HIGHLIGHTS OF PRESCRIBING INFORMATION

These highlights do not include all the information needed to use LuciBose safely and effectively. See full prescribing information for LuciBose.

INDICATIONS AND USAGE

LuciBose is an endothelin receptor antagonist indicated for the treatment of pulmonary arterial hypertension (PAH) (WHO Group 1):

• in adults to improve exercise ability and to decrease clinical worsening. Studies establishing effectiveness included predominantly patients with WHO Functional Class II-IV symptoms and etiologies of idiopathic or heritable PAH (60%), PAH associated with connective tissue diseases (21%), and PAH associated with congenital heart disease with left-toright shunts (18%) .

• in pediatric patients aged 3 years and older with idiopathic or congenital PAH to improve pulmonary vascular resistance (PVR), which is expected to result in an improvement in exercise ability.

DOSAGE AND ADMINISTRATION

• Patients older than 12 years of age: initiate at 62.5 mg orally twice daily; for patients weighing greater than 40 kg, increase to 125 mg orally twice daily after 4 weeks.

• Patients 12 years of age and younger: dosage is based on weight, full prescribing information.

• Reduce the dose and closely monitor patients developing aminotransferase elevations more than 3×Upper Limit of Normal (ULN) .

DOSAGE FORMS AND STRENGTHS

Tablets: 125 mg×56 tablets

CONTRAINDICATIONS

• Pregnancy

• Use with Cyclosporine A

• Use with Glyburide

• Hypersensitivity

WARNINGS AND PRECAUTIONS

• Fluid retention: May require intervention .

• Pulmonary veno-occlusive disease (PVOD): If signs of pulmonary edema occur, consider the diagnosis of associated PVOD and consider discontinuing LuciBose.

• Decreased sperm counts.

• Decreases in hemoglobin and hematocrit: Monitor hemoglobin levels after 1 and 3 months of treatment, then every 3 months thereafter .

ADVERSE REACTIONS

• Common adverse reactions (≥3% more than placebo) for the film-coated tablet are respiratory tract infection and anemia.

DRUG INTERACTIONS

• Cytochrome P450: Coadministration of LuciBose with drugs metabolized by CYP2C9 and CYP3A can increase exposure to LuciBose and/or the coadministered drug.

• Hormonal contraceptives: LuciBose use decreases contraceptive exposure and reduces effectiveness.

USE IN SPECIFIC POPULATIONS

• Nursing mothers: Choose breastfeeding or LuciBose.

Storage

Store at 20℃ to 25℃ (68℉ to 77℉), excursions permitted between 15℃ and 30℃ (59℉ and 86℉) [see USP Controlled Room Temperature]. Protect from moisture.