Long-term Outcomes After Avacopan Therapy in ANCA-associated Vasculitis: Relapse Risk After Discontinuation and Subgroup Analysis of Patients With Diffuse Alveolar Hemorrhage (AVA-CLEAR-DAH)

Long-term Outcomes After Avacopan Therapy in ANCA-associated Vasculitis: Relapse Risk After Discontinuation and Subgroup Analysis of Patients With Diffuse Alveolar Hemorrhage (AVA-CLEAR-DAH) - AVA-CLEAR-DAH

Status

Active, not recruiting

Phases

Unknown

Study type

Observational

Source

DRKS

Registry ID

DRKS00037247

Enrollment

Registered

2025-06-23

Start date

2025-07-01

Completion date

Unknown

Last updated

2026-08-03

For informational purposes only — not medical advice. Sourced from public registries and may not reflect the latest updates. Terms

Conditions

ANCA-associated Vasculitis M31.3 M31.7

Interventions

Group 1: The “AVA-CLEAR-DAH” study is a retrospective, non-interventional cohort analysis aimed at evaluating the long-term outcomes of patients with ANCA-associated vasculitis (AAV) treated with Avac

Eligibility

Sex/Gender

All

Age

18 Years to No maximum

Inclusion criteria

Inclusion criteria: - MPO-/PR3-associated ANCA-associated vasculitis (microscopic polyangiitis [MPA] / granulomatosis with polyangiitis [GPA]) - Induction therapy with Avacopan - VA-CLEAR: Follow-up period of at least 15 months after initiation of Avacopan induction therapy - AVA-DAH: Patients with diffuse alveolar hemorrhage (DAH) and a follow-up of at least 12 months after start of Avacopan induction therapy Definition of DAH (Diffuse Alveolar Hemorrhage): 1. Radiologic findings: o Compatible chest X-ray or CT scan showing diffuse pulmonary infiltrates AND 2. Exclusion of alternative causes: o No other plausible explanation for the infiltrates (e.g., volume overload, pulmonary infection) AND 3. At least one of the following criteria: o Evidence of alveolar hemorrhage on bronchoscopy or increasingly bloody bronchoalveolar lavage (BAL) fluid o Observed hemoptysis (coughing up blood) o Unexplained anemia with hemoglobin 1 g/dL from a baseline <10 g/dL o Elevated diffusion capacity for carbon monoxide (DLCO)

Exclusion criteria

Exclusion criteria: - <18 years - EGPA

Design outcomes

Primary

Measure	Time frame
Subproject AVA-CLEAR - Maintenance of sustained remission or relapse rate after discontinuation of Avacopan. Additionally, the timepoint of relapse after Avacopan induction will be assessed. Relapse is defined as a recurrence of vasculitis activity following prior remission, based on the presence of 1 major BVAS item, 3 minor BVAS items, or 1–2 minor BVAS items. Subproject AVA-DAH - Remission at 6 months, defined as a Birmingham Vasculitis Activity Score (BVAS) of 0, a glucocorticoid dose <7.5 mg/day, and no relapse within the first 6 months. Sustained remission at 12 months, defined as BVAS = 0 at both 6 and 12 months, a GC dose <7.5 mg/day, and absence of relapse.	—

Measure

Time frame

Subproject AVA-CLEAR - Maintenance of sustained remission or relapse rate after discontinuation of Avacopan. Additionally, the timepoint of relapse after Avacopan induction will be assessed. Relapse is defined as a recurrence of vasculitis activity following prior remission, based on the presence of 1 major BVAS item, 3 minor BVAS items, or 1–2 minor BVAS items. Subproject AVA-DAH - Remission at 6 months, defined as a Birmingham Vasculitis Activity Score (BVAS) of 0, a glucocorticoid dose <7.5 mg/day, and no relapse within the first 6 months. Sustained remission at 12 months, defined as BVAS = 0 at both 6 and 12 months, a GC dose <7.5 mg/day, and absence of relapse.

