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Disease

Granulomatosis with polyangiitis

Late-stage therapeutic development
Also known as GPA, Wegener granulomatosis, Wegener's granulomatosis, Wegener's syndrome+6 more

GPA, Wegener granulomatosis, Wegener's granulomatosis, Wegener's syndrome, granulomatosis - Wegener's, necrotizing respiratory granulomatosis, pauci-immune glomerulonephritis associated with granulomatosis with polyangiitis, Midline granulomatosis, ANCA-associated vasculitis, Wg.

MeSHGranulomatosis with Polyangiitis
18
Clinical trials
4
Associated genes
3
Related proteins
Current focus
B-lymphocyte cd20 biologyTherapeutic developmentInflammation & immunity

What's happening now

An analyst briefing on current research, clinical, regulatory and industry activity surrounding this disease.

Executive briefingUpdating summary…Momentum: Moderate
Key developments
  • 5 clinical trials expected to report results, the earliest in Q4 2026.
Upcoming Milestones
Major developments
Upcoming trial readoutHigh impact
Phase 3, Open-label Trial to Evaluate Safety, Pharmacokinetics, and Efficacy of Benralizumab in Children With Eosinophilic Diseases (CLIPS)
Results expected Q1 20282026-09-18
Upcoming trial readoutImportant
Salvage Therapy for Patients With Inadequate Response to Standard of Care Therapy in Granulomatosis With Polyangiitis
Results expected Q4 20282026-04-03
Product authorisation endedWorth watching
Product authorisation ended — Avacopan
Tavneos, in combination with a rituximab or cyclophosphamide regimen, is indicated for the treatment of adult patients with severe, active granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA).2026-08-04
Clinical Milestones6View
Results expected 5
Recently completed 1
Regulatory Updates1View
  • 2026-08-04Product authorisation ended — AvacopanTavneos, in combination with a rituximab or cyclophosphamide regimen, is indicated for the treatment of adult patients with severe, active granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA).
Activity timeline7

Therapeutic landscape

Therapies with a regulatory footing for this condition, alongside the wider set of agents co-studied with it in the literature.

Approved & established therapies
Avacopanapproved

Product authorisation ended — Tavneos, in combination with a rituximab or cyclophosphamide regimen, is indicated for th… (2026)

Rituximabapproved

Biosimilar approval — Ruxience is indicated in adults for the following indications: Non?Hodgkin’s lymphoma (NH… (2020)

Benralizumabapproved

Original application approval — AsthmaFasenra is indicated as an add on maintenance treatment in adult patients with seve… (2018)

Mepolizumabapproved

Original application approval — Severe eosinophilic asthmaNucala is indicated as an add-on treatment for severe refractor… (2015)

Clinical trials

14 sponsors · 1 new · 0 completed in the last 12 months (net +1)

The current development programme across all trial phases.

Clinical programme
18
All trials
6
Active
11
Late-stage
6
Completed
Late-stage studies

Phase 3, Open-label Trial to Evaluate Safety, Pharmacokinetics, and Efficacy of Benralizumab in Children With Eosinophilic Diseases (CLIPS)

Phase 3 · Recruiting · AstraZeneca

Salvage Therapy for Patients With Inadequate Response to Standard of Care Therapy in Granulomatosis With Polyangiitis

Phase 3 · Recruiting · Assistance Publique - Hôpitaux de Paris

Safe Effective Therapy With Low-Dose Glucocorticoid in ANCA-Associated Vasculitis (SAFE-LOW) Trial

Phase 3 · Recruiting · Ottawa Hospital Research Institute

A Multicenter, Single-arm/Randomized, Double-blind, Active-controlled, Parallel-group Phase 2/3 Clinical Study to Evaluate the Efficacy and Safety of SHR-1703 for Patients With EGPA

Phase 2/3 · Recruiting · Guangdong Hengrui Pharmaceutical Co., Ltd

The Assessment of Prednisone In Remission Trial (TAPIR) - Centers of Excellence Approach

Phase 3 · Completed · University of Pennsylvania

MAINtenance of Remission With RITuximab Versus Azathioprine for Patients With Newly-diagnosed or Relapsing Eosinophilic Granulomatosis With Polyangiitis. A Prospective, Randomized, Controlled, Double-blind Study: the MAINRITSEG Trial

Phase 3 · Completed · Assistance Publique - Hôpitaux de Paris
Recently completed

The Assessment of Prednisone In Remission Trial (TAPIR) - Centers of Excellence Approach

Phase 3 · Completed · University of Pennsylvania

MAINtenance of Remission With RITuximab Versus Azathioprine for Patients With Newly-diagnosed or Relapsing Eosinophilic Granulomatosis With Polyangiitis. A Prospective, Randomized, Controlled, Double-blind Study: the MAINRITSEG Trial

