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VX-371

Phase 2

Primary Ciliary Dyskinesia | Small molecule | Dermatology |Vertex Pharmaceuticals Incorporated|Last Updated: Dec 16, 2021

Success Probability

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Trial Design

RandomizedDouble-BlindPLACEBO_CONTROLLEDDMCBiomarker
Total Trials1
Total Enrollment123

FDA Designations

No designations recorded

Clinical trial landscape

VX-371 · 1 trial · 1 indication

Phase 2 1
NCT02871778Clearing Lungs With ENaC Inhibition in Primary Ciliary DyskinesiaPrimary Ciliary Dyskinesia
COMPLETED123 Analytics
PHASE2COMPLETED
Clearing Lungs With ENaC Inhibition in Primary Ciliary Dyskinesia
Primary Ciliary DyskinesiaUnlock trial analytics

Study Endpoints

Primary Endpoints

Part A: Number of Participants With Treatment Emergent Adverse Events (TEAEs) and Serious TEAEs
Part A: From first dose of study drug up 84 days

An AE was any untoward medical occurrence in a participant who received study drug without regard to possibility of causal relationship. AEs included abnormal clinically significant findings for spirometry, clinical laboratory parameters, standard 12-lead electrocardiograms (ECGs), vital signs and pulse oximetry examinations. Serious adverse event (SAE) was an AE resulting in any of the following outcomes or deemed significant for any other reason: death; initial or prolonged inpatient hospitalization; life-threatening experience (immediate risk of dying); persistent or significant disability/incapacity; congenital anomaly. Treatment-emergent were events between first dose of study drug and up to 84 days that were absent before treatment or that worsened relative to pretreatment state. TEAEs included both serious and non-serious TEAEs.

Part B: Number of Participants With Treatment Emergent Adverse Events (TEAEs) and Serious TEAEs
Part B: Day 85 up to 28 days after last dose of study drug (56 days)

An AE was any untoward medical occurrence in a participant who received study drug without regard to possibility of causal relationship. AEs included abnormal clinically significant findings for spirometry, clinical laboratory parameters, standard 12-lead electrocardiograms (ECGs), vital signs and pulse oximetry examinations. SAE was an AE resulting in any of the following outcomes or deemed significant for any other reason: death; initial or prolonged inpatient hospitalization; life-threatening experience (immediate risk of dying); persistent or significant disability/incapacity; congenital anomaly. Treatment-emergent were events between first dose of study drug and up to up to 28 days after last dose that were absent before treatment or that worsened relative to pretreatment state. TEAEs included both serious and non-serious TEAEs.

Part A: Absolute Change From Study Baseline in Percent Predicted Forced Expiratory Volume in 1 Second (ppFEV1) at Day 29
Part A: Study Baseline, Day 29 of each treatment period

FEV1 is the volume of air that can forcibly be blown out in one second, after full inspiration. The study baseline is defined as the most recent non-missing measurement (scheduled or unscheduled) collected before the first dose of study drug in the study.

Part B: Absolute Change From Study Baseline in Percent Predicted Forced Expiratory Volume in 1 Second (ppFEV1) at Day 29
Study Baseline, Day 29 of Part B

FEV1 is the volume of air that can forcibly be blown out in one second, after full inspiration. The study baseline is defined as the most recent non-missing measurement (scheduled or unscheduled) collected before the first dose of study drug in the study.

Part B: Absolute Change From Part B Baseline in Percent Predicted Forced Expiratory Volume in 1 Second (ppFEV1) at Day 29
Part B Baseline, Day 29 of Part B

FEV1 is the volume of air that can forcibly be blown out in one second, after full inspiration. Part B baseline was defined as the most recent non-missing measurement (scheduled or unscheduled) collected before the first dose of ivacaftor in Part B and after the last dose in Period 2.

Secondary Endpoints

Part A: Change From Study Baseline in Quality of Life-Primary Ciliary Dyskinesia (QOL-PCD) (Adult Version) Lower Respiratory Symptoms Domain Score at Day 29
Study Baseline, Day 29 of Part A
Part B: Change From Study Baseline in Quality of Life-Primary Ciliary Dyskinesia (QOL-PCD) (Adult Version) Lower Respiratory Symptoms Domain Score at Day 29
Study Baseline, Day 29 of Part B
Part B: Change From Part B Baseline in Quality of Life-Primary Ciliary Dyskinesia (QOL-PCD) (Adult Version) Lower Respiratory Symptoms Domain Score at Day 29
Part B Baseline, Day 29 of Part B
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Study Design & Arms

