Biopsy Proven Minimal Change Disease in a Patient With Primary Biliary Cholangitis
- Glomerular Diseases: Podocytopathies and Nephrotic Syndromes
November 03, 2022 | Location: Exhibit Hall, Orange County Convention Center‚ West Building
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1302 Glomerular Diseases: Immunology and Inflammation
- Moghadam, Bahman John, University of Southern California, Los Angeles, California, United States
- Wallace, William, University of Southern California, Los Angeles, California, United States
- Zhong, Yan, University of Southern California, Los Angeles, California, United States
Primary biliary cholangitis (PBC) is an autoimmune liver disease characterized by circulating anti-mitochondrial antibodies (AMA). Though rare, PBC has been associated with varied renal disorders including glomerular diseases such as minimal change disease (MCD). This is a case of biopsy proven MCD in a patient with PBC treated with steroid and achieving complete recovery.
A 70-year-old Hispanic lady with recent diagnosis of PBC presented with new onset dyspnea and anasarca. One month prior, she was evaluated for elevated alkaline phosphatase and abnormal liver function tests (LFTs) including AST/ALT and bilirubin. Workup showed antinuclear antibody titer of 1:1280 and AMA titer of 1:160 resulting in a clinical diagnosis of PBC without need for liver biopsy. On her current hospitalization, serum creatinine was at baseline 1.01 mg/dL, urine protein/creatinine ratio was 12.95 gm/day and serum albumin 1.7 g/dL. Nephrotic work up was unrevealing. Kidney biopsy showed extensive foot process effacement, consistent with MCD (Figure 1). Given worsening renal function and persistent anasarca, Prednisone 60mg with taper, diuretic and angiotensin-converting enzyme inhibitor were prescribed. At one month follow up she had resolution of nephrotic syndrome and normalization of LFTs.
MCD is a clinical and pathological entity defined by proteinuria and hypoalbuminaemia without in the absence of glomerular infiltrates or immunoglobulin deposits. It is thought that MCD is caused by an aberrant immune response and as such, the coexistence of PBC and MCD has previously been postulated to be a result of induction of cell-mediated immunity. Perhaps the combined autoimmune nature of PBC and immune mediated pathology of MCD is what makes the clinical course responsive to steroids. Review of a larger case series could allow for more in-depth insights.
Extensive foot process effacement with minimal change nephropathy