Cerezyme (imiglucerase)
Metabolic Disease Enzyme Replacement, Gaucher's Disease Intravenous Vial (Ea)
Last reviewed · Source: First Databank (FDB) drug data
Overview
Imiglucerase is a biosynthetic (recombinant DNA origin) form of human beta-glucocerebrosidase, a lysosomal enzyme that catalyzes the hydrolysis of glucocerebroside to glucose and ceramide.
Imiglucerase is a biosynthetic (recombinant DNA origin) form of human glucocerebrosidase. Imiglucerase differs from placental glucocerebrosidase by the substitution of histidine for arginine at position 495. Oligosaccharide chains at glycosylation sites on imiglucerase have been modified to terminate in mannose sugars; the mannose-terminated oligosaccharide chains are specifically recognized by endocytic carbohydrate receptors on macrophages (the cells that accumulate lipid in Gaucher disease), thereby increasing uptake of the enzyme by these cells (i.e., macrophage targeted).
This removal of oligosaccharide residues to expose mannose residues results in increased receptor specificity and improved activity of the exogenously administered enzyme.
As an enzyme replacement therapy, imiglucerase is intended to replace the deficient endogenous enzyme glucocerebrosidase in patients with Gaucher disease. Imiglucerase increases the degradation of glucocerebroside to glucose and ceramide.
Steady-state enzymatic activity of imiglucerase is reached in 30 minutes during a 1-hour infusion. The volume of distribution of imiglucerase ranges from 0.09-0.15 L/kg. The volume of distribution is reduced in patients who develop IgG antibodies.
Following the infusion, the half-life of plasma enzymatic activity ranged from 3.6-10.4 minutes. In patients who develop IgG antibodies, clearance is reduced and half-life is increased.
USES: Imiglucerase is used to treat a certain rare genetic problem (Gaucher disease). Imiglucerase replaces a certain natural substance (an enzyme called glucocerebrosidase) that is missing in people with Gaucher disease. This medication improves blood, bone, liver, and spleen problems caused by Gaucher disease.
Imiglucerase does not correct the genetic problem, and treatment must be continued for life.
Generic name: imiglucerase
Pronunciation: (im-eh-GLUE-ser-aze)
Drug class: Metabolic Disease Enzyme Replacement, Gaucher's Disease
Therapeutic class: Metabolic Disease Enzyme Replacement Agents
Reference product & biosimilars
No reference, biosimilar or interchangeable associated with this drug.
Today’s intelligence on Cerezyme
Indications
The following indications are listed for Cerezyme:
- Gaucher's Disease
Also known as: Cerebroside Lipidosis, Familial Splenic Anemia, Type 1 Gaucher Disease.
Dosing
No dosing information is available for this tab.
Imiglucerase is administered by IV infusion after reconstitution and dilution.
Imiglucerase lyophilized powder should be stored at 2-8degreesC.
| Drug label | Dosing type | Instructions |
|---|---|---|
| Cerezyme 400 Unit Vial | Maintenance | Infuse 2.5 unit/kg over 1-2 hour(s) by intravenous route 3 times per week. |
No dosing information is available for this tab.
Drug interactions
No formal drug interaction studies have been performed.
There are no contraindication interactions for this drug.
There are no severe interactions for this drug.
There are no moderate interactions for this drug.
Contraindications
*None.
There are no contraindication contraindications for this drug.
There are no severe contraindications for this drug.
- No disease contraindications
Adverse reactions
The most common adverse effects reported in adult and pediatric patients administered imiglucerase include back pain, chills, dizziness, fatigue, headache, hypersensitivity reactions, nausea, pyrexia, and vomiting.
More Frequent
- Tachycardia
Rare or Very Rare
- Anaphylaxis
- Angioedema
- Cyanosis
- Dyspnea
- Hypersensitivity drug reaction
- Urticaria
More Frequent
- Back pain
- Chills
- Diarrhea
- Fatigue
- Fever
Less Frequent
- Abdominal pain with cramps
- Dizziness
- Headache disorder
- Hypotension
- Nausea
- Pruritus of skin
- Skin rash
Rare or Very Rare
- Flushing
- Hypertension
- Injection site sequelae
Precautions
No enhanced messaging available.
Use of imiglucerase in pediatric patients with non-CNS manifestations of Type 1 Gaucher disease is supported by evidence from adequate and well-controlled studies in adults and pediatric patients >=12 years of age with additional data obtained from an observational study of Type 1 Gaucher disease in pediatric patients.
Use of imiglucerase in pediatric patients with non-CNS manifestations of Type 3 Gaucher disease is supported by evidence from adequate and well-controlled studies in adults and pediatric patients >=12 years of age with Type 1 Gaucher disease and additional data obtained from an observational study of Type 3 Gaucher disease in adults and pediatric patients.
No drug-associated risk of major birth defects, miscarriage, or adverse maternal or fetal outcomes was identified in pregnant women from clinical data within the international Gaucher disease registry, postmarketing reports, observational studies, and case reports. Adverse pregnancy outcomes may occur due to untreated Gaucher disease.
Pregnant women exposed to imiglucerase and clinicians are encouraged to contact the Gaucher patient registry by calling 1-800-745-4447 ext. 15500 or visiting https://www.registrynxt.com.
Clinical data suggest a small amount of imiglucerase is present in human milk immediately following an infusion. No adverse effects were reported in breastfed infants of lactating women administered imiglucerase in published postmarketing and case reports. There are no data on the effects of imiglucerase on milk production. The developmental and health benefits of breastfeeding should be considered along with the mother's clinical need for imiglucerase and any potential adverse effects on the breastfed child from the drug or the underlying maternal condition.
Lactating women with Gaucher disease treated with imiglucerase are encouraged to enroll in the Gaucher patient registry by calling 1-800-745-4447 ext. 15500 or visiting https://www.registrynxt.com.
Among patients with Type 1 Gaucher disease in a single study who had hemoglobin or platelet counts 1 to 3 years after initiating imiglucerase, 6% of patients were >=65 years of age and 1% were >=75 years of age. No overall differences in the efficacy of imiglucerase were observed between geriatric patients and younger adults with Type 1 Gaucher disease.
The safety of imiglucerase in patients >=65 years of age was assessed using data from a pharmacovigilance database; no new safety findings were reported. There was an insufficient number of patients >=65 years of age in clinical studies of Type 1 Gaucher disease in order to detect differences in safety between older and younger adult patients.
There was an insufficient number of patients >=65 years of age in clinical studies of Type 3 Gaucher disease in order to detect differences in efficacy and safety between older and younger adult patients.
ICD codes
| Code | Type | Description |
|---|---|---|
| Gaucher's disease | ||
| E75.22 | ICD10CM | Gaucher disease |
ICD code information from First Databank (FDB).
Formulary status
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Last reviewed · Source: First Databank (FDB) drug data
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