Study to Assess the Efficacy, Pharmacokinetics, Safety and Tolerability of Atrasentan in Pediatric Patients With Primary IgAN

A Phase III, single-arm, multicenter pediatric clinical study evaluating atrasentan in children and adolescents aged 2 to \

Inclusion Criteria: 1. Signed informed consent by parent(s)/legal guardian(s) for the pediatric patient must be obtained before any study-specific assessment is performed. A consent or assent may also be required for some participants depending upon their age and local requirement. 2. Male and female participants 2 to \< 18 years of age as of Day 1. 3. eGFR ≥ 30 mL/min/1.73m2 where eGFR is calculated using the modified Schwartz formula at Screening and confirmed during the Run-in Period. 4. Kidney biopsy-proven primary IgAN\*, with biopsy performed within 3 years of Screening with \< 50% tubulointerstitial fibrosis and \< 25% crescents. In case a kidney biopsy within 3 years from Screening is not available, a kidney biopsy may be performed if it is part of the planned diagnostic approach and clinical management of the participant. 5. Proteinuria due to primary diagnosis of IgAN as assessed by UPCR ≥ 1 g/g (113 mg/mmoL) sampled from FMV at Screening on Day -90 and Day -60 as well as during the Run-in Period despite treatment with maximum tolerated dose of ACE inhibitor/ARB for at least 120 days prior to Day 1. Note: UPCR will be assessed based on one FMV sample at Day -90 and based on the geometric mean of 2 FMV samples for the Day -60 visit and during the Run-in Period. 6. All participants must have been on supportive care including stable dose regimen of ACE inhibitor or ARB at either the locally approved maximal daily dose per body weight, or the maximally tolerated dose (per Investigator's judgment for pediatric use), for at least 120 days before first study drug administration. In addition, if participants are taking diuretics, other antihypertensive medication, or other background medication for IgAN (such as SGLT2 inhibitors), the doses should also be stabilized for at least 120 days prior to the first dosing of study treatment. Note: Participants with allergies or intolerance to ACE inhibitors and ARBs are eligible for the study. Participants with allergies or intolerance to RAS inhibitors are eligible but will not exceed \~5% of the total population treated (maximum of 2 participants). 7. The minimum body weight of enrolled pediatric participants is 10 kg at Screening and confirmed on Day 1. 8. Parent(s)/guardian(s) are to be able to communicate well with the investigator and to understand and comply with the study's requirements for their child. Exclusion Criteria: 1. Participation in any other investigational drug trial or use of other investigational drugs at the time of enrollment, or within 5 elimination half-lives of enrollment, or within 30 days of enrollment, whichever is longer; or longer if required by local regulations. 2. History of hypersensitivity to any of the study drugs or its excipients or to drugs of similar chemical classes. 3. Any secondary IgAN as defined by the investigator; secondary IgAN can be associated with cirrhosis, celiac disease, Human Immunodeficiency Virus (HIV) infection, Herpes Simplex virus infection, dermatitis herpetiformis, seronegative arthritis, small-cell carcinoma, lymphoma, disseminated tuberculosis, bronchiolitis obliterans, inflammatory bowel disease, and familial mediterranean fever before treatment. 4. A clinical diagnosis of IgA vasculitis (IgAV or Henoch-Schoenlein purpura) based on typical palpable purpura with or without arthralgia and abdominal pain. 5. Evidence of significant urinary obstruction or difficulty in voiding, any urinary tract disorder causing significant urinary obstruction or difficulty in voiding at Screening and confirmed at Baseline/Day 1. 6. Concurrent diagnosis of CKD other than IgAN at Screening and before first study drug administration. 7. Current acute kidney injury (AKI) defined by Acute Kidney Injury Network (AKIN) criteria within 4 weeks of Screening. 8. Presence of rapidly progressive glomerulonephritis (RPGN) as defined by 50% decline in eGFR within 3 months prior to Screening or during Screening and Run-in periods. 9. Presence of nephrotic syndrome at Screening based on the investigator's judgement. 10. BNP value of \>200 pg/mL at Screening. 11. Hemoglobin below 9 g/dL at Screening or prior history of blood transfusion for anemia within 3 months of Screening. 12. Platelet count \