tiopronin (Rx)

Brand and Other Names:Thiola, Tiopronin DR, more...Tiopronin-K DR, Thiola EC
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Dosing & Uses

AdultPediatric

Dosage Forms & Strengths

tablet (Thiola)

  • 100mg

tablet, delayed-release (Thiola EC)

  • 100mg
  • 300mg

Prevention of Nephrolithiasis

Indicated, in combination with high fluid intake, alkali, and diet modification, for the prevention of cystine stone formation in adults and pediatric patients ≥20 kg with severe homozygous cystinuria, who are not responsive to these measures alone

Initial: 800 mg/day PO divided TID

Adjust dose to maintain urinary cystine <250 mg/L (average daily dose is ~1000 mg/day)

Consider starting at lower dose with history of severe toxicity to d-penicillamine

Dosing Considerations

Measure urinary cystine 1 month after initiating and every 3 months thereafter

Assess for proteinuria before treatment and every 3-6 months during treatment

Discontinue in patients who develop proteinuria and monitor urinary protein and renal function

Consider restarting treatment at a lower dosage after resolution of proteinuria

Dosage Forms & Strengths

tablet (Thiola)

  • 100mg

tablet, delayed-release (Thiola EC)

  • 100mg
  • 300mg

Prevention of Nephrolithiasis

Indicated, in combination with high fluid intake, alkali, and diet modification, for the prevention of cystine stone formation in adults and pediatric patients ≥20 kg with severe homozygous cystinuria, who are not responsive to these measures alone

<20 kg: Safety and efficacy not established

≥20 kg

  • 15 mg/kg/day PO divided TID; not to exceed 50 mg/kg/day
  • Adjust dose to maintain urinary cystine levels <250 mg/L
  • Consider starting at lower dose with history of severe toxicity to d-penicillamine

Dosing Considerations

Measure urinary cystine 1 month after initiating and every 3 months thereafter

Assess for proteinuria before treatment and every 3-6 months during treatment

Discontinue in patients who develop proteinuria and monitor urinary protein and renal function

Consider restarting treatment at a lower dosage after resolution of proteinuria

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Interactions

Interaction Checker

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      Serious - Use Alternative

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            Contraindicated (0)

              Serious - Use Alternative (0)

                Monitor Closely (1)

                • ethanol

                  ethanol increases levels of tiopronin by Other (see comment). Modify Therapy/Monitor Closely. Comment: Tiopronin is released faster from the delayed-release product (Thiola EC) in the presence of alcohol and the risk for adverse events when taken with alcohol is unknown. Avoid alcohol consumption 2 hr before and 3 hr after taking delayed-release tiopronin.

                Minor (0)

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                  Adverse Effects

                  >10%

                  Nausea (12-25%)

                  Oral ulcers (12-18%)

                  Diarrhea/soft stools (6-18%)

                  Fatigue (14%)

                  Rash (12-14%)

                  Arthralgia (12%)

                  Weakness (4-12%)

                  1-10%

                  Emesis (10%)

                  Proteinuria (6-10%)

                  Fever (8%)

                  Anorexia (8%)

                  Urticaria (8%)

                  Abdominal pain (6%)

                  Peripheral edema (6%)

                  Chest pain (6%)

                  Impotence (6%)

                  Cough (6%)

                  Ecchymosis (6%)

                  Skin wrinkling (6%)

                  Pruritus (4-6%)

                  Anemia (2-6%)

                  Frequency Not Defined

                  Reduced taste perception

                  Hypogeusia

                  Postmarketing Reports

                  Cardiac disorders: CHF

                  Ear and labyrinth disorders: Vertigo

                  Gastrointestinal disorders: Abdominal discomfort, distension. or pain; chapped lips; diarrhea; dry mouth; dyspepsia; eructation; flatulence; gastrointestinal disorder; gastroesophageal reflux disease; nausea; vomiting; jaundice; liver transaminitis

                  General disorders and administration site conditions: Asthenia; chest pain; fatigue; malaise; pain; peripheral swelling; pyrexia; swelling

                  Investigations: GFR decreased; weight increased

                  Metabolism and nutrition disorders: Decreased appetite; dehydration; hypophagia

                  Musculoskeletal and connective tissue disorders: Arthralgia; back pain; flank pain; joint swelling; limb discomfort; musculoskeletal discomfort; myalgia; neck pain; pain in extremity

                  Nervous system disorders: Ageusia; burning sensation; dizziness; dysgeusia; headache; hypoesthesia

                  Renal and urinary disorders: Nephrotic syndrome; proteinuria; renal failure

                  Skin and subcutaneous tissue disorders: Dry skin; hyperhidrosis; pemphigus foliaceus; pruritus; rash; rash pruritic; skin irritation; skin texture abnormal; skin wrinkling; urticaria

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                  Warnings

                  Contraindications

                  Hypersensitivity

                  Cautions

                  Hypersensitivity reactions reported including drug fever, rash, fever, arthralgia, and lymphadenopathy

