Complement InhibitorPrescription OnlyPregnancy Cat. Not classifiedFDA Yes
Epysqli
ECULIZUMAB-AAGH
1 INDICATIONS AND USAGE EPYSQLI is a complement inhibitor indicated for: the treatment of patients with paroxysmal nocturnal hemoglobinuria (PNH) to reduce hemolysis. ( 1.1 ) the treatment of patients with atypical hemolytic uremic syndrome (aHUS) to inhibit complement-mediated thrombotic microangiopathy. ( 1.2 ) Limitation of Use EPYSQLI is not indicated for the treatment of patients with Shiga toxin E. coli related hemolytic uremic syndrome (STEC-HUS). the treatment of generalized myasthenia g...
Medically reviewed by MedCentralHub Medical Review Board, Licensed Pharmacists & Physicians ·
Brand names:EPYSQLI
Quick Reference
Medicine ClassComplement Inhibitor
FDA ApprovedYes
PregnancyCategory Not classified
Quick Facts
- Active ingredient
- ECULIZUMAB-AAGH
- Medicine class
- Complement Inhibitor
- Brand names
- EPYSQLI
- Availability
- Prescription only (Rx)
- Pregnancy category
- Not classified
- Half-life
- half-life of eculizumab ranged between 141 h to 882 h
- FDA approved
- Yes
What Is Epysqli?
1 INDICATIONS AND USAGE EPYSQLI is a complement inhibitor indicated for: the treatment of patients with paroxysmal nocturnal hemoglobinuria (PNH) to reduce hemolysis. ( 1.1 ) the treatment of patients with atypical hemolytic uremic syndrome (aHUS) to inhibit complement-mediated thrombotic microangiopathy. ( 1.2 ) Limitation of Use EPYSQLI is not indicated for the treatment of patients with Shiga toxin E. coli related hemolytic uremic syndrome (STEC-HUS). the treatment of generalized myasthenia g...
Epysqli (ECULIZUMAB-AAGH) belongs to the Complement Inhibitor class of medications. It was first approved by the FDA in Yes. This medication requires a prescription from a licensed healthcare provider.
Key Safety Information
- •Always follow the dose recommended by your healthcare professional. Taking more than prescribed increases the risk of side effects including serious complications.
- •Seek emergency care if you experience signs of an allergic reaction — hives, swelling, difficulty breathing, or rash. Stop the medication and call your doctor immediately.
- •Some medications can increase the risk of cardiovascular events. Tell your doctor if you have high blood pressure, a history of heart attack or stroke, or any pre-existing health condition.
- •Avoid or limit alcohol while taking Epysqli. Do not drink alcohol without first discussing it with your healthcare provider as it may increase adverse effects.
- •Take Epysqli with food or a full glass of water if stomach upset occurs. Stop use and talk to your doctor if you experience persistent stomach pain or GI symptoms.
- •Tell your doctor about all medications, supplements, and vitamins you take. Some combinations significantly increase the risk of harm.
This is a summary only. Always read the full prescribing information and consult your healthcare provider for personalized medical advice.
Uses & Indications
Epysqli is prescribed for the following conditions. Some uses are FDA-approved indications; others may be evidence-based off-label uses. Consult your healthcare provider for personalized guidance.
1 INDICATIONS AND USAGE EPYSQLI is a complement inhibitor indicated for: the treatment of patients with paroxysmal nocturnal hemoglobinuria (PNH) to reduce hemolysis.
( 1.1 ) the treatment of patients with atypical hemolytic uremic syndrome (aHUS) to inhibit complement-mediated thrombotic microangiopathy.
( 1.2 ) Limitation of Use EPYSQLI is not indicated for the treatment of patients with Shiga toxin E.
coli related hemolytic uremic syndrome (STEC-HUS).
the treatment of generalized myasthenia gravis (gMG) in adult patients who are anti-acetylcholine receptor (AchR) antibody positive.
( 1.3 ) 1.1 Paroxysmal Nocturnal Hemoglobinuria (PNH) EPYSQLI is indicated for the treatment of patients with paroxysmal nocturnal hemoglobinuria (PNH) to reduce hemolysis.
1.2 Atypical Hemolytic Uremic Syndrome (aHUS) EPYSQLI is indicated for the treatment of patients with atypical hemolytic uremic syndrome (aHUS) to inhibit complement-mediated thrombotic microangiopathy.
Limitation of Use EPYSQLI is not indicated for the treatment of patients with Shiga toxin E.
Dosage Guide
The following are general dosing guidelines only. Your actual dose should be determined by your healthcare provider based on your condition, renal/hepatic function, and other medications.
AD
Adult Dosage
2 DOSAGE AND ADMINISTRATION For intravenous infusion only; recommended dosage for: PNH: ( 2.2 ) aHUS and gMG in adults: ( 2.3 ) aHUS in pediatric patients: ( 2.4 ) 2.1 Recommended Vaccination and Prophylaxis for Meningococcal Infection Vaccinate patients against meningococcal infection (serogroups A, C, W, Y, and B) according to current ACIP recommendations at least 2 weeks prior to initiation of EPYSQLI [ see Warnings and Precautions ( 5.1 ) ]. If urgent EPYSQLI therapy is indicated in a patient who is not up to date with meningococcal vaccines according to ACIP recommendations, provide the patient with antibacterial drug prophylaxis and administer these vaccines as soon as possible. Healthcare providers who prescribe EPYSQLI must enroll in the EPYSQLI REMS [see Warnings and Precautions ( 5.2 )]. 2.2 Recommended Dosage for Adults – PNH The recommended dosage of EPYSQLI for the treatment of PNH in patients 18 years of age and older is administered as an intravenous infusion [see Dosage
Available Forms
Available Strengths
Side Effects
Common
- 6 ADVERSE REACTIONS The following serious adverse reactions are discussed in greater detail in other sections of the labeling: Serious Meningococcal Infections [ see Warnings and Precautions ( 5.1 ) ] Other Infections [ see Warnings and Precautions ( 5.3 ) ] Monitoring Disease Manifestations after EPYSQLI Discontinuation [ see Warnings and Precautions ( 5.4 ) ] Thrombosis Prevention and Management [ see Warnings and Precautions ( 5.5 ) ] Infusion-Related Reactions [ see Warnings and Precautions ( 5.6 ) ] The most frequently reported adverse reactions in the PNH randomized trial (≥10% overall and greater than placebo) are: headache, nasopharyngitis, back pain, and nausea.
