Cirrhosis-Associated IgAN Responsive to Corticosteroids in Three Liver Transplant-Ineligible Patients

Introduction: IgA nephropathy (IgAN) associated with cirrhosis (ESLD-IgAN) is a secondary type of IgAN in which immunosuppression does not have a clear role. Liver transplantation (LT) is often regarded as the only viable therapy. We present 3 cases of biopsy-proven ESLD-IgAN in LT-ineligible patients with decompensated alcoholic cirrhosis who demonstrated substantial improvement in proteinuria and renal function following corticosteroid therapy. Case Description: In all 3 cases,nephrology consultation was placed to rule out hepatorenal syndrome (HRS).The patients presented with acute kidney injury (AKI), hematuria, and proteinuria. Case 1: A 67-year-old man presented with serum creatinine (sCr) 4.5 mg/dL from baseline 0.7 and urine protein-to-creatinine ratio (UPCR) 4.9 g/g. Case 2: a 58-year-old man post-LT with acute rejection after immunosuppression discontinuation, presented with sCr 7.6 mg/dL from baseline 1.5 and a UPCR 1.9 g/g. Case 3: A 47-year-old woman presented with sCr 2.3 mg/dL from baseline 1.2 and a UPCR 3.6 g/g. Urinary sediment microscopy (uSEDI) revealed acanthocytes and/or RBC/WBC casts in all 3 cases. Kidney biopsies showed IgAN (M1, E1, S1, T0/T1, C0/C2). Consistent presence of endocapillary proliferation strengthens the suspicion that the glomerular lesions were associated with ESLD. Due to LT-ineligibility and active ETOH use, hospice was entertained in all 3 cases. However, given their functional status and active glomerular lesions, treatment was offered and prednisone was initiated at 40, 60, and 20 mg daily for cases 1, 2, and 3, respectively, and tapered down over 3-6 months. On follow-up (12-months for cases 1 and 3, 2-months for case 2), there was marked improvement in UPCR (case 1: 4.9 to 0.4 g/g, case 2: 1.9 to 1.0 g/g, and case 3: 3.6 to 0.2 g/g) and sCr (case 1: 7.5 to 3.0 mg/dL, case 2: 7.6 to 3.3 mg/dL, and case 3: 2.3 to 1.1 mg/dL) and resolution of “active” uSEDI. Case 3 completed 1 year of abstinence and has now been listed for LT. Discussion: Cases of ESLD-IgAN may respond favorably to corticosteroids. LT should not be deemed as the only therapeutic option. Successful treatment of ESLD-IgAN may enable LT listing down the road. Careful evaluation of AKI in ESLD should include uSEDI and consider all potential etiologies outside HRS.