EFFECTS OF ANTIBODY REMOVAL BY IMMUNOADSORPTION ON THE IMMUNE PHENOTYPE IN PATIENTS WITH ANTIBODY MEDIATED DISEASES - A *TARGET-TO-B* STUDY

Recruiting

• Conditions: ANCA-associated vasculitis; anti-GBM disease and cryoglobulinemic vasculitis (vasculitis = small vessel inflammatory disease); Myasthenia Gravis; 10003816; 10029317; 10029149

• Registration Number: NL-OMON48107
• Lead Sponsor: niversiteit Maastricht

Brief Summary

Not available

Detailed Description

Not available

Study Timeline

• Last Update Posted: 15 April 2024

Recruitment & Eligibility

• Status: Recruiting
• Sex: Not specified
• Target Recruitment: 80

Inclusion Criteria

In order to be eligible to participate in this study, a subject must meet the
following criteria:

#Patients with a new or previous diagnosis of:
* Anti-AChR or anti-MuSK Myasthenia Gravis
* MPO- or PR3-ANCA associated vasculitis
* Anti-GBM glomerulonephritis
* Cryoglobulinemia vasculitis with severe organ involvement
* Refractory and severe antibody-mediated diseases*

# Patients with an indication for plasma exchange or IA based on:
1. AChR or MuSK-positive MG with at least one of the following features:
- Insufficient clinical response to immunosuppressive therapy of severe
ocu-lar, bulbar or generalized muscle weakness.
- Exacerbation with severe bulbar or respiratory dysfunction.

2. MPO- or PR3-ANCA vasculitis defined at least by one of the following
features:
- Renal involvement indicated by renal biopsy and/or glomerular erythrocy-turia
WITH active glomerulonephritis and newly reported eGFR < 50 ml/min/1.73 m²
and/or rapidly deteriorating renal function.
- Pulmonary hemorrhage. Anti-GBM syndrome with at least one of the fol-lowing
features:

3. Anti-GBM syndrome with at least one of the following features:
- Anti-GBM glomerulonephritis proven by renal biopsy and/or urine sediment
suspecting active glomerulonephritis and newly reported eGFR < 50 ml/min/1.73
m² and/or rapidly deteriorating renal function.
- Pulmonary hemorrhage with anti-GBM autoantibodies (> 10 IU/ml).
- Serological evidence of circulating anti-GBM autoantibodies (> 10 IU/ml).

4. Cryoglobulinemia vasculitis with at least one of the following features:
- Symptomatic hyperviscosity syndrome.
- (Life-threatening) organ involvement (e.g. renal involvement (proven by renal
biopsy and newly reported eGFR < 50 ml/min/1.73 m² and/or rapidly deteriorating
renal function), pulmonary hemorrhage, progressive neuropa-thy).
- Severe, refractory cutaneous vasculitis.

5. Refractory antibody-mediated diseases
- This category may include autoimmune haemolytic anemia or other anti-body
positive B-cell mediated autoimmune diseases.
- Based on case-based peer review between the investigators, patients with rare
refractory antibody-mediated diseases can be considered for inclu-sion. In case
of paediatric patients, additional reviewing by the paediatric immunologist
involved in the *Target-to-B* consortium (Prof. dr. T. Kuijpers) can be
obtained before inclusion.

Exclusion Criteria

- Pregnancy at time of study entry.
- Previously reported allergic reactions to the immunosuppressive therapies or
IA.
- Plasmapheresis or IA within 3 months before inclusion.
- Intravenous immunoglobulin within 3 months before inclusion.
- Patients aged < 6 years.
- Treatment with >7 days of oral cyclophosphamide or >1 IV dose of
cyclophos-phamide within 3 months prior to inclusion and/or >7 days of
predni-sone/prednisolone (>30 mg/day or > 1 mg/kg/day in paediatric patients)
within 1 month prior to inclusion expect for patients with MG and/or >1 dose of
rituximab within 9 months prior to inclusion.

Study & Design

• Study Type: Observational invasive
• Study Design: Not specified