                  Not recommended if breastfeeding

                  Proteinuria

                  • Proteinuria reported, including nephrotic syndrome and membranous nephropathy
                  • Pediatric patients receiving >50 mg/kg/day may be at increased risk
                  • Monitor for development of proteinuria and discontinue therapy in patients who develop proteinuria

                  Drug interaction overview

                  • Thiola EC: Tiopronin is released faster from Thiola EC in the presence of alcohol and the risk for adverse events associated when taken with alcohol is unknown
                  • Avoid alcohol consumption 2 hr before and 3 hr after taking Thiola EC
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                  Pregnancy & Lactation

                  Pregnancy

                  Available published case report data with tiopronin have not identified a drug-associated risk for major birth defects, miscarriage, or adverse maternal or fetal outcomes

                  Clinical considerations

                  • Renal stones in pregnancy may increase risk of adverse pregnancy outcomes (eg, preterm birth, low birth weight)

                  Animal data

                  • No findings of fetal malformations could be attributed to the drug in reproduction studies in mice and rats at doses up to 2 times the highest recommended human dose of 2 grams/day (based on mg/m²)

                  Lactation

                  There are no data on the presence of tiopronin in either human or animal milk or on the effects of the breastfed child

                  Prescribing information describes a published study suggesting tiopronin may suppress milk production

                  Because of the potential for serious adverse reactions, including nephrotic syndrome, advise patients that breastfeeding is not recommended during treatment

                  Pregnancy Categories

                  A: Generally acceptable. Controlled studies in pregnant women show no evidence of fetal risk.

                  B: May be acceptable. Either animal studies show no risk but human studies not available or animal studies showed minor risks and human studies done and showed no risk.

                  C: Use with caution if benefits outweigh risks. Animal studies show risk and human studies not available or neither animal nor human studies done.

                  D: Use in LIFE-THREATENING emergencies when no safer drug available. Positive evidence of human fetal risk.

                  X: Do not use in pregnancy. Risks involved outweigh potential benefits. Safer alternatives exist.

                  NA: Information not available.

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                  Pharmacology

                  Mechanism of Action

                  Tiopronin is an active reducing agent which undergoes thiol-disulfide exchange with cystine to form a mixed disulfide of tiopronin-cysteine; from this reaction, a water-soluble mixed disulfide is formed and the amount of sparingly soluble cystine is reduced

                  Absorption

                  Peak plasma time: 1 hr (immediate-release); 3 hr (delayed-release)

                  Elimination

                  Excretion: Urine 48% (4 hr); 78% (72 hr)

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                  Administration

                  Oral Administration

                  Thiola: Take at least 1 hr before meals or 2 hr after meals

                  Thiola EC

                  • May take with or without food
                  • Swallow tablet whole; do not chew, crush, or split

                  Unable to swallow tablet whole

                  • Crush and mix with applesauce
                  • Administration with other liquids or foods has not been studied and not recommended
                  • Preparation and administration mixed in applesauce
                    • For patients who can swallow semi-solid food
                    • Crush tablet in a clean pill crusher or mortar and pestle; always crush 1 tablet at a time
                    • Measure ~1 tablespoon of applesauce and transfer it into a container with the crushed tablet
                    • Mix crushed tablet in applesauce until powder is well dispersed
                    • Administer entire drug-applesauce mixture immediately
                    • If this is not possible, may refrigerate mixture for up to 2 hr after adding crushed tablet to applesauce; discard any mixture that has not been given within 2 hr
                    • To assure that any leftover applesauce mixture from container is recovered, add tap water to the same container, mix, and administer

                  Storage

                  Store at 25°C (77°F); excursions permitted to 15-30°C (59-86°F)

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                  Images

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                  Patient Handout

                  Patient Education
                  tiopronin oral

                  TIOPRONIN - ORAL

                  (tie-oh-PRO-nin)

                  COMMON BRAND NAME(S): Thiola

                  USES: This medication is used to prevent kidney stones in people with a certain inherited disorder (cystinuria). Cystinuria occurs when there is too much of a certain natural substance (the amino acid cystine) in the urine, leading to formation of kidney stones. Tiopronin works by making cystine more dissolvable in the urine.This medication should be used in combination with other methods to prevent kidney stones (such as drinking plenty of water, taking alkali treatment, following a special diet).

                  HOW TO USE: Take this medication by mouth 1 hour before or 2 hours after meals as directed by your doctor, usually 3 times daily.Continue to drink plenty of water and to take the alkali medication as directed by your doctor. Closely follow the diet recommended by your doctor.The dosage is based on your medical condition, lab tests, and response to treatment. For children, the dosage may be based on their weight.Use this medication regularly to get the most benefit from it. To help you remember, take it at the same times each day.Do not increase your dose or use this drug more often or for longer than prescribed. Your condition will not improve any faster, and your risk of side effects will increase.Tell your doctor if your condition gets worse.

