Iptacopan in Patients With ANCA Associated Vasculitis

Inclusion Criteria:

- Newly diagnosed or relapsed GPA and MPA (according to the 2022 ACR/EULAR
classification criteria for GPA and MPA) requiring treatment with RTX and GC as per
investigator's judgement.

- BVAS assessment with ≥1 major item, or ≥3 minor items, or ≥2 renal items at
Screening.

- Positive antibody test for anti-proteinase 3 (PR3) or anti-myeloperoxidase (MPO)
antibodies at Screening or with history of documented evidence of a positive
antibody test.

Exclusion Criteria:

- Other systemic disease which constitutes the primary illness, including but not
limited to: eosinophilic granulomatosis with polyangiitis (EGPA), moderate to severe
systemic lupus erythematosus, IgA vasculitis (Purpura Schönlein-Henoch), rheumatoid
vasculitis, Sjögren's syndrome, anti-glomerular basement membrane (GBM) disease,
cryoglobulinemic vasculitis, autoimmune hemolytic anemia, autoimmune
lymphoproliferative syndrome or mixed connective tissue disease.

- Alveolar hemorrhage requiring invasive pulmonary ventilation support at Screening.

- Severe kidney disease defined as estimated glomerular filtration rate (eGFR) <15
mL/minute/1.73m2, or kidney failure defined as receiving renal replacement therapy
such as hemo(dia)filtration, hemo-/peritoneal dialysis, or having received a kidney
transplant.

- Received plasma exchange/-pheresis within 12 weeks prior to Screening.