Tiglutik (riluzole oral suspension) for amyotrophic lateral sclerosis
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What is Tiglutik for ALS?
Tiglutik (riluzole oral suspension) is a liquid formulation of riluzole approved for treating amyotrophic lateral sclerosis (ALS). The medication can slow disease progression, extending survival time or the need for a tracheostomy, which is when a breathing tube is inserted through the neck into the trachea.
It can be taken orally or administered through a feeding tube inserted directly into the stomach, called a PEG tube.
Tiglutik is marketed by ITF Pharma in the U.S and Martindale Pharma in Europe, where it’s sold under the name Teglutik. Other formulations of riluzole are also available for ALS, including a tablet formulation called Rilutek.
Therapy Snapshot
| Brand Name: | Tiglutik |
| Chemical Name: | Riluzole |
| Usage: | Used for the treatment of ALS; can increase time to a tracheostomy and/or death |
| Administration: | Oral suspension taken by mouth or via PEG tube |
How does Tiglutik work?
Glutamate is a neurotransmitter, or chemical messenger, needed for nerve cells to communicate with each other. Excessive levels of this neurotransmitter, however, can cause damage to nerve cells by making them fire too much. This is thought to be one of the mechanisms driving ALS.
The exact mechanism of action of riluzole-based medications in ALS is not well understood. But studies suggest the compound improves nerve cell health by inhibiting glumatate signaling and lowering the amount of TDP-43 protein clumps in cells, which is also thought to contribute to nerve cell death in ALS.
Who can use Tiglutik?
The U.S. Food and Drug Administration (FDA) first approved Tiglutik in 2018 as an oral treatment with ALS. That was later expanded to include feeding tube administration, making Tiglutik the first and only riluzole formulation approved for both oral and PEG tube use.
The medication also is approved under the brand name Teglutik in multiple European countries, as well as in Israel and Turkey.
Who should not use Tiglutik?
The medication should not be used in people with severe allergies to either riluzole or any other component in Tiglutik. It’s also not recommended for people with elevated levels of liver enzymes.
How is Tiglutik administered?
Tiglutik is available in an amber glass bottle containing 300 mL of a brown, opaque oral suspension. Each 1 mL of riluzole oral suspension contains 5 mg of active drug.
The recommended dosage is 50 mg, given via an oral syringe containing 10 mL of the suspension, taken twice daily (every 12 hours). It should be administered at least one hour before or two hours after a meal.
The suspension can be taken by mouth, wherein an oral syringe containing 10 mL of the medication is placed toward the inside of the cheek and slowly emptied, or given through a PEG tube. In this case, the PEG tube should be flushed with 1 ounce (nearly 30 mL) of water before and after each administration.
Tiglutik should be stored at room temperature, upright and protected from light. Each bottle should be used within 15 days of opening and kept tightly closed between each use.
Tiglutik in clinical trials
Tiglutik’s approvals were supported by results from two clinical trials of Rilutek, wherein the tablet formulation significantly extended survival time or needing a tracheostomy compared with a placebo.
The approval package also included data from bioavailability studies in healthy volunteers showing Tiglutik is comparable to Rilutek in terms of total exposure to riluzole. However, riluzole was more rapidly absorbed into circulation and reached a peak concentration about 20% higher with Tiglutik.
Common side effects of Tiglutik
The most common side effects of Tiglutik include:
- numbness in the mouth
- generalized weakness
- nausea
- reduced lung function
- high blood pressure
- abdominal pain.
Liver damage
Based on Rilutek clinical trials, riluzole-based medications like Tiglutik may cause liver damage. Patients should be monitored regularly for this potentially life-threatening side effect and discontinue treatment if they develop signs of liver impairment. Tiglutik also is not recommended for patients with elevated levels of liver enzymes.
Low levels of white blood cells
Neutropenia, a condition characterized by lower than normal levels of immune neutrophils, has been reported within the first months of Tiglutik treatment. Because the condition is often accompanied by a fever, patients should report any increases in temperature to their healthcare provider.
Lung scarring
The medication may lead to interstitial lung disease, or scarring of lung tissue, and should be immediately discontinued if this form of lung disease develops.
Use in pregnancy and breastfeeding
Tiglutik has not been adequately studied in pregnant people, but animal data suggest it may cause harm to a developing fetus. It’s also not known if it can pass to breast milk.
It’s recommended that patients who are or plan to become pregnant, or who are nursing or plan to breastfeed, talk with their healthcare team about the potential risks and benefits of Tiglutik treatment during these periods.
ALS News Today is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.
FAQs about Tiglutik
The U.S. Food and Drug Administration (FDA) approved Tiglutik as a treatment for amyotrophic lateral sclerosis in 2018. It was initially indicated for oral administration only, but its label was expanded in 2019 to enable administration via a PEG tube, or a feeding tube placed through the belly into the stomach.
Amyotrophic lateral sclerosis (ALS) is a progressive disease in which symptoms continue to worsen over time. Tiglutik and other riluzole-based medications can prolong the time to needing a tracheostomy (a tube in the neck to help with breathing) and/or extend survival in some patients, but these therapies will generally not reduce existing ALS symptoms.
A clinical trial that supported Tiglutik’s approval, which tested the tablet formulation Rilutek, found evidence of a significant extension in the time to needing a breathing tube in the neck or death one year after treatment initiation. But because each patient will have a different disease experience, each person’s treatment response also will be unique.
There is no known interaction between Tiglutik and alcohol. However, because alcohol can cause liver damage, which is also a known side effect of riluzole-based medications, patients are advised to talk with their healthcare team to understand when and how much alcohol is safe for them to drink.
Clinical trial data has shown that Tiglutik can slow disease progression in some amyotrophic lateral sclerosis patients. However, existing medications cannot cure the disease. Patients will continue to progress over time and will experience significant limitations despite treatment.
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