Actively Recruiting

Phase 2
Age: 18Years +
All Genders
NCT06782373

A Study to Assess the Effectiveness and Safety of Pacritinib in Patients With VEXAS Syndrome (PAXIS)

Led by Swedish Orphan Biovitrum · Updated on 2026-04-24

78

Participants Needed

39

Research Sites

155 weeks

Total Duration

On this page

Sponsors

S

Swedish Orphan Biovitrum

Lead Sponsor

P

PSI CRO

Collaborating Sponsor

AI-Summary

What this Trial Is About

This trial is to assess the effectiveness and safety of pacritinib in patients with VEXAS (i.e., Vacuoles in myeloid progenitors, E1 ubiquitin-activating enzyme, X-linked, autoinflammatory manifestations, and somatic) syndrome. 78 participants will be enrolled, randomized to either pacritinib dose A, pacritinib dose B + placebo, or placebo. Randomization will be stratified by prescribed GC dose on the day of randomization.

CONDITIONS

Official Title

A Study to Assess the Effectiveness and Safety of Pacritinib in Patients With VEXAS Syndrome (PAXIS)

Who Can Participate

Age: 18Years +
All Genders

Eligibility Criteria

Eligible

You may qualify if you...

  • Age 18 years or older
  • Documented pathogenic UBA1 mutation at methionine-41 or nearby splice site by genetic testing
  • Evidence of inflammatory involvement in at least one organ system (skin, blood vessels, joints, eyes, periorbital area, urinary tract, lungs) within 6 months prior to enrollment
  • Receiving stable glucocorticoid therapy with prednisone or prednisolone between 15 and 45 mg daily for at least 4 consecutive weeks
  • Karnofsky Performance Status of 50% or higher
  • Adequate organ function based on liver enzymes, bilirubin, kidney function, blood counts, clotting tests, and peripheral blood blast count
  • QTcF less than or equal to 450 msec in males or 470 msec in females; QRS less than or equal to 100 msec unless QTcF is less than or equal to 480 msec
  • Women of child-bearing potential must have negative pregnancy tests and agree to use effective contraception
  • Male participants must agree to use effective contraception
Not Eligible

You will not qualify if you...

  • Previous allogenic stem cell or solid organ transplant (except corneal)
  • Using systemic glucocorticoids for conditions other than VEXAS that interfere with study
  • More than one ICU admission for VEXAS flare in last 6 months
  • Received 9 or more units of red blood cell transfusions in 90 days before enrollment
  • Known high-risk myelodysplastic syndrome requiring treatment or transplant
  • Malignancy within 1 year except certain skin cancers or carcinoma in situ
  • Recent exposure to hypomethylating agents or certain anti-inflammatory or blood support therapies
  • Recent use of anti-platelet therapy except low-dose aspirin
  • Known multiple myeloma or certain blood protein abnormalities
  • Use of strong CYP3A4 inhibitors or inducers recently
  • Significant recent bleeding events
  • Significant cardiovascular disease or abnormal ECG findings
  • Recent arterial or venous blood clots
  • Moderate or severe liver impairment or active viral hepatitis
  • Uncontrolled HIV or detectable viral load on antiretrovirals
  • Positive tuberculosis test
  • History of disseminated mycobacterial infection
  • Concurrent participation in other interventional trials or recent experimental therapy
  • Pregnant, planning pregnancy, or breastfeeding
  • Active infection requiring systemic antibiotics except prophylactic or chronic use
  • Allergy to pacritinib or its inactive ingredients including microcrystalline cellulose, polyethylene glycol, magnesium stearate

