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Disease ID:1226
Associated with:0 target
3 immuno-relevant ligands
Inflammation of the blood vessels.
Database Links
Disease Ontology: DOID:865


No target related data available for Vasculitis


Key to terms and symbols Click ligand name to view ligand summary Click column headers to sort
Ligand References Clinical and Disease comments
Immuno Disease Comments: Phase 3 clinical candidate for the chronic inflammatory disease, antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis- see NCT02994927
Clinical Use: Avacopan (research code CCX168) was evaluated in clinical trials for efficacy in ANCA-associated vasculitis and C3 glomerulopathy. Phase 2 results in ANCA-associated vasculitis were reported in [1]. This study showed that avacopan safely and effectively replaced high dose glucocorticoid () therapy in patients with ANCA-associated vasculitis, which supported progression to Phase 3 trial.
The EMA granted orphan designations for avacopan as a treatment for granulomatosis with polyangiitis, microscopic polyangiitis (both 2014), and C3 glomerulopathy (2017).
Positive results from Phase 3 (ADVOCATE trial NCT02994927 [3]) led to FDA approval of avacopan as an adjunct (in combination with standard therapy) for the treatment of severe ANCA-associated vasculitis in October 2021. | View clinical data
Immuno Disease Comments: Approved drug for vasculitis.
Clinical Use: Anti-tumour agent used in the treatment of acute lymphocytic leukemia (ALL), meningeal leukemia, non-Hogkin's lymphoma, breast, lung and head and neck cancers. Also indicated in the treatment of gestational choriocarcinoma, chorioadenoma destruens and hydatidiform mole, and in the treatment of autoimmune conditions including severe psoriasis and rheumatoid arthritis. The first oral methotraxate solution (Xatmep®) was FDA approved in April 2017 for the treatment of ALL and polyarticular juvenile idiopathic arthritis (pJIA) in pediatric patients. A 2017 a paper reported that a combination of methotrexate with leflunomide relieves the immune defects and ameliorates symptoms of rheumatoid arthritis [4]. | View clinical data
Immuno Disease Comments: In clinical trial for this indication- see NCT01663623.
Clinical Use: Approved as an adjunctive treatment for systematic lupus erythematosus (SLE) [2]. The original formulation of belimumab was administered intravenously. In July 2017, a new self-injectable subcutaneous formulation of belimumab for the treatment of adult patients with active, autoantibody‑positive SLE was FDA approved, which allows patients to self-administer their medicine at home rather than going to medical facilities to receive their i.v. infusions.
Additional indications being evaluated with belimumab include vasculitis (Phase 3), and myaesthenia gravis and transplant rejection (both Phase 2). | View clinical data


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1. Jayne DRW, Bruchfeld AN, Harper L, Schaier M, Venning MC, Hamilton P, Burst V, Grundmann F, Jadoul M, Szombati I et al.. (2017) Randomized Trial of C5a Receptor Inhibitor Avacopan in ANCA-Associated Vasculitis. J Am Soc Nephrol, 28 (9): 2756-2767. [PMID:28400446]

2. Lutalo PM, D'Cruz DP. (2014) Update on belimumab for the management of systemic lupus erythematosus. Expert Opin Biol Ther, 14 (11): 1701-8. [PMID:25303323]

3. Merkel PA, Jayne DR, Wang C, Hillson J, Bekker P. (2020) Evaluation of the Safety and Efficacy of Avacopan, a C5a Receptor Inhibitor, in Patients With Antineutrophil Cytoplasmic Antibody-Associated Vasculitis Treated Concomitantly With Rituximab or Cyclophosphamide/Azathioprine: Protocol for a Randomized, Double-Blind, Active-Controlled, Phase 3 Trial. JMIR Res Protoc, 9 (4): e16664. [PMID:32088663]

4. Zhong W, Zhao L, Liu T, Jiang Z. (2017) IL-22-producing CD4+T cells in the treatment response of rheumatoid arthritis to combination therapy with methotrexate and leflunomide. Sci Rep, 7: 41143. [PMID:28117352]