Safety of Abatacept in patients with interstitial lung disease and Common variable immunodeficiency (CVID) and related disease

Phase 2

• Conditions: D83.9; J84.9; Common variable immunodeficiency, unspecified; Interstitial pulmonary disease, unspecified

• Registration Number: DRKS00008783
• Lead Sponsor: niversitätsklinikum Freiburg

Brief Summary

Not available

Detailed Description

Not available

Study Timeline

• Study Start: 2016-05-31
• Study Completion: 2018-09-26
• Last Update Posted: 19 August 2024

Recruitment & Eligibility

• Status: Complete
• 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. Interstitial lung disease or granuloma diagnosed by chest CT positive for nodules, lines or ground-glass signs
3. Age 18 years and above
4. Signed written informed consent
5. Not responsive to steroid monotherapy for a minimum of three months or intolerance of steroid therapy
6. Need for intervention measured by either reduced oxygen saturation, drop of oxygen saturation under exercise or reduced DLCOcSB
OR
progress of interstitial lung disease measured by progressive changes in the CT scan of the lung

Exclusion Criteria

1. Patient without legal capacity who is unable to understand the nature, significance and consequences of the study
2. Other immunosuppressive therapy including biologicals beyond steroid at screening phase
3. Previous treatment with Abatacept
4. Active Hepatitis B infection or tuberculosis infection
5. Other Uncontrolled infection
6. Lymphoma within the past 5 years
7. Pregnancy indicated by positive urine pregnancy test at screening
8. Breast feeding patients
9. Fertile patients refusing to use safe contraceptive methods during the study
10. Simultaneous participation in other interventional clinical trials

Study & Design

• Study Type: interventional
• Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
(1) Number and type of severe infections (intravenous treatment, hospitalization, ICU, opportunistic infection, death) under treatment.<br>(2) Number, type and severity of all other (serious) adverse events ((S)AE) in relation to study drug.<br>Data collection over a time period of 13 months<br>

Secondary Outcome Measures

Name	Time	Method
(1) lung function parameter (DLCOcSB).<br>(2) other lung function parameters: FVC, DLCOc/VA, TLC SB, Predicted forced expiratory volume in one second FEV1 [L], pO2 at rest and under exercise (six minute walking test). <br>(3) Laboratory parameters: sIL-2-Receptor, Neopterin. <br>(4) CT scan lung: Global and specific scores for nodules, lines, consolidation and ground glass.<br>(5) Cumulative steroid dose.<br>(6)Quality of life measured by SF36, CRDQ and SGRQ.<br>(7)Overall Survival.<br><br>Prospective data collection at Baseline (day 0) and Follow-up visits at 3, 6 and 12 months (each +/- 14 days) after start of treatment (= Baseline (day 0)).<br><br>Additionally for (1),(2),(3),(4),(5): Retrospective data collection up to 12 months or all available data respectively.