AI-Screening

AI-Powered Screening

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Trial Site Locations

Total: 39 locations

1

Mayo Clinic - Scottsdale

Scottsdale, Arizona, United States, 85259

Actively Recruiting

2

Dana Farber Cancer Institute

Boston, Massachusetts, United States, 02215

Actively Recruiting

3

Mayo Clinic - Rochester

Rochester, Minnesota, United States, 55905

Actively Recruiting

4

NYU Langone Health

New York, New York, United States, 10016

Actively Recruiting

5

Cleveland Clinic - Cleveland

Cleveland, Ohio, United States, 44195

Actively Recruiting

6

The James Cancer Hospital and Solove Research Institute

Columbus, Ohio, United States, 43210

Actively Recruiting

7

UT MD Anderson Cancer Center

Houston, Texas, United States, 77030

Actively Recruiting

8

University of Utah Healthcare

Salt Lake City, Utah, United States, 84132

Actively Recruiting

9

Fred Hutchinson Cancer Center

Seattle, Washington, United States, 98109

Actively Recruiting

10

Vancouver Coastal Health Research Institute

Vancouver, British Columbia, Canada, V5Z 1M9

Actively Recruiting

11

Queen Elizabeth II Health Sciences Center

Halifax, Nova Scotia, Canada, B3H 2Y9

Actively Recruiting

12

Princess Margaret Cancer Centre

Toronto, Ontario, Canada, M5G 2M9

Actively Recruiting

13

Hospital du Sacre-Coeur in Montreal

Montreal, Quebec, Canada, H4J 1C5

Actively Recruiting

14

Lille University Hospital Center

Lille, France, 59037

Actively Recruiting

15

Saint-Antoine Hospital - APHP

Paris, France, 75012

Actively Recruiting

16

Tenon Hospital - APHP

Paris, France, 75020

Actively Recruiting

17

Hospices Civils de Lyon - Lyon Sud

Pierre-Bénite, France, 69310

Actively Recruiting

18

University Hospital Center of Poitiers

Poitiers, France, 86000

Actively Recruiting

19

IUCT-Oncopole

Toulouse, France, 31100

Actively Recruiting

20

University Hospital Tuebingen, Medical Clinic II, Hematology, Oncology, Clinical Immunology and Rheumatology

Tübingen, Baden-Wurttemberg, Germany, 72076

Actively Recruiting

21

Hospital Rechts der Isar of the Technical University of Munich, Clinic and Polyclinic for Internal Medicine III: Hematology and Internal Oncology

Munich, Bavaria, Germany, 81675

Actively Recruiting

22

University Hospital Hamburg-Eppendorf

Hamburg, Free and Hanseatic City of Hamburg, Germany, 20246

Actively Recruiting

23

University Hospital Duesseldorf

Düsseldorf, North Rhine-Westphalia, Germany, 40225

Actively Recruiting

24

University Hospital Carl Gustav Carus Dresden, Medical Clinic and Polyclinic I

Dresden, Saxony, Germany, 01307

Actively Recruiting

25

University Hospital Schleswig-Holstein

Lübeck, Schleswig-Holstein, Germany, 23538

Actively Recruiting

26

Hospital San Raffaele, IRCCS, Unit of Immunology, Rheumatology, Allergy and Rare Diseases

Milan, Italy, 20132

Actively Recruiting

27

University Hospital of Padova, Rheumatology Unit, Department of Medicine - DIMED

Padova, Italy, 35128

Actively Recruiting

28

AUSL of Reggio Emilia - Hospital Arcispedale S. Maria Nuova, Complex Structure of Rheumatology

Reggio Emilia, Italy, 42123

Actively Recruiting

29

Foundation PTV - Polyclinic Tor Vergata Biomedicine and prevention

Roma, Italy, 00133

Actively Recruiting

30

Fukushima Medical University Hospital

Fukushima, Japan, 960-1295

Actively Recruiting

31

Nagasaki University Hospital

Nagasaki, Japan, 852-8501

Actively Recruiting

32

Yokohama City University Hospital

Yokohama, Japan, 236-0004

Actively Recruiting

33

Hospital Clinic of Barcelona

Barcelona, Spain, 08036

Actively Recruiting

34

Catalan Institute of Oncology, Hospital Duran i Reynals, Department of Clinical Hematology

L'Hospitalet de Llobregat, Spain, 08908

Actively Recruiting

35

University Clinical Hospital of Salamanca

Salamanca, Spain, 37007

Actively Recruiting

36

St James's University Hospital

Leeds, United Kingdom, LS9 7TF

Actively Recruiting

37

Royal Free Hospital

London, United Kingdom, NW3 2QG

Actively Recruiting

38

King's College Hospital, Department of Hematology

London, United Kingdom, SE5 9RS

Actively Recruiting

39

Churchill Hospital

Oxford, United Kingdom, OX3 7LE

Actively Recruiting

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Research Team

S

Study Physician

CONTACT

How is the study designed?

Study Type

INTERVENTIONAL

Masking

TRIPLE

Allocation

RANDOMIZED

Model

PARALLEL

Primary Purpose

TREATMENT

Number of Arms

3

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A Study to Assess the Effectiveness and Safety of Pacritinib in Patients With VEXAS Syndrome (PAXIS) | DecenTrialz