- ( 6.1 ) The most frequently reported adverse reactions in aHUS single arm prospective trials (≥20%) are: headache, diarrhea, hypertension, upper respiratory infection, abdominal pain, vomiting, nasopharyngitis, anemia, cough, peripheral edema, nausea, urinary tract infections, pyrexia.
- ( 6.1 ) The most frequently reported adverse reaction in the gMG placebo-controlled clinical trial (≥10%) in adult patients is musculoskeletal pain.
- ( 6.1 ) To report SUSPECTED ADVERSE REACTIONS, contact Teva Pharmaceuticals at 1-888-483-8279 or FDA at 1-800-FDA-1088 or www.fda.gov/medwatch .
- 6.1 Clinical Trial Experience Because clinical trials are conducted under widely varying conditions, adverse reaction rates observed in the clinical trials of a drug cannot be directly compared to rates in the clinical trials of another drug and may not reflect the rates observed in practice.
- Meningococcal infections are the most important adverse reactions experienced by patients receiving eculizumab.
- In PNH clinical studies, two patients experienced meningococcal sepsis.
Serious
- 5 WARNINGS AND PRECAUTIONS Use caution when administering EPYSQLI to patients with any other systemic infection.
- ( 5.3 ) Infusion-Related Reactions: Monitor patients during infusion, interrupt for reactions, and institute appropriate supportive measures.
- ( 5.6 ) 5.1 Serious Meningococcal Infections Eculizumab products, complement inhibitors, increase a patient's susceptibility to serious, life-threatening, or fatal infections caused by meningococcal bacteria (septicemia and/or meningitis) in any serogroup, including non-groupable strains.
- Life-threatening and fatal meningococcal infections have occurred in both vaccinated and unvaccinated patients treated with complement inhibitors.
- The initiation of EPYSQLI treatment is contraindicated in patients with unresolved serious Neisseria meningitidis infection.
Rare
Medicine Interactions
Always inform your healthcare provider and pharmacist about ALL medications you take, including prescriptions, OTC medicines, vitamins, and supplements.
MajorModerateMinor
Warnings & Contraindications
5 WARNINGS AND PRECAUTIONS Use caution when administering EPYSQLI to patients with any other systemic infection. ( 5.3 ) Infusion-Related Reactions: Monitor patients during infusion, interrupt for reactions, and institute appropriate supportive measures. ( 5.6 ) 5.1 Serious Meningococcal Infections Eculizumab products, complement inhibitors, increase a patient's susceptibility to serious, life-thr
How Epysqli Works
12.1 Mechanism of Action Eculizumab-aagh, the active ingredient in EPYSQLI, is a monoclonal antibody that specifically binds to the complement protein C5 with high affinity, thereby inhibiting its cleavage to C5a and C5b and preventing the generation of the terminal complement complex C5b-9. Eculizumab products inhibit terminal complement-mediated intravascular hemolysis in PNH patients and complement-mediated thrombotic microangiopathy (TMA) in patients with aHUS. The precise mechanism by which eculizumab exerts its therapeutic effect in gMG patients is unknown, but is presumed to involve reduction of terminal complement complex C5b-9 deposition at the neuromuscular junction.
Pharmacokinetics
Half-Life
half-life of eculizumab ranged between 141 h to 882 h
Pregnancy & Breastfeeding Safety
Not classified
Breastfeeding
Many medications pass into breast milk in varying amounts. Before using Epysqliwhile breastfeeding, discuss the benefits and risks with your healthcare provider or pharmacist — they can weigh your dose, your infant's age, and available lactation safety data to find the safest option for you and your baby.
Storage & Handling
Store at room temperature. Keep away from moisture and heat. Keep out of reach of children.
Frequently Asked Questions
Related on MedCentralHub
- Class hub
Complement Inhibitor alternatives
Compare all Complement Inhibitor medications — uses, side effects, and cost differences
- Detail
Epysqli dosage guide
Adult, pediatric, renal, and hepatic dosing for Epysqli
- Detail
Epysqli side effects
Complete adverse effect profile including common, serious, and rare reactions
Sources & References
- • FDA Prescribing Information — Official labeling and package insert for Epysqli
- • U.S. National Library of Medicine — MedlinePlus drug information
- • American Hospital Formulary Service (AHFS) — Clinical pharmacology monograph
- • PubMed / MEDLINE — View clinical studies
Last reviewed by MedCentralHub Medical Review Board · MedCentralHub Editorial Policy
Medical Disclaimer
The information on this page is for educational purposes only and is not intended as medical advice, diagnosis, or treatment. Always consult your doctor, pharmacist, or qualified healthcare provider before starting, stopping, or changing any medication.