                  SIDE EFFECTS: Nausea, diarrhea, or decreased sense of taste/smell may occur. If any of these effects last or get worse, tell your doctor or pharmacist promptly.Remember that your doctor has prescribed this medication because he or she has judged that the benefit to you is greater than the risk of side effects. Many people using this medication do not have serious side effects.Tell your doctor right away if you have any serious side effects, including: muscle/joint pain, swollen lymph nodes, loss of appetite, tiredness, signs of kidney problems (such as change in the amount of urine, painful urination, cloudy/bloody/frothy urine), muscle weakness.A very serious allergic reaction to this drug is rare. However, get medical help right away if you notice any symptoms of a serious allergic reaction, including: rash, itching/swelling (especially of the face/tongue/throat), severe dizziness, trouble breathing.This is not a complete list of possible side effects. If you notice other effects not listed above, contact your doctor or pharmacist.In the US -Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088 or at www.fda.gov/medwatch.In Canada - Call your doctor for medical advice about side effects. You may report side effects to Health Canada at 1-866-234-2345.

                  PRECAUTIONS: Before taking tiopronin, tell your doctor or pharmacist if you are allergic to it; or if you have any other allergies. This product may contain inactive ingredients, which can cause allergic reactions or other problems. Talk to your pharmacist for more details.Before using this medication, tell your doctor or pharmacist your medical history, especially of: liver disease, kidney disease.Before having surgery, tell your doctor or dentist about all the products you use (including prescription drugs, nonprescription drugs, and herbal products).During pregnancy, this medication should be used only when clearly needed. Discuss the risks and benefits with your doctor.It is unknown if this medication passes into breast milk. Because of possible risk to the infant, breast-feeding while using this drug is not recommended. Consult your doctor before breast-feeding.

                  DRUG INTERACTIONS: Drug interactions may change how your medications work or increase your risk for serious side effects. This document does not contain all possible drug interactions. Keep a list of all the products you use (including prescription/nonprescription drugs and herbal products) and share it with your doctor and pharmacist. Do not start, stop, or change the dosage of any medicines without your doctor's approval.

                  OVERDOSE: If someone has overdosed and has serious symptoms such as passing out or trouble breathing, call 911. Otherwise, call a poison control center right away. US residents can call their local poison control center at 1-800-222-1222. Canada residents can call a provincial poison control center.

                  NOTES: Do not share this medication with others.Lab and/or medical tests (such as urine cystine levels, urine tests) should be done while you are taking this medication. Keep all medical and lab appointments. Consult your doctor for more details.

                  MISSED DOSE: If you miss a dose, take it as soon as you remember. If it is near the time of the next dose, skip the missed dose. Take your next dose at the regular time. Do not double the dose to catch up.

                  STORAGE: Store at room temperature away from light and moisture. Do not store in the bathroom. Keep all medications away from children and pets.Do not flush medications down the toilet or pour them into a drain unless instructed to do so. Properly discard this product when it is expired or no longer needed. Consult your pharmacist or local waste disposal company.

                  Information last revised July 2019. Copyright(c) 2021 First Databank, Inc.

                  IMPORTANT: HOW TO USE THIS INFORMATION: This is a summary and does NOT have all possible information about this product. This information does not assure that this product is safe, effective, or appropriate for you. This information is not individual medical advice and does not substitute for the advice of your health care professional. Always ask your health care professional for complete information about this product and your specific health needs.

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                  Formulary

                  FormularyPatient Discounts

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                  • View the formulary and any restrictions for each plan.
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                  The above information is provided for general informational and educational purposes only. Individual plans may vary and formulary information changes. Contact the applicable plan provider for the most current information.

                  Tier Description
                  1 This drug is available at the lowest co-pay. Most commonly, these are generic drugs.
                  2 This drug is available at a middle level co-pay. Most commonly, these are "preferred" (on formulary) brand drugs.
                  3 This drug is available at a higher level co-pay. Most commonly, these are "non-preferred" brand drugs.
                  4 This drug is available at a higher level co-pay. Most commonly, these are "non-preferred" brand drugs or specialty prescription products.
                  5 This drug is available at a higher level co-pay. Most commonly, these are "non-preferred" brand drugs or specialty prescription products.
                  6 This drug is available at a higher level co-pay. Most commonly, these are "non-preferred" brand drugs or specialty prescription products.
                  NC NOT COVERED – Drugs that are not covered by the plan.
                  Code Definition
                  PA Prior Authorization
                  Drugs that require prior authorization. This restriction requires that specific clinical criteria be met prior to the approval of the prescription.
                  QL Quantity Limits
                  Drugs that have quantity limits associated with each prescription. This restriction typically limits the quantity of the drug that will be covered.
                  ST Step Therapy
                  Drugs that have step therapy associated with each prescription. This restriction typically requires that certain criteria be met prior to approval for the prescription.
                  OR Other Restrictions
                  Drugs that have restrictions other than prior authorization, quantity limits, and step therapy associated with each prescription.
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                  Medscape prescription drug monographs are based on FDA-approved labeling information, unless otherwise noted, combined with additional data derived from primary medical literature.