What is the normal range for C1 esterase inhibitor?
Normal levels of C1-INH generally range from 16 to 33 milligrams per deciliter. If your C1-INH levels are lower or higher than normal, it may be a sign of: hereditary or acquired angioedema. SLE.
What does C1 esterase do?
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 but also as the major regulator of the contact system.
What is C1 esterase deficiency?
Hereditary angioedema, otherwise known as C1 esterase deficiency, is defined by recurrent episodes of angioedema without urticaria or pruritus. These skin conditions typically involve the legs, hands, face, upper respiratory tract, as well as gastrointestinal tract.
What is C1 esterase blood test?
The test checks the level of a substance called C1 esterase inhibitor in your blood. This substance is important in regulating the immune system. A very low level of C1 esterase inhibitor would suggest you have an inherited problem affecting how much of this substance your body is able to produce.
What causes low C1 esterase inhibitor?
The main causes of acquired C1-inhibitor deficiency are lymphoproliferative disease and autoimmune disease.
What are the symptoms of angioedema?
Swelling: The hallmark of angioedema is swelling and puffiness of the eyes or lips. It may also affect the hands, feet or legs, and less often may involve genitalia. 1 Swelling can develop in the throat and tongue as well, and this can affect breathing or eating due to physical obstruction of the airway and food pipe.
How do you administer a C1 esterase inhibitor?
CINRYZE ® (C1 esterase inhibitor [human]) is administered by intravenous (or IV) injection, meaning it is injected into a vein in the arm. You can choose to have your injection administered by a healthcare provider or caregiver, or a healthcare provider can show you how to give it to yourself.
What triggers hereditary angioedema?
Exposure to common foods or environmental factors can trigger hereditary angioedema attacks. These include seafood, shellfish, nuts, eggs, milk, animal hair, insect bites, pollen, sun, extreme warm or cold weather, and latex.
Is angioedema serious?
Angioedema can be life-threatening if swelling of the tongue or in the throat blocks the airway.
Is angioedema an auto immune disease?
In an estimated 30–50% of the cases, idiopathic angioedema may be associated with an underlying autoimmune disease, such as systemic lupus erythematosus (SLE).