Analysis of Disease Modulation by Immunoglobulin Replacement in patients with variable immunodeficiency syndrome (CVID)

Recruiting

Conditions: D83.9
Common variable immunodeficiency, unspecified

Registration Number: DRKS00034189

Lead Sponsor: niversitätsklinikum Freiburg

Brief Summary: Not available

Detailed Description: Not available

Recruitment & Eligibility

Status: Recruiting

Sex: All

Target Recruitment: 10

Inclusion Criteria

1. Diagnosis of CVID according to ESID/PAGID criteria or diagnosis of related disorders which fulfill the diagnostic criteria for CVID.
2. Patients with the need for IgGRT according to standard guidelines.
3. Patients who received an initial intravenous IgG replacement therapy (IVIG)
4. Signed written informed consent

Exclusion Criteria

1. Immunosuppressive therapy beyond 7.5 mg prednisone equivalent/day during the observation period, including biologicals
2. Patients on IgGRT within the last two years before collection of baseline data
3. Lymphoma or other form of malignancy within the past 5 years before collection of baseline data

Study & Design

Study Type: observational

Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
Defined set of candidate biomarkers, to distinguish the immune response at three months after initiation of IgGRT in infection-only-CVID (CVIDio) patients versus patients with inflammatory and autoimmune complications-CVID (CVIDc)

Secondary Outcome Measures

Name	Time	Method
• Identified pathways, cellular and molecular components to distinguish CVIDio vs CVIDc before initiation of IgGRT<br>• Identified pathways, cellular and molecular components to distinguish CVIDio vs CVIDc immediate and six months after initiation of IgGRT<br>• Defined set of biomarkers distinguishing the immune status before, immediately, and six months after initiation of IgGRT in CVIDio vs CVIDc.