Epysqli

Pronunciation: Eh-pis’-klee
Generic name: eculizumab
Dosage form: Injection for intravenous use

Medically reviewed by Carmen Pope, BPharm. Last updated on Jul 24, 2024.

What is Epysqli?

Epysqli (eculizumab-aagh), a biosimilar to Soliris, targets the complement system to treat paroxysmal nocturnal hemoglobinuria (PNH) and atypical hemolytic uremic syndrome (aHUS).

• Epysqli is not approved to treat hemolytic uremic syndrome due to Shiga toxin E. coli (STEC-HUS).

Epysqli is a monoclonal antibody that works by inhibiting the activity of the complement system, which is part of the immune system. It specifically binds to complement protein C5 with high affinity, inhibiting its cleavage to C5a (a proinflammatory toxin) and C5b (which initiates the membrane attack complex (MAC or C5b-9) which leads to osmolysis and the rupture of the cell wall.  Epysqli prevents complement-mediated red blood cell rupture in patients with PNH and complement-mediated thrombotic microangiopathy (TMA) in patients with aHUS.

Epysqli was FDA-approved on July 19, 2024. It is a biosimilar to Soliris but does not have an interchangeability designation. A biosimilar is a biological product that is similar to a reference biologic (in this case Soliris) and for which there are no clinically meaningful differences in terms of safety, purity, and potency. To be substituted for Soliris, Epysqli has to be specifically prescribed by the healthcare provider.

What is Epysqli REMS?

Epysqli is available only under a special program called the Epysqli REMS. Before you can receive it, your healthcare provider must:

• enroll in the REMS program
• counsel you about the risk of serious meningococcal infections
• give you information about the signs and symptoms of serious meningococcal infection
• make sure that you are vaccinated against serious infections caused by meningococcal bacteria and that you receive antibiotics if you need to start Epysqli right away and you are not up to date on your vaccines
• give you a Patient Safety Card about your risk of meningococcal infection.

Related/similar drugs

Empaveli, Ultomiris, Soliris, Fabhalta, eculizumab, pegcetacoplan, ravulizumab

Epysqli side effects

The most common Epysqli side effects in people with PNH include:

• headache
• back pain
• pain or swelling of your nose or throat (nasopharyngitis)
• nausea.

The most common Epysqli side effects in people with aHUS include:

• headache
• stomach-area (abdominal) pain
• low red blood cell count (anemia)
• nausea
• diarrhea
• urinary tract infections
• high blood pressure (hypertension)
• vomiting
• cough
• fever
• pain or swelling of your nose or throat (nasopharyngitis)
• swelling of legs or feet (peripheral edema)
• common cold (upper respiratory infection).

Serious side effects and warnings

Epysqli carries a Boxed Warning for serious meningococcal infections.

Epysqli increases your chance of getting serious meningococcal infections caused by Neisseria meningitidis bacteria. Meningococcal infections may quickly become life-threatening or cause death if not recognized and treated early.

• You must complete or update your meningococcal vaccines at least 2 weeks before your first dose of Epysqli.
• If you have not completed your meningococcal vaccines and Epysqli must be started right away, you should receive the required vaccine(s) as soon as possible.
• If you have not been vaccinated and Epysqli must be started right away, you should also receive antibiotics to take for as long as your healthcare provider tells you.
• If you had a meningococcal vaccine in the past, you might need additional vaccines before starting Epysqli.
• Your healthcare provider will decide if you need additional meningococcal vaccines.

Meningococcal vaccines do not prevent all meningococcal infections. Call your healthcare provider or get emergency medical care right away if you get any of these signs and symptoms of a serious meningococcal infection:

• Fever with or without a headache, rash, or high heart rate
• Confusion
• Muscle aches with flu-like symptoms
• Headache with nausea or vomiting or a stiff neck or stiff back
• Eyes that are sensitive to light.

Your healthcare provider will give you a Patient Safety Card about the risk of serious meningococcal infection. Carry it with you at all times during treatment and for 3 months after your last dose of Epysqli. Your risk of meningococcal infection may continue for several weeks after your last dose of Epysqli. It is important to show this card to any healthcare provider who treats you. This will help them diagnose and treat you quickly. Epysqli is only available through a program called the Epysqli Risk Evaluation and Mitigation Strategy (REMS) (see above).

Epysqli can cause other serious side effects, including the following.

Epysqli can increase your risk of other serious infections caused by encapsulated bacteria, including Streptococcus pneumoniae, Haemophilus influenzae, and Neisseria gonorrhoeae.

