack can result in rapid swelling of the hands, feet, limbs, face, intestinal tract, larynx (voicebox), or trachea (windpipe).

Is angioedema serious?

Angioedema is swelling underneath the skin. It’s usually a reaction to a trigger, such as a medicine or something you’re allergic to. It is not normally serious, but it can be a recurring problem for some people and can very occasionally be life-threatening if it affects breathing.

What autoimmune disease causes angioedema?

In contrast, chronic angioedema with urticaria is most often caused by autoimmune disease, such as Hashimoto’s thyroiditis,10 or drug reactions (eg, NSAIDs).

What triggers HAE?

While there’s often no clear cause for an attack of hereditary angioedema (HAE), certain activities, events, or situations are known to trigger attacks. These triggers include some physical activities, trauma, stress, and certain medications.

Is angioedema a blood disease?

Hereditary angioedema is a rare inherited disorder characterized by recurrent episodes of the accumulation of fluids outside of the blood vessels, blocking the normal flow of blood or lymphatic fluid and causing rapid swelling of tissues in the hands, feet, limbs, face, intestinal tract, or airway.

Is hereditary angioedema an autoimmune disease?

Hereditary angioedema may be associated with autoimmune diseases, such as systemic lupus erythematosus, rheumatoid arthritis, autoimmune thyroiditis and glomerulonephritis.

Does angioedema get worse over time?

Medication is the main treatment for angioedema, although many cases get better after a few days without treatment. Allergic angioedema and idiopathic angioedema are usually treated in the same way, using a combination of antihistamines and corticosteroids to help relieve the swelling.

Is there a blood test for angioedema?

Laboratory analysis of blood samples, or genetic samples, are required to establish an HAE diagnosis. There are three specific blood tests used to confirm Hereditary Angioedema Type I or II. This is the most common form of the disease and is characterized by low quantitative levels of C1-inhibitor.

What does complement C1 inhibitor protein mean?

Complement C1 esterase inhibitor is a man-made form of a protein in blood that helps control swelling in the body. People with a condition called hereditary angioedema do not have enough of this protein.

What is C1 inhibitor?

View/Edit Mouse. C1-inhibitor (C1-inh, C1 esterase inhibitor) is a protease inhibitor belonging to the serpin superfamily. Its main function is the inhibition of the complement system to prevent spontaneous activation.

What is C1 and C2 esterase?

C1 esterase inhibitor . C1 esterase inhibitor (C1-INH) is a protein found in the fluid part of your blood . It controls a protein called C1, which is part of the complement system. This system is a group of proteins that move freely through your bloodstream. The proteins work with your immune system and play a role in the development of inflammation.