The effect of high dose versus low dose antibody supplementation on airway disease in primary antibody defciency

Phase 1

• Conditions: Primary antibody deficiency:- Unclassified antibody deficiency (unPAD)- IgA deficiency- Specific polysaccharide antibody deficiency (SPAD)- IgG subclass deficiency (IgSD)- Common variable immunodeficiency (CVID)- Agammaglobulinemia (X-linked or otherwise); Therapeutic area: Diseases [C] - Immune System Diseases [C20]

• Registration Number: EUCTR2021-005001-26-NL
• Lead Sponsor: MC Utrecht

Brief Summary

Not available

Detailed Description

Not available

Study Timeline

• Results First Posted: 1900-01-01
• Study Start: 2021-12-08
• Last Update Posted: 10 January 2022

Recruitment & Eligibility

• Status: Authorised-recruitment may be ongoing or finished
• Sex: All
• Target Recruitment: 100

Inclusion Criteria

In order to be eligible to participate in this study, a subject must meet all of the following criteria:
1.Age 4-60 years
2.Diagnosis of Primary Antibody Deficiency / Common Variable Immunodeficiency Disorder (see Appendix 1).
3.Indication for immunoglobulin replacement therapy and/or treated with immunoglobulin replacement therapy
4.Current IgG dosing 0.4 - 0.6 gr / kg / 3-4 weeks
5.Receiving treatment and follow up for PAD by one of the physicians in the participating centers
6.Written informed consent
7.Normal lung status, or mild to medium severe pulmonary disease (measured by pulmonary function test and on CT scan, scored by an independent radiologist based on the following criteria):
a.Baseline AD score < 5 and ILD score < 7, or;
b.Baseline AD score > 5 and/or ILD score > 7 without clinical diagnosis of severe respiratory insufficiency (defined as: saturations in room air <92% and / or oxygen dependency).
c.Baseline pulmonary function (FEV1 and FVC >70% expected for age and body weight / length)

Are the trial subjects under 18? yes
Number of subjects for this age range: 50
F.1.2 Adults (18-64 years) yes
F.1.2.1 Number of subjects for this age range 50
F.1.3 Elderly (>=65 years) no
F.1.3.1 Number of subjects for this age range

Exclusion Criteria

A potential subject who meets any of the following criteria will be excluded from participation in this study:
1.Age above 60 year
2.Diagnosis of Combined Immunodeficiency (CID) disease at onset of study (see Appendix A). Explanation: Combined Immunodeficiency is featured by the occurrence of more viral infections and reactivations and thus less comparable to PAD.
3.Severe pulmonary disease, determined by an independent radiologist:
a.Baseline AD score > 5 and/or ILD score > 7, in combination with:
i.Saccular bronchiectasis on CT scan, or;
ii.Clinical diagnosis of severe respiratory insufficiency (defined as: (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)

Study & Design

• Study Type: Interventional clinical trial of medicinal product
• Study Design: Not specified