Phase 3 · Completed · Assistance Publique - Hôpitaux de Paris

Abatacept (CTLA4-Ig) for the Treatment of Relapsing, Non-Severe, Granulomatosis With Polyangiitis (Wegener's) (ABROGATE)

Phase 3 · Completed · University of South Florida

Mepolizumab Long-term Access Programme for Subjects Who Participated in Study MEA115921 (Placebo-controlled Study of Mepolizumab in the Treatment of Eosinophilic Granulomatosis With Polyangiitis in Subjects Receiving Standard-of-care Therapy)

Phase 3 · Completed · GlaxoSmithKline

Evaluation of Rituximab-based Regimen Compared to Conventional Therapeutic Strategy For Remission Induction In Patients With Newly-Diagnosed or Relapsing Eosinophilic Granulomatosis With Polyangiitis. Prospective, Randomized, Controlled, Double-blind Study

Phase 3 · Completed · Assistance Publique - Hôpitaux de Paris

Short-Course Glucocorticoids and Rituximab in ANCA-Associated Vasculitis

Phase 4 · Completed · Massachusetts General Hospital

Regulatory timeline

Drug regulatory events matched to this condition by indication — EMA.

Product approvals and authorisations
2022emaOriginal application approval — Avacopan· Tavneos, in combination with a rituximab or cyclophosphamide regimen, is indicated for the treatment of adult patients with severe, active granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA). source ↗
2020emaBiosimilar approval — Rituximab· Ruxience is indicated in adults for the following indications: Non?Hodgkin’s lymphoma (NHL) Ruxience is indicated for the treatment of previously untreated patients with stage III?IV follicular lymphoma in combination with chemotherapy. Ruxience maintenance therapy is indicated for the treatment of follicular lymphoma patients responding to induction therapy. Ruxience monotherapy is indicated for treatment of patients with stage III?IV follicular lymphoma who are chemoresistant or are in their second or subsequent relapse after chemotherapy. Ruxience is indicated for the treatment of patients with CD20 positive diffuse large B cell non?Hodgkin’s lymphoma in combination with CHOP (cyclophosphamide, doxorubicin, vincristine, prednisolone) chemotherapy. Chronic lymphocytic leukaemia (CLL) Ruxience in combination with chemotherapy is indicated for the treatment of patients with previously untreated and relapsed/refractory CLL. Only limited data are available on efficacy and safety for patients previously treated with monoclonal antibodies including rituximab or patients refractory to previous rituximab plus chemotherapy. Rheumatoid arthritis Ruxience in combination with methotrexate is indicated for the treatment of adult patients with severe active rheumatoid arthritis who have had an inadequate response or intolerance to other disease?modifying anti?rheumatic drugs (DMARD) including one or more tumour necrosis factor (TNF) inhibitor therapies. Ruxience has been shown to reduce the rate of progression of joint damage as measured by X?ray and to improve physical function, when given in combination with methotrexate. Granulomatosis with polyangiitis and microscopic polyangiitis Ruxience, in combination with glucocorticoids, is indicated for the treatment of adult patients with severe, active granulomatosis with polyangiitis (Wegener’s) (GPA) and microscopic polyangiitis (MPA). Pemphigus vulgaris Ruxience is indicated for the treatment of patients with moderate to severe pemphigus vulgaris (PV). source ↗
2018emaOriginal application approval — Benralizumab· AsthmaFasenra is indicated as an add on maintenance treatment in adult patients with severe eosinophilic asthma inadequately controlled despite high-dose inhaled corticosteroids plus long acting β agonists (see section 5.1).Eosinophilic granulomatosis with polyangiitis (EGPA)Fasenra is indicated as an add-on treatment for adult patients with relapsing or refractory eosinophilic granulomatosis with polyangiitis (see section 5.1). source ↗
2017emaBiosimilar approval — Rituximab· Riximyo is indicated in adults for the following indications: Non-Hodgkin’s lymphoma (NHL) Riximyo is indicated for the treatment of previously untreated adult patients with stage III-IV follicular lymphoma in combination with chemotherapy. Riximyo maintenance therapy is indicated for the treatment of adult follicular lymphoma patients responding to induction therapy. Riximyo monotherapy is indicated for treatment of adult patients with stage III-IV follicular lymphoma who are chemoresistant or are in their second or subsequent relapse after chemotherapy. Riximyo is indicated for the treatment of adult patients with CD20 positive diffuse large B cell non-Hodgkin’s lymphoma in combination with CHOP (cyclophosphamide, doxorubicin, vincristine, prednisolone) chemotherapy. Riximyo in combination with chemotherapy is indicated for the treatment of paediatric patients (aged ? 6 months to < 18 years old) with previously untreated advanced stage CD20 positive diffuse large B-cell lymphoma (DLBCL), Burkitt lymphoma (BL)/Burkitt leukaemia (mature B-cell acute leukaemia) (BAL) or Burkitt-like lymphoma (BLL). Chronic lymphocytic leukaemia (CLL) Riximyo in combination with chemotherapy is indicated for the treatment of patients with previously untreated and relapsed/refractory chronic lymphocytic leukaemia. Only limited data are available on efficacy and safety for patients previously treated with monoclonal antibodies including rituximab or patients refractory to previous rituximab plus chemotherapy. See section 5.1 for further information. Rheumatoid arthritis Riximyo in combination with methotrexate is indicated for the treatment of adult patients with severe active rheumatoid arthritis who have had an inadequate response or intolerance to other disease-modifying anti-rheumatic drugs (DMARD) including one or more tumour necrosis factor (TNF) inhibitor therapies. Rituximab has been shown to reduce the rate of progression of joint damage as measured by X-ray and to improve physical function, when given in combination with methotrexate. Granulomatosis with polyangiitis and microscopic polyangiitis Riximyo, in combination with glucocorticoids, is indicated for the treatment of adult patients with severe, active granulomatosis with polyangiitis (Wegener’s) (GPA) and microscopic polyangiitis (MPA). Riximyo, in combination with glucocorticoids, is indicated for the induction of remission in paediatric patients (aged ? 2 to < 18 years old) with severe, active GPA (Wegener’s) and MPA. Pemphigus vulgaris Riximyo is indicated for the treatment of patients with moderate to severe pemphigus vulgaris (PV). source ↗
