How does BERINERT work to treat hereditary angioedema?

October 7, 2025 •

3 min read

Affecting roughly 1 in 50,000 people, hereditary angioedema (HAE) is a rare genetic disorder characterized by severe, unpredictable swelling attacks. BERINERT is an on-demand medication that works by directly addressing the root cause of these attacks by replacing a critical, deficient protein.

Quick Summary

BERINERT, a plasma-derived C1 esterase inhibitor, treats acute hereditary angioedema attacks by replacing the missing or non-functional protein. This restores normal C1-INH levels, regulating pathways that cause swelling and reducing bradykinin production.

Key Points

Replenishes C1-INH: BERINERT works by providing a functional C1 esterase inhibitor (C1-INH) to patients with hereditary angioedema (HAE) who are deficient in this critical protein.
Inhibits Swelling Pathways: The administered C1-INH restores regulation to the body's complement and contact systems, which become overactive during an HAE attack.
Reduces Bradykinin Levels: By inhibiting key enzymes like plasma kallikrein, BERINERT prevents the excessive generation of bradykinin, the primary cause of HAE swelling.
On-Demand Treatment: BERINERT is used as an on-demand, fast-acting treatment for acute attacks affecting the abdomen, face, and larynx, rather than for long-term prevention.
Addresses Root Cause: Unlike medications that only treat symptoms, BERINERT directly addresses the root biochemical cause of HAE attacks by replacing the missing protein.
Available for Self-Administration: With proper training, BERINERT can be self-administered via an IV infusion, empowering patients to manage attacks promptly and effectively at home.

The Cause of Hereditary Angioedema (HAE)

Hereditary angioedema is a genetic condition typically caused by a deficiency or dysfunction of a protein called C1 esterase inhibitor (C1-INH). C1-INH is a crucial protein that helps regulate several inflammatory and coagulation pathways in the body. When C1-INH is not working correctly, it fails to inhibit key enzymes like plasma kallikrein and factor XIIa.

Without this critical regulatory control, these pathways become overactive and lead to the excessive generation of a powerful substance called bradykinin. High levels of bradykinin cause a number of effects that result in an HAE attack, including:

Increased vascular permeability, or 'leakiness,' in blood vessels.
Fluid accumulation leaking out of the vessels and into surrounding tissues.
Localized swelling (edema) in various parts of the body, such as the face, abdomen, hands, and feet.
In life-threatening cases, dangerous swelling of the throat (larynx).

How BERINERT Works: The Mechanism of Action

BERINERT provides a functional, human plasma-derived C1 esterase inhibitor directly to the bloodstream via intravenous (IV) infusion. This replacement therapy, which directly addresses the underlying cause of HAE, is considered a cornerstone of treatment for acute attacks.

Here is a step-by-step breakdown of how BERINERT works:

Replenishes C1-INH levels: When a patient is administered BERINERT, the infusion immediately increases the level of working C1-INH in their plasma.
Inhibits uncontrolled pathways: The newly introduced C1-INH restores regulation to the complement and contact systems, effectively inhibiting the excessive activity of key enzymes like plasma kallikrein and factor XIIa.
Reduces bradykinin: By inhibiting the enzymes that produce bradykinin, BERINERT significantly reduces the overproduction of this key mediator.
Decreases vascular permeability: With less bradykinin being produced, the permeability of the blood vessel walls returns to normal.
Reverses swelling: This regulation prevents further fluid leakage, allowing the built-up fluid to be reabsorbed by the body, which effectively resolves the swelling associated with the HAE attack.

BERINERT Administration and Efficacy

BERINERT is administered as an on-demand treatment, meaning it is used during an acute HAE attack rather than for long-term prevention. It is given as a slow intravenous (IV) infusion, typically taking around 10 to 15 minutes. For many patients, the ability to self-administer at home or elsewhere after proper training is a significant benefit. Clinical studies have shown BERINERT can provide rapid relief for abdominal, facial, and laryngeal attacks.

Comparing BERINERT to Other HAE Treatments

While BERINERT is a C1-INH replacement therapy, other medications for HAE work through different mechanisms. The table below compares BERINERT with other common HAE treatments.


Feature	BERINERT	FIRAZYR® (Icatibant)	TAKHZYRO® (Lanadelumab)
Mechanism	Replaces deficient C1-INH.	Bradykinin B2 receptor antagonist.	Plasma kallikrein inhibitor (monoclonal antibody).
Indication	Acute on-demand treatment for attacks.	Acute on-demand treatment for attacks.	Routine prevention of attacks.
Administration	Intravenous (IV) infusion.	Subcutaneous (SC) injection.	Subcutaneous (SC) injection.
Target	Replaces missing regulator.	Blocks the effect of bradykinin.	Inhibits an enzyme upstream of bradykinin.
Frequency	As needed for an attack.	As needed for an attack.	Every 2 or 4 weeks.

Potential Risks and Considerations

As with any medication, BERINERT carries potential risks and side effects that patients and their healthcare providers should consider.

Risk of blood clots (thromboembolic events): This is a rare but serious risk, particularly for patients with certain risk factors like a prior history of thrombosis, obesity, or immobility.
Hypersensitivity reactions: Severe allergic reactions, including anaphylaxis, can occur. Patients and caregivers should be trained to recognize the symptoms of a serious reaction, which can sometimes mimic an HAE attack.
Transmissible infectious agents: Because BERINERT is derived from human plasma, there is a theoretical, though extremely small, risk of transmitting infectious agents. The risk is minimized through extensive donor screening and manufacturing processes.
Side effects: Common side effects reported include an unpleasant taste in the mouth (dysgeusia), headache, and nausea.

Conclusion

BERINERT provides a crucial, life-saving treatment for individuals suffering from acute hereditary angioedema attacks. By delivering a replacement for the deficient C1 esterase inhibitor, it restores the body's natural regulatory function and halts the inflammatory cascade that leads to swelling. Understanding how BERINERT works is vital for both patients and healthcare providers to manage HAE effectively and make informed treatment decisions during an acute attack. While it is a highly effective, on-demand therapy, awareness of potential risks and careful administration are key to ensuring patient safety. For comprehensive safety and prescribing information, patients should always refer to the official FDA guidelines.

Frequently Asked Questions

BERINERT is a replacement therapy that provides the C1 esterase inhibitor protein that patients with HAE are missing. Other treatments, like FIRAZYR, block the effect of the swelling-causing substance, bradykinin, while preventative options like TAKHZYRO inhibit its production.

BERINERT provides rapid relief for acute HAE attacks, with some patients experiencing relief from laryngeal attacks in as little as 15 minutes, and abdominal/facial attacks in under an hour.

No, BERINERT is indicated for the treatment of acute HAE attacks as they occur, not for routine prophylaxis (prevention). Other C1-INH medications are specifically designed for long-term prevention.

The appropriate dosage for BERINERT is determined by a healthcare professional based on individual factors and the severity of the attack. It is administered intravenously.

The most common side effects of BERINERT include an unpleasant or altered sense of taste (dysgeusia), headache, and nausea. Serious side effects like blood clots are rare but possible.

Seek emergency medical help immediately if you experience symptoms of a severe allergic reaction, such as hives, chest tightness, wheezing, or swelling of the face, tongue, or throat.

Yes, with proper training from a healthcare professional, patients or their caregivers can self-administer BERINERT at home for acute attacks.

There are no adequate studies on BERINERT in pregnant women to determine drug-associated risks, so it should be used only if clearly needed. Patients should discuss risks and benefits with their doctor.