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Clinical Trials/2024-513124-41-00
2024-513124-41-00
Not yet recruiting
Phase 3

Influencing Progression of Airway Disease in Primary Antibody Deficiency

Universitair Medisch Centrum Utrecht3 sites in 1 country100 target enrollmentStarted: July 12, 2024Last updated:

Overview

Phase
Phase 3
Status
Not yet recruiting
Sponsor
Universitair Medisch Centrum Utrecht
Enrollment
100
Locations
3
Primary Endpoint
Difference in mean AD and ILD scores (as measured with CT scanning) between t=0 and t=2 years in patients with standard vs higher Ig replacement therapy dosing.

Overview

Brief Summary

To show the protective value and to measure cost effectiveness of higher Ig dosing on progression of lung disease in PAD.

Eligibility Criteria

Ages
0 years to 64 years (18-64 Years, 0-17 Years)
Accepts Healthy Volunteers
Yes

Inclusion Criteria

  • Age 8-60 years
  • Diagnosis of Primary Antibody Deficiency / Common Variable Immunodeficiency Disorder (see Appendix I protocol)
  • Indication for immunoglobulin replacement therapy and/or treated with immunoglobulin replacement therapy
  • Current IgG dosing 0.25 - 0.6 gr / kg / 3-4 weeks
  • Receiving treatment and follow-up for PAD by a physician in one of the participating centers
  • Written informed consent

Exclusion Criteria

  • Diagnosis of Combined Immunodeficiency (CID) disease at onset of study (see Appendix 1). Explanation: Combined Immunodeficiency is featured by the occurrence of more viral infections and reactivations and thus less comparable to PAD.
  • Severe pulmonary disease, determined by an independent radiologist: a. Baseline AD score > 7 and/or ILD score > 5, in combination with: i. Saccular bronchiectasis on CT scan, or; ii. Clinical diagnosis of severe respiratory insufficiency ( (defined as: saturations in room air <92%, and/ or oxygen dependency). b. Baseline pulmonary function (FEV1 and FVC) <70% expected for age and body weight / length)
  • Active smoker

Outcomes

Primary Outcomes

Difference in mean AD and ILD scores (as measured with CT scanning) between t=0 and t=2 years in patients with standard vs higher Ig replacement therapy dosing.

Difference in mean AD and ILD scores (as measured with CT scanning) between t=0 and t=2 years in patients with standard vs higher Ig replacement therapy dosing.

Secondary Outcomes

  • Number of upper- and lower respiratory tract infections before onset of study and during study, i.e.: Sino pulmonary disease, otitis, pneumonia, collected by study CRF's.
  • Pulmonary symptoms will be evaluated on a daily basis (during two periods of 2 months) using a diary that can be directly entered in Castor.
  • Days missed from school and work due to infections, measured by the PCQ (productivity cost questionnaire) instrument.
  • Quality of life, measured with the EQ-5D questionnaire
  • Ig dosing and IgG trough levels in intervention and in control group
  • Total health costs (consisting of costs for health care professional visits, medication, hospitalizations, imaging and biochemical investigations) in intervention and control group, collected from electronic patient files.
  • Immunological laboratory phenotype, collected from electronic patient files.
  • Adverse events: reporting and monitoring of patients and Adverse Events (AEs), Serious Adverse Events (SAEs) and Suspected Unexpected Serious Adverse Reaction (SUSARs) will be done by an independent monitor.

Investigators

Sponsor
Universitair Medisch Centrum Utrecht
Sponsor Class
Hospital/Clinic/Other health care facility
Responsible Party
Principal Investigator
Principal Investigator

dr. J.M. van Montfrans

Scientific

Universitair Medisch Centrum Utrecht

Study Sites (3)

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