2017emaBiosimilar approval — Rituximab· Rixathon is indicated in adults for the following indications: Non-Hodgkin’s lymphoma (NHL) Rixathon is indicated for the treatment of previously untreated adult patients with stage III-IV follicular lymphoma in combination with chemotherapy. Rixathon maintenance therapy is indicated for the treatment of adult follicular lymphoma patients responding to induction therapy. Rixathon monotherapy is indicated for treatment of adult patients with stage III-IV follicular lymphoma who are chemoresistant or are in their second or subsequent relapse after chemotherapy. Rixathon is indicated for the treatment of adult patients with CD20 positive diffuse large B cell non-Hodgkin’s lymphoma in combination with CHOP (cyclophosphamide, doxorubicin, vincristine, prednisolone) chemotherapy. Rixathon in combination with chemotherapy is indicated for the treatment of paediatric patients (aged ? 6 months to < 18 years old) with previously untreated advanced stage CD20 positive diffuse large B-cell lymphoma (DLBCL), Burkitt lymphoma (BL)/Burkitt leukaemia (mature B-cell acute leukaemia) (BAL) or Burkitt-like lymphoma (BLL). Chronic lymphocytic leukaemia (CLL) Rixathon in combination with chemotherapy is indicated for the treatment of patients with previously untreated and relapsed/refractory chronic lymphocytic leukaemia. Only limited data are available on efficacy and safety for patients previously treated with monoclonal antibodies including rituximab or patients refractory to previous rituximab plus chemotherapy. See section 5.1 for further information. Rheumatoid arthritis Rixathon in combination with methotrexate is indicated for the treatment of adult patients with severe active rheumatoid arthritis who have had an inadequate response or intolerance to other disease modifying anti-rheumatic drugs (DMARD) including one or more tumour necrosis factor (TNF) inhibitor therapies. Rituximab has been shown to reduce the rate of progression of joint damage as measured by X-ray and to improve physical function, when given in combination with methotrexate. Granulomatosis with polyangiitis and microscopic polyangiitis Rixathon, in combination with glucocorticoids, is indicated for the treatment of adult patients with severe, active granulomatosis with polyangiitis (Wegener’s) (GPA) and microscopic polyangiitis (MPA). Rixathon, in combination with glucocorticoids, is indicated for the induction of remission in paediatric patients (aged ? 2 to < 18 years old) with severe, active GPA (Wegener’s) and MPA. Pemphigus vulgaris Rixathon is indicated for the treatment of patients with moderate to severe pemphigus vulgaris (PV). source ↗
2015emaOriginal application approval — Mepolizumab· Severe eosinophilic asthmaNucala is indicated as an add-on treatment for severe refractory eosinophilic asthma in adults, adolescents and children aged 6 years and older . Chronic rhinosinusitis with nasal polyps (CRSwNP)Nucala is indicated as an add-on therapy with intranasal corticosteroids for the treatment of adult patients with severe CRSwNP for whom therapy with systemic corticosteroids and/or surgery do not provide adequate control. Eosinophilic granulomatosis with polyangiitis (EGPA)Nucala is indicated as an add-on treatment for patients aged 6 years and older with relapsing-remitting or refractory eosinophilic granulomatosis with polyangiitis (EGPA). Hypereosinophilic syndrome (HES)Nucala is indicated as an add-on treatment for adult patients with inadequately controlled hypereosinophilic syndrome without an identifiable non-haematologic secondary cause. source ↗
1998emaOriginal application approval — Rituximab· MabThera is indicated in adults for the following indications: Non Hodgkin’s lymphoma (NHL) MabThera is indicated for the treatment of previously untreated adult patients with stage III?IV follicular lymphoma in combination with chemotherapy. MabThera maintenance therapy is indicated for the treatment of adult follicular lymphoma patients responding to induction therapy. MabThera monotherapy is indicated for treatment of adult patients with stage III?IV follicular lymphoma who are chemoresistant or are in their second or subsequent relapse after chemotherapy. MabThera is indicated for the treatment of adult patients with CD20 positive diffuse large B cell non?Hodgkin’s lymphoma in combination with CHOP (cyclophosphamide, doxorubicin, vincristine, prednisolone) chemotherapy. MabThera in combination with chemotherapy is indicated for the treatment of paediatric patients (aged ? 6 months  to < 18 years old) with previously untreated advanced stage CD20 positive diffuse large B-cell lymphoma (DLBCL), Burkitt lymphoma (BL)/Burkitt leukaemia (mature B-cell acute leukaemia) (BAL) or Burkitt-like lymphoma (BLL). Chronic lymphocytic leukaemia (CLL) MabThera in combination with chemotherapy is indicated for the treatment of patients with previously untreated and relapsed/refractory CLL. Only limited data are available on efficacy and safety for patients previously treated with monoclonal antibodies including MabThera or patients refractory to previous MabThera plus chemotherapy. Rheumatoid arthritis MabThera in combination with methotrexate is indicated for the treatment of adult patients with severe active rheumatoid arthritis who have had an inadequate response or intolerance to other disease?modifying anti?rheumatic drugs (DMARD) including one or more tumour necrosis factor (TNF) inhibitor therapies. MabThera has been shown to reduce the rate of progression of joint damage as measured by X?ray and to improve physical function, when given in combination with methotrexate. Granulomatosis with polyangiitis and microscopic polyangiitis MabThera, in combination with glucocorticoids, is indicated for the treatment of adult patients with severe, active granulomatosis with polyangiitis (Wegener’s) (GPA) and microscopic polyangiitis (MPA). MabThera, in combination with glucocorticoids, is indicated for the induction of remission in paediatric patients (aged ? 2 to < 18 years old) with severe, active GPA (Wegener’s) and MPA. Pemphigus vulgaris MabThera is indicated for the treatment of patients with moderate to severe pemphigus vulgaris (PV). source ↗
Other regulatory activity
2026emaProduct authorisation ended — Avacopan· Tavneos, in combination with a rituximab or cyclophosphamide regimen, is indicated for the treatment of adult patients with severe, active granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA). source ↗