• If your child is treated with Epysqli, they should receive vaccines against Streptococcus pneumoniae and Haemophilus influenzae type b (Hib).
• Certain people may be at risk of serious infections with gonorrhea. Talk to your healthcare provider about whether you are at risk for gonorrhea infection, about gonorrhea prevention, and regular testing.
• Certain fungal infections (aspergillus) may also happen if you take Epysqli and have a weak immune system or a low white blood cell count

Serious infusion-related reactions can happen during your Epysqli infusion. Tell your healthcare provider or nurse right away if you get any of these symptoms during your Epysqli infusion: chest pain, trouble breathing or shortness of breath, feeling faint or passing out, or swelling of your face, tongue, or throat. If you have an infusion-related reaction to Epysqli, your healthcare provider may need to infuse Epysqli more slowly, or stop Epysqli.

Tell your healthcare provider about any side effect that bothers you or does not go away. These are not all the possible side effects of Epysqli For more information, ask your doctor or pharmacist. Call your doctor for medical advice about side effects. You may report side effects to the FDA at 1-800-FDA-1088.

Before taking

Do not receive Epysqli if you have a serious meningococcal infection when you are starting Epysqli treatment.

Before you receive Epysqli, tell your healthcare provider about all of your medical conditions, including if you:

• are allergic to Epysqli, eculizumab, or Soliris
• have an infection or fever
• are pregnant or plan to become pregnant
• are breastfeeding or plan to breastfeed.

Pregnancy

It is not known if Epysqli will harm your unborn baby.

Breastfeeding

It is not known if Epysqli passes into your breast milk.

How should I receive Epysqli?

Epysqli is administered by a healthcare provider into your vein through an intravenous (IV) line usually over 35 minutes in adults and 1 to 4 hours in children.

Adults will usually receive an Epysqli infusion:

• weekly for 5 weeks, then
• every 2 weeks.

The frequency of an Epysqli infusion for children less than 18 years of age depends on their age and body weight and will be determined by your healthcare provider.

After each infusion, you should be monitored for at least 1 hour for infusion-related reactions

• If you have an infusion-related reaction during your Epysqli infusion, your healthcare provider may decide to give Epysqli more slowly or stop your infusion.

Epysqli dosage

Recommended Epysqli dosage for PNH

Adults

• 600 mg weekly for the first 4 weeks, followed by
• 900 mg for the fifth dose 1 week later, then
• 900 mg every 2 weeks thereafter.

Administer Epysqli at the recommended dosage regimen time points, or within two days of these time points

Children (aged less than 18 years)

• Administer Epysqli based on body weight. See the Epysqli Package Insert.

Recommended Epysqli dosage for aHUS

Adults

• 900 mg weekly for the first 4 weeks, followed by
• 1200 mg for the fifth dose 1 week later, then
• 1200 mg every 2 weeks thereafter.

Children

• Administer Epysqli based on body weight. See the Epysqli Package Insert.

What happens if I miss a dose?

If you miss an Epysqli infusion, call your healthcare provider right away.

What happens when I stop Epysqli?

If you have PNH, your healthcare provider will need to monitor you closely for at least 8 weeks after stopping Epysqli. Stopping treatment may cause the breakdown of your red blood cells due to PNH. Symptoms or problems that can happen due to red blood cell breakdown include:

• drop in the number of your red blood cell count
• kidney problems
• drop in your platelet counts
• blood clots
• confusion
• difficulty breathing
• chest pain.

If you have aHUS, your healthcare provider will need to monitor you closely for at least 12 weeks after stopping Epysqli for signs of worsening aHUS symptoms or problems related to abnormal clotting (thrombotic microangiopathy). Symptoms or problems that can happen with abnormal clotting may include:

• stroke
• difficulty breathing
• confusion
• kidney problems
• seizures
• swelling in your arms or legs
• chest pain (angina)
• a drop in your platelet count.

What other drugs will affect Epysqli?

Tell your healthcare provider about all the medicines you take, including prescription and over-the-counter medicines, vitamins, and herbal supplements. Epysqli and other medicines can affect each other causing side effects.

Intravenous immunoglobulin (IVIg) treatment may decrease serum Epysqli concentrations. 

Know the medications you take and the vaccines you receive. Keep a list of them to show your healthcare provider and pharmacist when you get a new medicine. See the Epysqli Package Insert for a full list of interactions.

Storage

Your healthcare provider will store Epysqli in the refrigerator at 2°C to 8°C (36°F to 46°F) in the original carton to protect it from light until the time of use. Do not freeze, do not shake.

Before administration, if needed, unopened vials of Epysqli may be stored in the original carton at controlled room temperature [not more than 30°C (86°F)] for a single 1-month period and then returned to refrigeration for 3 days.

Do not use beyond the expiration date shown on the carton.

Epysqli ingredients

Active ingredient: eculizumab-aagh.

Inactive ingredients: dibasic sodium phosphate, monobasic sodium phosphate, polysorbate 80 (vegetable origin), trehalose, and Water for Injection.

Supplied as one 300 mg/30 mL (10 mg/mL) single-dose vial per carton.

Who makes Epysqli?

Samsung Bioepis Co., Ltd makes Epysqli.

Popular FAQ

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Further information

Always consult your healthcare provider to ensure the information displayed on this page applies to your personal circumstances.

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