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Abstract: FR-PO0734

Biopsy-Proven Minimal Change Disease in a Patient with Primary Biliary Cholangitis

Session Information

Category: Glomerular Diseases

  • 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics

Authors

  • Duong, Heidi, University of Southern California, Los Angeles, California, United States
  • Moghadam, Bahman John, University of Southern California, Los Angeles, California, United States
  • Sharma, Neeraj, University of Southern California, Los Angeles, California, United States
Introduction

We present a case of biopsy-proven MCD in a patient with PBC treated with a course of steroids and achieving complete recovery.

Case Description

Hispanic 70F with a recent diagnosis of PBC presented with new onset of shortness of breath and anasarca. During her hospitalization, serum creatinine was at near baseline 1.01 mg/dL, UPCR was 12.95 mg/day, and serum albumin 1.7 g/dL. Nephrotic workup was unrevealing and subsequently a kidney biopsy was pursued which demonstrated extensive foot process effacement consistent with MCD. Given the patient’s worsening renal function and persistent anasarca, patient was started on prednisone 60 mg with tapering regimen, diuretic, ACEI, and a low-sodium diet were prescribed. At one month follow up, patient had complete resolution of nephrotic symptoms, and repeat UPCR was 8.05 mg/day.

Discussion

We present a case of MCD in a patient with clinically diagnosed PBC. Our patient presented with typical signs and symptoms associated with nephrotic syndrome, and biopsy was pursued due to unrevealing possible cause of patient's nephrotic syndrome which demonstrated extensive foot process effacement consistent with MCD. It is thought that MCD is caused by an aberrant immune response by T cell dysregulation, thus the coexistence of both PBC and MCD, it is safe to postulate PBC as the underlying autoimmune cause. Suspecting the autoimmune nature of PBC and MCD, is what makes the clinical course responsive to steroids. Even more, current KDIGO guidelines recommendations include oral glucocorticoid treatment for MCD followed by steroid taper 2 weeks after complete remission (max steroid course length is 16 weeks). For patients with contraindications to steroids or frequent relapses, alternative immunosuppressants such as cyclophosphamide, calcineurin inhibitors, mycophenolate-mofetil, and rituximab can be considered.

Figure 1: Electron Microscopy 4800x; extensive foot process effacement consistent with minimal change nephropathy.