AllocationRANDOMIZED
MaskingTRIPLE
ModelCROSSOVER
PurposeTREATMENT

Treatment Arms

ArmTypeDescription
Part A: VX-371 in Hypertonic Saline (HS), Then HSEXPERIMENTALParticipants received 85 microgram (mcg) VX-371 diluted in 3 milliliter (mL) 4.2 percent (%) HS twice daily through oral nebulized inhalation from Day 1 through Day 29 in treatment period 1 followed by a 28 day washout period (from Day 29 through Day 56) and then received 3 mL 4.2% HS through oral nebulized inhalation twice daily for 28 days (from Day 57 through Day 85) in treatment period 2.
Part A: HS, Then VX-371 in HSEXPERIMENTALParticipants received 3 mL 4.2% HS through oral nebulized inhalation twice daily from Day 1 through Day 29 in treatment period 1 followed by a 28 day washout period (from Day 29 through Day 56) and then received 85 mcg VX-371 diluted in 3 mL 4.2% HS through oral nebulized inhalation twice daily for 28 days (from Day 57 through Day 85) in treatment period 2.
Part A: VX-371, Then PlaceboEXPERIMENTALParticipants received 85 mcg VX-371 diluted in 3 mL 0.17% Saline (placebo) through oral nebulized inhalation twice daily from Day 1 through Day 29 in treatment period 1 followed by a 28 day washout period (from Day 29 through Day 56) and then received 3 mL 0.17% saline (placebo) through oral nebulized inhalation twice daily for 28 days (from Day 57 through Day 85) in treatment period 2.
Part A: Placebo, Then VX-371EXPERIMENTALParticipants received 3 mL 0.17% saline (placebo) through oral nebulized inhalation twice daily from Day 1 through Day 29 in treatment period 1 followed by a 28 day washout period (from Day 29 through Day 56) and then received 85 mcg VX-371 diluted in 3 mL 0.17% saline (placebo) through oral nebulized inhalation twice daily for 28 days (from Day 57 through Day 85) in treatment period 2.
Part B: VX-371 in HS + IvacaftorEXPERIMENTALParticipants who were on 85 mcg VX-371 diluted in 3 mL 4.2% HS through oral nebulized inhalation twice daily for 28 days (from Day 57 through Day 85) in treatment period 2 continued their inhaled study drug regimen from Treatment Period 2 and also received ivacaftor 150 mg tablet twice daily for 28 days (from Day 85 through Day 113) in treatment period 3.
Part B: HS + IvacaftorEXPERIMENTALParticipants who were on 3 mL 4.2% HS through oral nebulized inhalation twice daily for 28 days (from Day 57 through Day 85) in treatment period 2 continued their inhaled study drug regimen from Treatment Period 2 and also received ivacaftor 150 mg tablet twice daily for 28 days (from Day 85 through Day 113) in treatment period 3.
Part B: VX-371 + IvacaftorEXPERIMENTALParticipants who were on 85 mcg VX-371 diluted in 3 mL 0.17% saline (placebo) through oral nebulized inhalation twice daily for 28 days (from Day 57 through Day 85) in treatment period 2 continued their inhaled study drug regimen from Treatment Period 2 and also received ivacaftor 150 mg tablet twice daily for 28 days (from Day 85 through Day 113) in treatment period 3.
Part B: Placebo + IvacaftorPLACEBO_COMPARATORParticipants who were on 3 mL 0.17% saline (placebo) through oral nebulized inhalation twice daily for 28 days (from Day 57 through Day 85) in treatment period 2 continued their inhaled study drug regimen from Treatment Period 2 and also received ivacaftor 150 mg tablet twice daily for 28 days (from Day 85 through Day 113) in treatment period 3.

Interventions

NameTypeDescription
VX-371DRUG -
Hypertonic SalineDRUG -
Placebo (0.17% saline)DRUG -
VX-371 + HSDRUG -
IvacaftorDRUG -
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Eligibility Criteria

Age Range12 Years to N/A
SexALL
Healthy VolunteersNo
Study Sites34

Inclusion Criteria: * The subject must have evidence supportive of a PCD diagnosis. * Subjects with percent predicted FEV1 of ≥40 to \<90 percentage points * Non-smoker for at least 90 days prior to the Screening Visit and less than a 5 pack-year lifetime history of smoking * Stable regimen of medi...

Countries:United StatesCanadaDenmarkGermanyItalyNetherlandsPolandUnited Kingdom
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Frequently asked questions about VX-371

What is VX-371 used for?

VX-371 is an investigational small molecule being studied for cystic fibrosis and primary ciliary dyskinesia. It has been evaluated in Phase 2 clinical trials for these conditions. The drug is not approved and remains in clinical development.

Who makes VX-371?

VX-371 is being developed by Vertex Pharmaceuticals Incorporated, a biopharmaceutical company traded on the NASDAQ under the ticker VRTX. Vertex has sponsored Phase 2 clinical trials of VX-371 in cystic fibrosis and primary ciliary dyskinesia.

What phase is VX-371 in?

VX-371 is in Phase 2 clinical development. Two Phase 2 trials have been completed, one in cystic fibrosis and one in primary ciliary dyskinesia. VX-371 is an investigational drug and has not been approved by regulatory authorities.

What clinical trials has VX-371 been in?

VX-371 has been studied in two completed Phase 2 trials. NCT02709109 evaluated VX-371 in 147 patients with cystic fibrosis who are homozygous for the F508del-CFTR mutation. NCT02871778 evaluated VX-371 in 123 patients with primary ciliary dyskinesia. Both trials were randomized, double-blind, and placebo-controlled.

Is VX-371 the same as any other drug?

VX-371 is also known by other names, though the specific alternative names are not publicly listed in the trial records. Researchers and clinicians may refer to the drug by its code name VX-371 in publications and clinical trial registries.