A Rollover Extension Program (REP) to Evaluate the Long-term Safety and Tolerability of Open Label Iptacopan/LNP023 in Participants With Primary IgA Nephropathy

Inclusion Criteria:

• For LNP023X2203, participants must have completed part 1 or part 2 of the trial. ForLNP023A2301, participants must have completed the entire core trial defined as thefull 24 month treatment period.

• eGFR* ≥ 20 ml/min/1.73m2

*eGFR calculated using the CKD-EPI formula (or modified MDRD formula according tospecific ethnic groups and local practice guidelines)

• Per investigator's clinical judgement, the participant may benefit from receivingthe open-label treatment of iptacopan 200 mg b.i.d.

• Prior Vaccination against Neisseria meningitidis, Streptococcus pneumoniae andHaemophilus influenzae infections should be up to date (i.e. any boosters requiredadministered according to local regulations.

• All participants must be on supportive care regimen of ACEi or ARB* as per KDIGOguidelines.

• participants who are not taking KDIGO guideline doses because they havedocumented allergies or intolerance to ACEi and ARB are eligible for the study

Exclusion Criteria:

• participants who screen or baseline failed in the CLNP023X2203 Part 1 or Part 2, orCLNP023A2301 studies or who prematurely withdrew from either study for any reason.

• Evidence of severe urinary obstruction or difficulty in voiding; any urinary tractdisorder other than IgAN at screening and before dosing with LNP023.

• Current (within 4 weeks of study drug administration in the REP) acute kidney injury (AKI)

• Presence of Rapidly Progressive Glomerulonephritis (RPGN) as defined by 50% declinein eGFR within the last 3 months.

• Participants treated with immunosuppressive or other immunmodulatory agents such asbut not limited to cyclophosphamide, rituximab, infliximab, eculizumab, canakinumab,mycophenolate mofetil (MMF) or mycophenolate sodium (MPS), cyclosporine, tacrolimus,sirolimus, everolimus and/or systemic corticosteroids exposure (>7.5 mg/dprednisone/prednisolone equivalent) within 5 half-lives of respective medication or 90 days prior to first study drug administration, whichever is shorter. Rituximabrequires 180 days wash out.

• Use of other investigational drugs at the time of enrolment, or within 5 half-livesof enrolment or within 30 days whichever is longer.

• History of recurrent invasive infections caused by encapsulated organisms, such asmeningococcus and pneumococcus.