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Targeting Complement Activation in Antineutrophil Cytoplasmic Autoantibodies (ANCA)-Vasculitis - Eculizumab

Phase 2
Withdrawn
Conditions
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis
Interventions
Drug: Standard of care treatment
Registration Number
NCT01275287
Lead Sponsor
University of North Carolina, Chapel Hill
Brief Summary

The purpose of this research study is to see if Eculizumab (Soliris®) can safely be used in addition to conventional therapy in patients with active ANCA (Antineutrophil Cytoplasmic Autoantibodies ) vasculitis and lead to a more rapid decrease in disease activity.

ANCA vasculitis is an inflammation of the small vessels whereby ANCA antibodies inappropriately activate one's own white blood cells (neutrophils) and cause damage to the small blood vessels.

Detailed Description

Recent laboratory studies have identified that an important pathway of inflammation called the "complement pathway" may play an important role in how Antineutrophil Cytoplasmic Autoantibodies (ANCA) cause damage to the blood vessels. Eculizumab is a monoclonal antibody that targets a key component of the complement pathway named C5, and blocks its activation.

In a mouse model of ANCA vasculitis, it has been shown that blocking C5 activation can block the development of vasculitis or greatly reduce its severity.

The researchers in this study would like to see if taking eculizumab, in addition to the drugs usually used to treat ANCA vasculitis, would be beneficial in treating ANCA vasculitis.

Currently, the conventional treatment of ANCA vasculitis consists of corticosteroids and cyclophosphamide. The corticosteroids are given as by vein (methylprednisolone) for 3 days followed by prednisone by mouth daily for about 4-5 months. Cyclophosphamide is typically given by vein every 4 weeks for at least 3 months, but sometimes longer depending on whether the vasculitis is still active or not. After the vasculitis is in remission, a maintenance treatment with azathioprine or mycophenolate mofetil may be used. For patients who cannot tolerate cyclophosphamide, or who have received it in large doses previously, another medication called rituximab may be used instead. However, patients who need rituximab or have recently been treated with rituximab cannot participate in this study.

The study drug, eculizumab, is Food and Drug Administration (FDA) approved for indications other than ANCA vasculitis. It is an investigational drug and it is NOT FDA-approved for the treatment of ANCA vasculitis.

In this study, eculizumab will be given in addition to the standard of care treatment for the patients that will be randomised to the eculizumab group.

Recruitment & Eligibility

Status
WITHDRAWN
Sex
All
Target Recruitment
Not specified
Inclusion Criteria

• Patients with active Antineutrophil Cytoplasmic Autoantibodies (ANCA) glomerulonephritis and/or small vessel vasculitis with de novo or relapsing disease (BVAS≥5).

  • Patients must have a current or a history of positive ANCA by the ELISA technique.
  • De novo or relapsing disease requiring immunosuppression.
  • Patients must have evidence of active glomerulonephritis as evidenced by the presence of glomerular hematuria (dysmorphic Red Blood Cells (RBCs) or RBC casts) with or without an increase in serum creatinine.
  • Patients will be eligible within 10 days of commencing induction therapy (i.e., they may have already received pulse methylprednisolone and first dose of cyclophosphamide).
Exclusion Criteria

• Pregnancy or lactation, or women of child bearing potential who are not willing or able to comply with 2 contraceptive methods.

  • Patients with severe renal failure: creatinine > 6 mg/dL or receiving hemodialysis and/or receiving plasmapheresis therapy.
  • Patients with severe pulmonary hemorrhage requiring ventilation and/or plasmapheresis therapy.
  • Patients with active bacterial or viral infection.
  • Absolute neutrophils count < 1000/mm^3 to minimize the risk of infections
  • Hemoglobin < 8.5 g/dL
  • Prior therapy with a monoclonal antibody (for example rituximab)within the previous 6 months. Peripheral CD-20 B-cells count <= 1% due to rituximab even longer than 6 months.
  • Severe coexisting conditions precluding immunosuppressive therapy or conditions requiring intravenous antibiotic therapy.
  • History of infection with Hepatitis B virus (HBV), Hepatitis C virus (HCV), HIV, tuberculosis or syphilis.

Study & Design

Study Type
INTERVENTIONAL
Study Design
PARALLEL
Arm && Interventions
GroupInterventionDescription
Standard of careStandard of care treatmentStandard of care for ANCA vasculitis treatment- depends on severity of disease and individual characteristics and medical history of each patient so this won't be described here.
Eculizumab armeculizumabStandard of care for ANCA vasculitis + eculizumab treatment Standard of care for ANCA vasculitis treatment- depends on severity of disease and individual characteristics and medical history of each patient so this won't be described here.
Primary Outcome Measures
NameTimeMethod
Birmingham Vasculitis Activity Score (BVAS)12 weeks

Change in disease activity as measured by BVAS at 12 weeks.

Secondary Outcome Measures
NameTimeMethod
Complement levels elevationup to 52 weeks

Evaluation of complement levels at study entry to determine which may be elevated in active disease (Bb, C3a, C3d, C3d/C3, C4d, C5a or C5b-9

Birmingham Vasculitis Activity Score(BVAS)up to 52 weeks

Percent of patients with a BVAS =0 at 3 months

Change in complement levelsfrom baseline to week 12

Change in complement levels between groups from baseline to week 12

Normalisation of complement activationup to 52 weeks

Normalization of complement activation at 4 weeks, 8, 12, 24, 36 and 52 weeks

change in complement levels 2up to 52 weeks

Change in these complement levels with treatment and decrease in disease activity for each patient

Birmingham Vasculitis Activity Score (BVAS) 2up to 52 weeks

Mean BVAS at 24, 36 and 52 weeks

Trial Locations

Locations (1)

UNC Kidney Center

🇺🇸

Chapel Hill, North Carolina, United States

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