—

Secondary

Measure	Time frame
Subproject AVA-CLEAR - Comparison between patients who discontinued Avacopan and achieved steroid-free remission versus those who continued glucocorticoid therapy. Daily steroid dose will be stratified as follows: =10 mg/day, >5–9 mg/day, >2.5–5 mg/day, =2.5 mg/day, and 0 mg/day. Time to glucocorticoid discontinuation will also be recorded. - Stratification and adjustment according to induction therapy regimen (Rituximab vs. Cyclophosphamide vs. combined Rituximab/Cyclophosphamide) and ANCA subtype (MPO vs. PR3 antibodies). - Longitudinal analysis of clinical and laboratory parameters: BVAS, eGFR slope, serum creatinine, CRP, albuminuria (UACR), proteinuria (UPCR), and hematuria—measured at approximately 1, 3, 6, 9, and 12 months post-induction. - Duration of Avacopan treatment and documented reasons for discontinuation. - Adverse events (AEs), serious adverse events (SAEs), hospitalization rate, mortality, infections, and glucocorticoid-related side effects (categorized for the Avacopan treatment phase vs. the post-Avacopan phase). Subproject AVA-DAH - Laboratory parameters (evaluated at 1, 3, 6, 9, and 12 months): eGFR, serum creatinine, CRP, albuminuria, proteinuria, hematuria. Renal parameters are only assessed in patients with documented renal involvement. - Relapse, defined as the return of vasculitis activity after remission, meeting one major BVAS item, three minor items, or one to two minor items and requiring escalation of immunosuppressive therapy. Adjustments to GC dosage without clinical relapse will not be classified as relapse. - Subgroup analysis: severe vs. non-severe DAH. Severe DAH is defined as SpO2 <85% on room air or requiring high-flow nasal cannula (HFNC). - Subgroup analysis by time to Avacopan initiation: patients will be divided into early vs. delayed Avacopan start based on the median time from diagnosis to initiation. - Subgroup analysis: ICU-treated patients vs. non-ICU patients. - Radiographic follow-up (chest X-ray or CT)	—

Measure

Time frame

Subproject AVA-CLEAR - Comparison between patients who discontinued Avacopan and achieved steroid-free remission versus those who continued glucocorticoid therapy. Daily steroid dose will be stratified as follows: =10 mg/day, >5–9 mg/day, >2.5–5 mg/day, =2.5 mg/day, and 0 mg/day. Time to glucocorticoid discontinuation will also be recorded. - Stratification and adjustment according to induction therapy regimen (Rituximab vs. Cyclophosphamide vs. combined Rituximab/Cyclophosphamide) and ANCA subtype (MPO vs. PR3 antibodies). - Longitudinal analysis of clinical and laboratory parameters: BVAS, eGFR slope, serum creatinine, CRP, albuminuria (UACR), proteinuria (UPCR), and hematuria—measured at approximately 1, 3, 6, 9, and 12 months post-induction. - Duration of Avacopan treatment and documented reasons for discontinuation. - Adverse events (AEs), serious adverse events (SAEs), hospitalization rate, mortality, infections, and glucocorticoid-related side effects (categorized for the Avacopan treatment phase vs. the post-Avacopan phase). Subproject AVA-DAH - Laboratory parameters (evaluated at 1, 3, 6, 9, and 12 months): eGFR, serum creatinine, CRP, albuminuria, proteinuria, hematuria. Renal parameters are only assessed in patients with documented renal involvement. - Relapse, defined as the return of vasculitis activity after remission, meeting one major BVAS item, three minor items, or one to two minor items and requiring escalation of immunosuppressive therapy. Adjustments to GC dosage without clinical relapse will not be classified as relapse. - Subgroup analysis: severe vs. non-severe DAH. Severe DAH is defined as SpO2 <85% on room air or requiring high-flow nasal cannula (HFNC). - Subgroup analysis by time to Avacopan initiation: patients will be divided into early vs. delayed Avacopan start based on the median time from diagnosis to initiation. - Subgroup analysis: ICU-treated patients vs. non-ICU patients. - Radiographic follow-up (chest X-ray or CT)

—

Countries

Germany

Contacts

Public ContactAdrian Schreiber

Charité – Universitätsmedizin Berlin

adrian.schreiber@charite.de+49 030 450 665277

Outcome results

None listed

Source: DRKS (via WHO ICTRP) · Data processed: Aug 10, 2026