European Medicines Agency (CC BY 4.0). Events are matched to this condition by drug indication text — approvals/updates for drugs indicated for it, not disease-specific acts.

Associated genes

4 matches

Genes associated with this disease in the canonical knowledge graph (Open Targets evidence). Number shows the association score.

MS4A10.58C5AR10.49TNFSF13B0.27PRTN30.12

Disease biology

3 matches

Proteins whose encoding gene is associated with this disease, reached through the canonical gene→disease graph. Number shows the gene's association score.

B-lymphocyte antigen CD20via MS4A10.58C5a anaphylatoxin chemotactic receptor 1via C5AR10.49Tumor necrosis factor ligand superfamily member 13Bvia TNFSF13B0.27

Reference

Authoritative identity, definition & identifiers.

Defined in MeSH

A multisystemic disease of a complex genetic background. It is characterized by inflammation of the blood vessels (VASCULITIS) leading to damage in any number of organs. The common features include granulomatous inflammation of the RESPIRATORY TRACT and KIDNEYS. Most patients have measurable autoantibodies (ANTINEUTROPHIL CYTOPLASMIC ANTIBODIES) against MYELOBLASTIN.

Identifiers
MeSHGranulomatosis with Polyangiitis
Synonyms

GPA, Wegener granulomatosis, Wegener's granulomatosis, Wegener's syndrome, granulomatosis - Wegener's, necrotizing respiratory granulomatosis, pauci-immune glomerulonephritis associated with granulomatosis with polyangiitis, Midline granulomatosis, ANCA-associated vasculitis, Wg

References & data sources
  • Disease identity & definition — NLM Medical Subject Headings (MeSH), public domain
  • Clinical trials — ClinicalTrials.gov (U.S. National Library of Medicine)
  • Related entities are derived from literature co-mention (studied together) — associative, not causal.